Rare Cancer News & Clinical Trials » Hemangioendothelioma

Hemangioendothelioma (168 unread)

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Idiopathic spontaneous liver hemorrhage A report of two cases and a review of the literature

Fetched: September 16th, 2021, 5:01am GMT by Bruno Cirillo

Ann Ital Chir. 2021;92:361-364.

ABSTRACT

Spontaneous liver hemorrhage (SLH) is a serious, extremely rare, and life-threatening occurrence requiring a multidisciplinary approach. Since diagnosis might be difficult, a high mortality rate is reported. Survival depends on a prompt diagnosis followed by an appropriate management. If left untreated, SLH progresses, in fact, to a hemorrhagic shock and death. SLH is rarely idiopathic, whereas more commonly is secondary to severe preeclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome, hepatocellular carcinoma (HCC), adenoma, focal nodular hyperplasia or hemangioma, and connective tissue diseases. We report two patients presenting with an idiopathic SLH successfully treated with angioembolization, and the results of an extensive literature review. KEY WORDS: Intrahepatic hematoma, Spontaneous liver hemorrhage, Spontaneous liver rupture.

PMID:34524110

A new form of Kaposi sarcoma

Fetched: September 16th, 2021, 5:01am GMT by Leonie A J Derickx

Ned Tijdschr Geneeskd. 2021 Aug 19;165:D5966.

ABSTRACT

BACKGROUND: Kaposi sarcoma is an vascular neoplasm caused by infection with human herpesvirus-8. Known risk groups are Mediterranean, eastern European Jewish and African ancestry men or men with AIDS. Nowadays we distinguish more subgroups.

CASE DESCRIPTION: We present a healthy 39 year old man with a lesion on the right foot, having homosexual contacts, without HIV infection. Previous histology revealed signs of hemangioma. Recent clinical signs and histology confirmed multifocal Kaposi sarcoma. He was treated with radiotherapy. We also present a 65 year old MSM with and a lesion on the sole of the foot. Histology revealed for a solitary nodular Kaposi sarcoma. A short term relapse after surgical excision occurred.

CONCLUSION: Kaposi sarcoma is subdivided into different categories. Kaposi Sarcoma in HIV-negative MSM is seen more frequently today, yet usually shows an indolent course. An adjusted less aggressive treatment and follow-up is therefore justified.

PMID:34523837

Intramuscular Hemangioma of Masseter Muscle: Case Report of Rare Clinical Entity

Fetched: September 16th, 2021, 5:01am GMT by Ramanpal Singh Makkad

Ann Maxillofac Surg. 2021 Jan-Jun;11(1):148-151. doi: 10.4103/ams.ams_100_20. Epub 2021 Jul 24.

ABSTRACT

RATIONALE: Intramuscular hemangiomas are unique benign vascular tumours of skeletal muscles; involving masseter and trapezius muscles in the majority of cases. The rationale was to emphasize that the diagnosis of asymptomatic swelling in the masseteric region is important as due to their deep anatomic location and unfamiliar presentation, they are often misdiagnosed as a parotid swelling or other muscular pathologies.

PATIENT CONCERN: This report describes a rare case of a 25-year-old healthy male patient who presented with an asymptomatic swelling in the right masseteric region. The patient had cosmetic concerns due to the large size.

DIAGNOSIS: Colour Doppler ultrasonography was done to assess the vascularity within the lesion.

TREATMENT: Complete excision was successfully achieved using combined Risdon's and preauricular approach.

OUTCOME: No signs of recurrence were observed after 6 months.

TAKE-AWAY LESSONS: Appropriate selection of diagnostic modalities enables the clinician in making an accurate preoperative diagnosis of progressive swelling in the masseteric region.

PMID:34522672 | PMC:PMC8407625 | DOI:10.4103/ams.ams_100_20

Conjunctival Pyogenic Granuloma Masquerading as Malignant Melanoma

Fetched: September 16th, 2021, 5:01am GMT by Priyanka R Rao

Cureus. 2021 Aug 9;13(8):e17029. doi: 10.7759/cureus.17029. eCollection 2021 Aug.

ABSTRACT

Pyogenic granuloma is a common, benign, vascular growth that often appears as a rapidly growing mass on mucus membrane-lined surfaces such as the conjunctiva. Conjunctival pyogenic granulomas are common following trauma, burst chalazion or ill-fitting prosthesis. Also known as 'lobular capillary hemangiomas', these lesions typically appear bright red, fleshy and pedunculated. Treatment options include excision, topical steroid therapy and topical beta-blocker therapy. In this communication, the authors describe a rapidly enlarging, pedunculated black coloured conjunctival mass lesion in a 44-year-old woman, who had a recent history of chalazia. Given the location and the clinical appearance, a melanocytic tumour was suspected and the mass was excised. Histopathology and immunohistochemical studies confirmed the diagnosis to be consistent with that of a a necrotic pyogenic granuloma. Pigmented lesions of the conjunctiva, especially rapidly enlarging ones, need to be viewed with a high degree of suspicion to rule out malignant melanoma. Rarely though, benign lesions such as pyogenic granulomas that undergo necrosis may masquerade as conjunctival melanomas.

PMID:34522510 | PMC:PMC8425498 | DOI:10.7759/cureus.17029

Permalink for 'Clinical Importance of Incidentally Detected Hyperenhancing Liver Observations on Portal Venous Phase Computed Tomography in Patients Without Known Malignancy or Liver Disease'

Clinical Importance of Incidentally Detected Hyperenhancing Liver Observations on Portal Venous Phase Computed Tomography in Patients Without Known Malignancy or Liver Disease

Fetched: September 15th, 2021, 5:01am GMT by Michael T Corwin

J Comput Assist Tomogr. 2021 Jul-Aug 01;45(4):516-521. doi: 10.1097/RCT.0000000000001154.

ABSTRACT

OBJECTIVE: The aim of the study was to determine the prevalence of clinically important masses among incidental hyperenhancing liver observations on portal venous phase computed tomography (CT) in patients without known malignancy or liver disease.

METHODS: Retrospective search of portal venous phase CTs was performed to identify hyperenhancing liver observations in patients without cancer or liver disease. Observations were assigned a morphology of homogeneous, hemangioma, or heterogeneous. The reference standard was pathology (n = 2), liver protocol CT/magnetic resonance imaging (n = 40), follow-up portal venous phase CT for 2 years or more (n = 81), or clinical follow-up for 5 years or more (n = 107).

RESULTS: There were no clinically important masses among 83 observations with homogeneous morphology or 110 with hemangioma morphology. There were 2 clinically important masses (1 hepatocellular carcinoma and 1 hepatic adenoma) among 37 (5.4%) heterogeneous morphology observations.

CONCLUSIONS: Incidental hyperenhancing liver observations on portal venous phase CT with homogeneous or typical hemangioma morphology in patients without known cancer or liver disease are highly likely benign.

PMID:34519450 | DOI:10.1097/RCT.0000000000001154

Truncus arteriosus as presentation of PHACE syndrome

Fetched: September 15th, 2021, 5:01am GMT by Ekarat Nitiyarom

BMJ Case Rep. 2021 Sep 13;14(9):e244620. doi: 10.1136/bcr-2021-244620.

ABSTRACT

PHACE syndrome is a rare neurocutaneous syndrome that describes the association of large segmental infantile haemangioma involving the head and neck, along with other systemic anomalies. Complex congenital heart disease has rarely been reported in this syndrome. We present a report of a patient with PHACE syndrome and truncus arteriosus.

PMID:34518186 | DOI:10.1136/bcr-2021-244620

Surgical Management of Giant Hepatic Haemangioma - Need for Redefining the Nomenclature According to the Size

Fetched: September 14th, 2021, 5:01am GMT by Lileswar Kaman

Pol Przegl Chir. 2021 Mar 22;93(4):28-34. doi: 10.5604/01.3001.0014.8105.

ABSTRACT

INTRODUCTION: Haemangiomas are the most common benign tumours of the liver. Most of them are asymptomatic. Giant hepatic haemangioma is defined as size greater than 5 cm in diameter. The surgical treatment for giant hepatic haemangioma is not very well defined and reported. Here we analysed the treatment outcome of giant hepatic haemangiomas and redefined the nomenclature according to the size and proposing an algorithm for management of all hepatic haemangiomas.

MATERIAL AND METHODS: Retrospective analyses of 6 giant hepatic haemangioma more than 10 cm in size treated by a single surgeon were included. The clinical characteristics, diagnosis, managements and outcomes were recorded. Review of literature was done for definitions, management strategies and outcome of giant haemangiomas and an algorithm was proposed.

RESULTS: Five patients were female and the mean age was 36.6 years (range 32 to 45). Pain abdomen was the most common symptoms (100%). Ultrasound abdomen was the initial diagnostic modality followed by contrast enhanced computed tomography. The mean size of haemangioma was 17.4cm (range 12cm to 32cm). Four patients had haemangioma in the left lobe and two had in the right lobe of liver. Formal hepatectomy was done in three patients and enucleation was done in three patients. The mean operating time was 3.66 hours (range 2.5 hours to 5 hours). The mean blood loss was 840ml (range 300ml to 1500ml). There was no surgical morbidity or mortality. On follow-up no haemangioma related complications are reported.

CONCLUSIONS: Giant hepatic haemangioma is mostly symptomatic. Hepatectomy and enucleation can be done without significant morbidity and mortality. There is a need for redefining the nomenclature of giant hepatic haemangiomas according to the size.

PMID:34515653 | DOI:10.5604/01.3001.0014.8105

Multifocal Pseudomyogenic Hemangioendothelioma Involving the Scalp and Nose, Misdiagnosed as A Sarcoma: A Rare Case Report

Fetched: September 14th, 2021, 5:01am GMT by Neha Mittal

Turk Patoloji Derg. 2021 May 17. doi: 10.5146/tjpath.2021.01539. Online ahead of print.

ABSTRACT

This case report aims to present clinicopathological features of an extremely rare case of multifocal pseudomyogenic hemangioendothelioma (PMHE) in the scalp. A 21-year-old male developed multiple, focally ulcerated, nodules over the root of his nose and scalp. One of the skin lesions was sampled at another dermatology clinic, where this was diagnosed as a sarcoma. A review of biopsy sections showed well-circumscribed dermal lesions, comprising plump spindle and epithelioid cells, mimicking rhabdomyoblasts. Immunohistochemically, tumor cells were positive for AE1/AE3, CD31, FLI-1 and ERG. INI-1 was retained. A diagnosis of PMHE was offered. Subsequently, the patient underwent wide excision and has been asymptomatic for 8 months, post-surgery. PMHE is rarely reported in the head and neck region, where it can constitute a diagnostic pitfall. Awareness of this tumor and appropriate immunohistochemical stains are necessary for its timely diagnosis, in order to avoid radical treatments. A review of similar, previously documented cases is presented.

PMID:34514569 | DOI:10.5146/tjpath.2021.01539

Liver transplantation in malignant disease

Fetched: September 14th, 2021, 5:01am GMT by Sven Arke Lang

World J Clin Oncol. 2021 Aug 24;12(8):623-645. doi: 10.5306/wjco.v12.i8.623.

ABSTRACT

Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology. Following the implementation of the Milan criteria, hepatocellular carcinoma (HCC) was the first generally accepted indication for transplantation in patients with cancer. Subsequently, more liberal criteria for HCC have been developed, and research on this topic is still ongoing. The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies. Regarding perihilar cholangiocarcinoma, more and more evidence supports the use of liver transplantation, especially after neoadjuvant therapy. In addition, some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma. Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer. In contrast, patients with hepatic angiosarcoma are currently not considered to be optimal candidates. In secondary liver tumors, neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation. Recently, some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases. This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.

PMID:34513597 | PMC:PMC8394155 | DOI:10.5306/wjco.v12.i8.623

Pyogenic Granuloma Mimicking T1 Colorectal Carcinoma

Fetched: September 14th, 2021, 5:01am GMT by Naoki Asayama

Cureus. 2021 Aug 29;13(8):e17536. doi: 10.7759/cureus.17536. eCollection 2021 Aug.

ABSTRACT

Pyogenic granuloma (PG), a benign capillary hemangioma, is extremely rare in the colon. Here, we present a case of PG that was difficult to distinguish from T1 (deep submucosal invasive) colorectal carcinoma. A 57-year-old woman with no remarkable history was referred to us for a detailed investigation of a positive fecal occult blood test. Colonoscopy revealed a reddish, irregular-shaped, protruding lesion (5 mm) in the rectum. We performed endoscopic mucosal resection of the lesion as total excisional biopsy because T1 colorectal carcinoma was suspected, despite the lesion's small size after observation by magnifying endoscopy. Histologically, the protruding lesion mainly consisted of numerous capillaries lined with plump and flat endothelial cells without signs of malignancy. Colorectal carcinoma, on the other hand, is composed of tall columnar atypical epithelial cells showing neoplastic proliferation. Thus, cell morphology is completely different between PG and colorectal carcinoma. The final diagnosis was colonic PG with a negative vertical margin. In conclusion, physicians should be aware of a colorectal protruding lesion devoid of malignant potential, as in this case, where the lesion was difficult to diagnose accurately and to distinguish from T1 colorectal carcinoma on magnifying endoscopy. Physicians should consider PG as a differential diagnosis in similar cases.

PMID:34513527 | PMC:PMC8405384 | DOI:10.7759/cureus.17536

Propranolol in the Treatment of Infantile Hemangiomas

Fetched: September 14th, 2021, 5:01am GMT by Xin Tan

Clin Cosmet Investig Dermatol. 2021 Sep 3;14:1155-1163. doi: 10.2147/CCID.S332625. eCollection 2021.

ABSTRACT

Propranolol, as the first generation of β-blocker family, was initially introduced in the clinical application for tachycardia and hypertension in the 1960s. However, the occasional discovery of propranolol in the involution of infantile hemangiomas (IHs) brought us a new perspective. IHs are the most common infantile tumor, affecting 4-10% newborns. So far, oral propranolol is the first-line medication for IHs treatment. At the same time, local injection and topical propranolol are developing. Despite the worldwide application, the precise mechanism of propranolol of IHs has not been completely studied. In this article, we reviewed and summarized the current information on pharmacology, mechanism, efficacy, and adverse effects of propranolol. Novel design of biomaterials and bioactive molecules are needed for new treatment and ideal pathway to attain the minimal effective treatment concentration and eliminate the adverse effects.

PMID:34511960 | PMC:PMC8423716 | DOI:10.2147/CCID.S332625

Recurrent Epistaxis and Unilateral Intranasal Mass in A Teenager

Fetched: September 14th, 2021, 5:01am GMT by Brad Bradshaw

Am J Case Rep. 2021 Sep 13;22:e933075. doi: 10.12659/AJCR.933075.

ABSTRACT

BACKGROUND Epistaxis in children is a common problem encountered in outpatient clinics and emergency departments. A wide variety of conditions may cause recurrent epistaxis in children. We describe clinical, radiologic, and histologic features of a lobular capillary hemangioma presenting as a rapidly growing intranasal mass in a child with recurrent epistaxis. CASE REPORT A 16-year-old male presented with a 2-month history of recurrent unilateral epistaxis requiring multiple visits to the emergency department. The child had nasal obstruction, snoring, no recurrent sinus infections, no anosmia nor hyposmia, no weight loss, no night sweats, no fever, no decreased activity, and no easy bruising. He denied any history of local trauma. On physical examination, a fleshy violaceous mass was found, protruding from and obliterating the right nasal cavity. Magnetic resonance imaging documented an avidly enhancing mass centered at the right nasal vestibule. Upon resection, histologic evaluation indicated a pyogenic granuloma. At the 2-month followup, the surgical site was healed with no evidence of recurrent lesion. CONCLUSIONS Lobular capillary hemangioma, although uncommon, should be considered in the differential diagnosis of recurrent epistaxis and intranasal mass in children.

PMID:34511595 | DOI:10.12659/AJCR.933075

Permalink for 'Circumscribed Choroidal Hemangioma: Comparative Efficacy of Transpupillary Thermotherapy, Indocyanine Green-Enhanced Transpupillary Thermotherapy, and Photodynamic Therapy and Baseline Clinical Features Affecting Treatment Outcomes'

Circumscribed Choroidal Hemangioma: Comparative Efficacy of Transpupillary Thermotherapy, Indocyanine Green-Enhanced Transpupillary Thermotherapy, and Photodynamic Therapy and Baseline Clinical Features Affecting Treatment Outcomes

Fetched: September 13th, 2021, 5:01am GMT by Ahmet Kaan Gündüz

Photodiagnosis Photodyn Ther. 2021 Sep 9:102529. doi: 10.1016/j.pdpdt.2021.102529. Online ahead of print.

ABSTRACT

PURPOSE: To compare the efficacy of transpupillary thermotherapy (TTT), indocyanine green-enhanced TTT (ICG-TTT), and photodynamic therapy (PDT) in the management of circumscribed choroidal hemangioma (CCH) and to investigate the factors affecting treatment outcomes METHODS: Retrospective review of clinical records of 60 eyes with CCH which underwent TTT (25), ICG-TTT (22), or PDT (13). Main study outcomes were 1) final visual acuity (VA) ≤20/200, 2) ˂2 Snellen lines VA increase, 3) persistent subretinal fluid (SRF), and 4) ˂20% decrease in tumor thickness.

RESULTS: Multivariable factors associated with final VA ≤20/200 included longer duration of symptoms (p=0.015), lower initial VA (0.030), and presence of retinoschisis overlying the tumor (0.047). Multivariable factors for ˂2 Snellen lines VA increase were longer duration of symptoms (p=0.018) and previous failed treatment (p=0.003). By multivariable analysis, the only significant factor for persistent SRF was the presence of retinoschisis (p=0.001). Multivariable factors associated with a decrease in tumor thickness by ˂20% were smaller initial tumor thickness (p=0.045) and presence of retinoschisis (p=0.014). By Pearson Chi-Square/Fisher Exact Test, final VA ≤20/200 rates (48.0%, 13.6%, 30.8% respectively, p=0.041) and VA improvement by ˂2 lines rates (64.0%, 27.3%, 38.5% respectively, p=0.036) were significantly different between TTT, ICG-TTT, and PDT groups. However, by post hoc analysis, ICG-TTT yielded statistically better outcomes compared to TTT with respect to final visual acuity (VA) ≤20/200 (p=0.012) and ˂2 Snellen lines VA increase (p=0.012).

CONCLUSIONS: Longer symptom duration, previous failed treatment, lower initial VA, and presence of retinoschisis were risk factors for worse visual outcomes. Eyes with retinoschisis displayed more SRF persistence after treatment. Smaller initial tumor thickness and presence of retinoschisis were risk factors for ˂20% decrease in tumor thickness.

PMID:34509682 | DOI:10.1016/j.pdpdt.2021.102529

Surgical treatment of intraorbital lesions

Fetched: September 13th, 2021, 5:01am GMT by A Kaywan Aftahy

World Neurosurg. 2021 Sep 9:S1878-8750(21)01349-8. doi: 10.1016/j.wneu.2021.09.011. Online ahead of print.

ABSTRACT

BACKGROUND: Resection of pure intraorbital tumors is challenging due to immediate vicinity to the optic apparatus. We report our experience with different intraorbital tumors and discuss classic approaches and obstacles.

METHODS: A retrospective case series at a tertiary neurosurgical center of patients who underwent surgery for intraorbital tumors between 06/2007 - 01/2020 was performed.

RESULTS: We included 34 patients (median age 58, range of 18-87 years, 55.9% [19/34] female, 44.1% [15/34] male). Preoperative proptosis was observed in 67.6% (23/34), visual impairment in 52.9% (18/34), diplopia in 41.2% (14/34) and ptosis in 38.3% (13/34). Intraconal tumors were found in 58.8% (20/34). Most common lesions were cavernous hemangiomas in 26.5% (9/34) and metastases in 14.7% (5/34). Gross total resection rate was 73.5% (25/34). Planned biopsy was performed in 14.7% (5/34). Median follow-up time was 15.5 months (range 0-113). 23.5% (8/34) underwent a supraorbital approach, 52.9% (18/34) a pterional approach, 14.7% (5/34) a lateral orbitotomy, 5.9% (2/34) a transnasal approach and one patient (2.9%) underwent a combined approach (transnasal/lateral orbitotomy). Excluding planned biopsies, gross total resection was achieved in 80.0% (12/15) with a pterional, 100% (7/7) with a supraorbital approach, 80.0% (4/5) with a lateral orbitotomy, 100% (1/1) via a transnasal and a combined approach, respectively. Complication rate requiring surgical intervention was 11.8 % (4/34).

CONCLUSION: Considering the low operative morbidity and satisfying functional outcome, gross total resection of intraorbital lesions is feasible. We support the use of classic transcranial and transorbital approaches. More invasive and complicated approaches were not needed in our series.

PMID:34509678 | DOI:10.1016/j.wneu.2021.09.011

Multiple Cavernous Haemangioma of Orbit and Cranium: A Case Report

Fetched: September 12th, 2021, 5:01am GMT by Purnima Rajkarnikar Sthapit

JNMA J Nepal Med Assoc. 2021 Jan 31;59(233):74-76. doi: 10.31729/jnma.5606.

ABSTRACT

A 32-year-old male presented with painless proptosis and diminution of vision in left eye. Imaging shows multiple well-defined masses, suggestive of cavernous haemangioma, in orbit and cranium with adjoining bones being thickened with cystic spaces. Histopathology proved the diagnosis. Cavernous haemangioma usually presents as a solitary intraconal and sometimes extraconal mass with vision usually preserved unless it extends to the apical portion. Here we report a rare case of multiple simultaneous locations of cavernous haemangiomas in orbit and cranium with significant diminution of vision.

PMID:34508450 | PMC:PMC7893390 | DOI:10.31729/jnma.5606

Gastrointestinal infantile hemangioma: A rare cause of digestive tract bleeding in children to consider

Fetched: September 12th, 2021, 5:01am GMT by Idalia Cura-Esquivel

Clin Case Rep. 2021 Sep 5;9(9):e04722. doi: 10.1002/ccr3.4722. eCollection 2021 Sep.

ABSTRACT

Intestinal hemangiomas are an infrequent cause of gastrointestinal bleeding and chronic anemia in infants, which diagnosis depends on high suspicion. Propranolol has been described as an effective treatment.

PMID:34504695 | PMC:PMC8418681 | DOI:10.1002/ccr3.4722

Antiproliferative therapy with sirolimus and propranolol for congenital vascular anomalies in newborns (Case reports)

Fetched: September 12th, 2021, 5:01am GMT by Cătălin Cîrstoveanu

Exp Ther Med. 2021 Oct;22(4):1097. doi: 10.3892/etm.2021.10531. Epub 2021 Aug 2.

ABSTRACT

We present a series of four newborns diagnosed with complicated congenital vascular anomalies, with different localization: Congenital lymphatic malformation (CLM) on the left hemithorax extending on the left upper limb; congenital hepatic hemangioma (CHH) with important complications in the first 7 weeks of life; Kaposiform hemangioendothelioma (KHE) of the left lower limb complicated with Kasabach Merritt phenomenon (KMM) and most probable diffuse capillary malformation with overgrowth (DCMO). All patients were treated with combined antiproliferative therapy with sirolimus and propranolol. The initial dose of sirolimus was 0.45-0.5 mg/m² with doses adjusted according to plasmatic levels. Therapeutic intervals of sirolimus were considered at plasmatic levels of 7-12 ng/ml. Our aim was to use the lowest therapeutic dose in order to avoid possible side effects. Propranolol was initiated in doses of 0.5-1.0 mg/kg/day and was increased up to 3.0 mg/kg/day depending on tolerability. Following two months, every patient showed a marked reduction in the size of the mass, improvement in overall appearance or even calcification in the liver vascular tumor. No patient showed life threatening side effects to the treatment. Hypertriglyceridemia was the only side effect noted in all patients. This is in accordance with several international studies, which try to demonstrate the importance of sirolimus in neonatal vascular malformations in monotherapy or combined with different drugs.

PMID:34504551 | PMC:PMC8383751 | DOI:10.3892/etm.2021.10531

Multiple Acquired Elastotic Hemangioma in a Single Patient: A Case Report

Fetched: September 12th, 2021, 5:01am GMT by Pramisha Kharel

JNMA J Nepal Med Assoc. 2020 Oct 15;58(230):809-812. doi: 10.31729/jnma.5186.

ABSTRACT

Acquired elastotic hemangioma is a hemangioma variant which was first described in 2002. Usually it is characterized by being a benign, solitary, slow growing lesion and associated with solar exposure but here we discuss a case of 50 years old male from Australia, who had multiple pigmented and violaceous lesions on the back which he had noticed for 5 years. The cases of multiple lesions are reported few in the literature. The lesions had characteristic clinical dermatoscopic feature showing typical violaceous lesions with widespread shiny white structure without any vessels. Histopathology revealed band like proliferation of blood vessels involving superficial dermis arranged horizontally parallel to the epidermisalong with elastosis in dermis. Treatment was done through surgical excision with no relapse reported. Keywords: acquired elastotic hemangioma;basal cell carcinoma;elastosis.

PMID:34504372 | PMC:PMC7654483 | DOI:10.31729/jnma.5186

A Huge Plantar Intramuscular Hemangioma in the Plantar Area Treated Surgically: A Case Report and Literature Review

Fetched: September 12th, 2021, 5:01am GMT by Hong Seop Lee

Int J Environ Res Public Health. 2021 Aug 28;18(17):9088. doi: 10.3390/ijerph18179088.

ABSTRACT

Intramuscular hemangioma (IH) is rare, accounting for only 0.8% of all hemangioma cases. In particular, IH of the foot has only been reported a few times. In such cases, the symptoms typically include tenderness and swelling, often in relation to physical activity, but tingling or impaired function may also be present. Here, we report a patient who presented with a significant IH in the plantar area treated surgically. A 25-year-old female visited our hospital with pain in the plantar aspect of the right foot. She had noticed a mass about 10 years prior. She had previously experienced pain only when pressing the mass, but the pain subsequently became more regular pain and was exacerbated by exercise. In fact, the pain became so intense that she could not sleep well. Upon physical examination, mild swelling and tenderness of the plantar area were noted in the second to the fourth metatarsal. Sensation and motor reflexes were normal and the results of Tinel's test were negative. Plain radiographs of the right foot revealed phleboliths scattered throughout the first to third intermetatarsal spaces. Magnetic resonance imaging revealed a space-occupying multilobulated mass (5.6 × 2.8 × 2.5 cm) located in the flexor digitorum brevis (FDB) muscle, which penetrated the plantar fascia and spread to the subcutaneous layer. In T2-weighted images, the lesion displayed a hyperintense signal compared to the surrounding skeletal muscle. Based on radiological findings, we suspected IH. The mass surrounded by the FDB muscle was exposed and completely removed via wide excision. IH consisting of cavernous-like vascular structures was diagnosed on pathology. At 1-year follow-up, the patient was almost asymptomatic and had recovered almost full range of motion in the plantar area. Histological analysis and surgery are recommended to remove intramuscular hemangiomas in the plantar area, but if the patient is not suitable for surgery, sclerotherapy or combination treatment should also be considered.

PMID:34501676 | PMC:PMC8431436 | DOI:10.3390/ijerph18179088

Pulmonary sclerosing pneumocytoma with spindle cell characteristics and lymph node metastasis: report of a case

Fetched: September 12th, 2021, 5:01am GMT by R Q Wang

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1067-1069. doi: 10.3760/cma.j.cn112151-20201230-00988.

NO ABSTRACT

PMID:34496505 | DOI:10.3760/cma.j.cn112151-20201230-00988

Endometrial cavernous hemangiomatous polyp: report of a case

Fetched: September 12th, 2021, 5:01am GMT by S G Zhou

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1061-1063. doi: 10.3760/cma.j.cn112151-20210319-00215.

NO ABSTRACT

PMID:34496503 | DOI:10.3760/cma.j.cn112151-20210319-00215

Pitfalls in the management of subglottic paragangliomas at unusual location: a case report and literature review

Fetched: September 10th, 2021, 5:01am GMT by Juanjuan Hu

BMC Surg. 2021 Sep 8;21(1):340. doi: 10.1186/s12893-021-01337-6.

ABSTRACT

BACKGROUND: Subglottic paragangliomas (PGs) are exceptionally rare and unpredictable, occasionally presenting at an atypical location. There are three different clinical forms of subglottic PGs: intraluminal (tracheal PGs), extraluminal (thyroid PGs) and the mixed type (both intraluminal and extraluminal, mixed-subglottic PGs). These tumors are usually misdiagnosed as other relatively common primary thyroid or laryngotracheal tumors, and the treatment is troublesome.

CASE PRESENTATION: A 22-year-old male patient with subglottic PGs has been successively misdiagnosed as thyroid tumors and subglottic hemangiomas, and lastly underwent local extended lumpectomy and laryngotracheal reconstruction with a pedicled thoracoacromial artery perforator flap (PTAPF). The patient was decannulated successfully after the second-stage tracheal reconstruction with a local flap, and no evidence of local recurrence and distant metastasis of the tumor until now.

CONCLUSION: Subglottic PGs can be easily misdiagnosed as laryngotracheal or thyroid tumors when presented at an atypical location. It is essential for otolaryngologists and head and neck surgeons to remain vigilant against these tumors. If the tumor is not diagnosed or removed completely, patients may encounter a risk of lethal paroxysm, which is incredibly troublesome.

PMID:34496808 | PMC:PMC8424930 | DOI:10.1186/s12893-021-01337-6

Pediatric vascular tumors of liver:a clinicopathological study of 22 cases

Fetched: September 10th, 2021, 5:01am GMT by L Yuan

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1029-1033. doi: 10.3760/cma.j.cn112151-20201231-00993.

ABSTRACT

Objective: To investigate the clinicopathological features of hepatic vascular tumors in children. Methods The clinical characteristics, histology and immunohistochemical staining results were summarized and analyzed in 22 cases of hepatic vascular tumors in children at Guangzhou Women and Children's Medical Center from September 2007 to November 2020. Results: The 22 patients aged from 1.0 month to 2.5 years (mean age 9 months). There were 10 males and 12 females. Five cases were found in premature and had low birth weight infants; three cases were discovered in the antenatal period; one patient also had cutanous hemangioma; six patients had associated anemia; Kasabach-Merritt phenomenon was not seen in any patient. CT examination showed 17 tumors were solitary and five were multifocal lesions. Macroscopically, the tumors size ranged from was 0.6 cm to 11.0 cm; the cut surface was solid, gray red and brown in color, and in six cases there were hemorrhage and necrosis in the central area. Microscopically,15 cases of solitary congenital hepatic hemangiomas showed characteristic necrosis in the central area, with loose fibrous tissues at periphery. Proliferation of capillaries, residual bile ducts between the vascular lumens, and dilated thrombosed vascular channels were seen, and contained extramedullary hematopoietic foci and calcification. Five cases of multiple hepatic infantile hemangiomas showed capillaries of different sizes composing of plump endothelium and pericytes and were arranged in lobular or diffuse patterns. Two cases of cavernous hemangioma (venous malformation) consisted of dilated thin-walled blood vessels with branch-like pattern lined with flat endothelial cells. Immunohistochemically, all 22 case expressed vascular endothelial markers CD31 and CD34, but D2-40 was negative. Glut1 was positive in five cases of multiple hepatic infantile hemangiomas, and the other cases were negative. Conclusion: Hepatic vascular tumors in children are rare, and their classification is different from that of adults. It is of great significance to make clear pathologic diagnosis.

PMID:34496494 | DOI:10.3760/cma.j.cn112151-20201231-00993

Giant Hepatic Hemangioma Regressed Significantly Without Surgical Management: A Case Report and Literature Review

Fetched: September 8th, 2021, 5:01am GMT by Jingcong Zhang

Front Med (Lausanne). 2021 Aug 19;8:712324. doi: 10.3389/fmed.2021.712324. eCollection 2021.

ABSTRACT

Hepatic hemangioma (HH) is a congenital vascular anomaly comprising networks of abnormal blood and/or lymphatic vessels with endothelial cell proliferation. Their pathophysiology is not fully understood, and no specific drug is available to treat them. Conservative management, which limits observation, is preferred for most patients. A HH larger than 4 cm is considered a giant HH that may be treated using surgery ranging from embolization to hepatic resection or liver transplantation. Here, we describe a case with multiple and giant HHs that regressed significantly after treatment with azithromycin (AZM). A systematic literature review of HH and the effects of AZM on angiogenesis was then conducted.

PMID:34490301 | PMC:PMC8416894 | DOI:10.3389/fmed.2021.712324

Permalink for 'Postoperative liver metastasis of primary inflammatory pseudotumorous follicular dendritic cell sarcoma of the spleen: a case report'

Postoperative liver metastasis of primary inflammatory pseudotumorous follicular dendritic cell sarcoma of the spleen: a case report

Fetched: September 8th, 2021, 5:01am GMT by Run-Hua Pang

Ann Palliat Med. 2021 Aug;10(8):9297-9303. doi: 10.21037/apm-21-2155.

ABSTRACT

Follicular dendritic cell sarcoma (FDCS) is a rare and low incidence tumor. So far, there is no standard treatment for the disease. Surgery is the main treatment for FDCS. Here, we report the case of a 51-year-old woman who was admitted with the chief complaint of "spleen-occupying lesion detected via physical examination more than 1 month ago". Spiral plain scan and enhanced computed tomography (CT) of the upper abdomen showed a vascular-derived tumor, with a high possibility of hemangioma or hemangiolymphangioma. Later, on 25 December 2019, the patient underwent laparoscopic splenectomy, and the pathological diagnosis of primary splenic FDCS was made. The patient did not receive adjuvant chemotherapy or radiotherapy. At a regular follow-up inspection 11 months later, on 18 December 2020, an abdominal scan + enhanced CT revealed multiple new abnormal intrahepatic nodules, which combined with her history, indicated the possibility of metastases. Subsequently, she was readmitted to hospital and surgical treatment was decided upon after multi-disciplinary consultation and discussion. Laparoscopic S1/S3/S4/S5 hepatectomy plus cholecystectomy was performed on 31 December 2020. The postoperative pathology findings revealed (liver tumor, S3, S4, S5) metastatic inflammatory pseudotumor follicular dendritic sarcoma, tumor diameter 0.6-1.2 cm. Due to the lack of clinical reports on postoperative organ metastasis of this disease, less experience, and controversy in drug selection of radiotherapy and chemotherapy, the patient refused to receive radiotherapy and chemotherapy. She now undertakes regular outpatient reexamination, and has been followed-up until now, during which time she has not progressed, and the efficacy is considered satisfactory.

PMID:34488416 | DOI:10.21037/apm-21-2155

$Permalink for 'The efficacy and safety of low-energy carbon dioxide fractional laser use in the treatment of early-stage pediatric hypertrophic scars: A prospective, randomized, split-scar study'$

The efficacy and safety of low-energy carbon dioxide fractional laser use in the treatment of early-stage pediatric hypertrophic scars: A prospective, randomized, split-scar study

Fetched: September 8th, 2021, 5:01am GMT by TaeHo Won

Lasers Surg Med. 2021 Sep 6. doi: 10.1002/lsm.23459. Online ahead of print.

ABSTRACT

BACKGROUND: Various laser therapies have been introduced in scar management. However, pain during treatment has limited the application of laser therapy in pediatrics.

OBJECTIVES: To evaluate whether the use of the low-energy mode of a carbon dioxide (CO2 ) laser improves hypertrophic scars in a pediatric population.

METHODS: This prospective, randomized, split-scar trial was designed to assess the safety and efficacy of low-energy CO2 laser use. Patients aged <12 years with hypertrophic scars were enrolled. Each hypertrophic scar was equally divided into three parts: the two ends of each scar were randomly assigned to control and experimental groups, and the center portion was considered a transition zone and was not included in the analysis. A total of three laser treatments were performed at 1-month intervals. Scar scale scores 6 months after the final treatment was the primary outcome. Additionally, the Visual Analog Scale (VAS) was used to evaluate pain after each treatment.

RESULTS: Of the 23 patients enrolled, 20 completed the study. The total Patient and Observer Scar Assessment Scale (POSAS) score at the 6-month follow-up was significantly lower for the treated site (44.95 for the treated group vs. 64.85 for the control group, p < 0.0001). Both the patient and observer POSAS scores showed an obvious difference between the treated and control groups (19.95 vs. 29.95 for patient scores, respectively, p < 0.0001, and 26.00 vs. 34.90 for observer scores, respectively, p < 0.0001). All observer and patient scores describing pain, pruritus, color, stiffness, and thickness were statistically different and favored the treated site. No significant difference was found in patient score of irregularity. The average VAS therapeutic pain score was 3.5 ± 1.43 out of 10.

CONCLUSIONS: Low-energy CO2 fractional laser therapy improved hypertrophic scars in a pediatric population. Therefore, for children with hypertrophic scar, low-energy CO2 laser with less procedure pain may be more appropriate.

PMID:34487566 | DOI:10.1002/lsm.23459

Permalink for 'Differentiation of Benign From Malignant Sinonasal Masses Using Diffusion Weighted Imaging and Dynamic Contrast Enhanced Magnetic Resonance Imaging'

Differentiation of Benign From Malignant Sinonasal Masses Using Diffusion Weighted Imaging and Dynamic Contrast Enhanced Magnetic Resonance Imaging

Fetched: September 8th, 2021, 5:01am GMT by Radhika Daga

Am J Rhinol Allergy. 2021 Sep 5:19458924211040602. doi: 10.1177/19458924211040602. Online ahead of print.

ABSTRACT

BACKGROUND: The sinonasal region is affected by a variety of neoplasms. A differentiation between benign and malignant masses is essential both for management and prognostication. Morphological analysis often does not allow this differentiation.

OBJECTIVES: This article aims to assess the value of advanced MRI (diffusion [DWI] and dynamic contrast enhanced MRI [DCE-MRI]) in differentiation of benign and malignant sinonasal masses.

METHODS: This prospective study included 40 patients with sinonasal masses who underwent advanced MR on 3T MR scanner. The lesions were analyzed based on morphological characteristics, qualitative, quantitative diffusion parameters, and time signal intensity curves. Apparent diffusion coefficient (ADC) values were acquired using b values of 50 and 1000 s/mm². The accuracy of DWI, DCE-MRI, and combined DWI/DCE-MRI in differentiating benign from malignant sinonasal masses were analyzed.

RESULTS: Perineural extension and growth pattern of the tumor were the best morphological discriminators. Mean ADC values for benign and malignant lesions were 1.675 ± 0.561 and 0.903 ± 0.405 × 10^-3 mm²/sec, ,respectively. ROC revealed that ADC cutoff value of 1.005 × 10^-3 mm²/sec provided an accuracy of 92.5% in differentiating benign from malignant masses (P value <.01). On excluding the benign vascular masses (Juvenile Nasopharyngeal Angiofibroma and hemangioma), the time signal intensity curve showed 78% accuracy (P value <.001). The highest diagnostic performance was achieved by combining DWI and DCE-MRI (95% accuracy).

CONCLUSION: DWI has higher accuracy than DCE-MRI. Quantitative DWI is preferable over qualitative DWI. Accuracy of DCE-MRI can be increased by excluding vascular masses with characteristic imaging features. DWI and DCE-MRI have the highest accuracy when used in combination than either of them alone in differentiating benign from malignant sinonasal masses.

PMID:34486401 | DOI:10.1177/19458924211040602

Permalink for 'Diaphyseal and Metaphyseal Modeling Defects-Clinical Findings and Identification of WRAP53 Deficiency in Craniometadiaphyseal Dysplasia'

Diaphyseal and Metaphyseal Modeling Defects-Clinical Findings and Identification of WRAP53 Deficiency in Craniometadiaphyseal Dysplasia

Fetched: September 8th, 2021, 5:01am GMT by Yun Hao

Front Genet. 2021 Aug 12;12:684905. doi: 10.3389/fgene.2021.684905. eCollection 2021.

ABSTRACT

Background: Diaphyseal and metaphyseal modeling defects lead to severe changes in bone mass and shape, which are common features in osteoporosis that linked to non-vertebral fractures. Original mechanism of diaphyseal and metaphyseal modeling defects has proved elusive. Studying rare syndromes can elucidate mechanisms of common disorders and identify potential therapeutic targets. Methods: We evaluated a family pedigree with craniometadiaphyseal dysplasia (CRMDD, OMIM 269300), a genetic disorder that is characterized by cortical-bone thinning, limb deformity, and absent of normal metaphyseal flaring and diaphyseal constriction. Systemic radiographic examination and serum hormone test were made for this rare disease. One patient and her two normal parents were examined by means of whole-exome sequencing (WES) to identify the candidate pathogenic gene and rule out mucopolysaccharidosis and Prader-Willi Syndrome by means of Sanger sequencing. Results: There are several conspicuous radiographic characteristics: (1) bullet-shaped phalanges, (2) long and narrow pelvic inlet, absent of supra-acetabular constriction, (3) round rod-shaped long tubular bones, (4) prominent aiploic mastoid, (5) bending-shaped limb, genua varus and genu varum, and (6) congenital dislocation of elbow. Here, we did not find any wormian bones, and there are several typical clinical characteristics: (1) macrocephaly and wide jaw, (2) Avatar elf-shaped ears, pointed and protruding ears, (3) hypertrophy of limbs, (4) flat feet and giant hand phenomenon, (5) nail dystrophy, (6) limb deformity, (7) high-arched palate, (8) superficial hemangiomas, (9) tall stature, and intellectual disability. In this patient, we found biallelic frameshift deletion mutations in WRAP53, and those two mutations were transmitted from her parents respectively. Conclusions: We describe her clinical and radiological findings and presented a new subtype without wormian bones and with a tall stature. Our study showed that craniometadiaphyseal dysplasia was caused by a deficiency of WRAP53 with autosomal recessive inheritance.

PMID:34484289 | PMC:PMC8416243 | DOI:10.3389/fgene.2021.684905

Littoral cell angioma of the spleen. Report of one case

Fetched: September 8th, 2021, 5:01am GMT by María J Irarrázaval

Rev Med Chil. 2021 Apr;149(4):626-629. doi: 10.4067/s0034-98872021000400626.

ABSTRACT

Splenic vascular neoplasms are the most common form of spleen tumors. Among them, littoral cell angioma is rare and it is frequently an incidental finding in imaging studies. It has no specific clinical, laboratory or imaging findings. Splenectomy allows definitive diagnosis throughout a histopathological examination. We report a 52-year-old man presenting with asthenia and abdominal distension. Computed tomography with intravenous contrast showed multiple splenic hypodense masses and a prostatic enlargement. Presuming a lymphoma, a laparoscopic splenectomy was performed. Histopathologic examination diagnosed littoral cell angioma. During urological follow-up, a prostate adenocarcinoma was diagnosed.

PMID:34479351 | DOI:10.4067/s0034-98872021000400626

Hemangiomatous neck plaques in a child with cleft lip

Fetched: September 8th, 2021, 5:01am GMT by Katherine I Jicha

Pediatr Dermatol. 2021 Jul;38(4):929-931. doi: 10.1111/pde.14541.

NO ABSTRACT

PMID:34448228 | DOI:10.1111/pde.14541

Kaposiform hemangioendothelioma in an adult - lack of response to topical sirolimus and response to radiotherapy

Fetched: September 5th, 2021, 5:01am GMT by S A Der Sarkissian

Clin Exp Dermatol. 2021 Sep 4. doi: 10.1111/ced.14928. Online ahead of print.

ABSTRACT

A 65-year-old male presented with a twelve month history of a tender plaque on his neck. His past medical history was remarkable for cirrhosis, chronic kidney disease, thrombocytopenia, cognitive impairment and bipolar affective disorder. Examination demonstrated a poorly demarcated firm burgundy plaque over a 6cm x 5cm area (Figure 1).

PMID:34480811 | DOI:10.1111/ced.14928

Treatment of a patient with giant solitary fibrous tumor of the left hip

Fetched: September 5th, 2021, 5:01am GMT by E A Drobyazgin

Khirurgiia (Mosk). 2021;(9):93-99. doi: 10.17116/hirurgia202109193.

ABSTRACT

The authors report surgical treatment of a 59-year-old female with a giant solitary-fibrous tumor of the left hip. Previous excision of hip hemangioma was performed in 2016. Recurrent tumor with fast growth has occurred since 2018. The neoplasm consisted of 2 tumors connected by vascular structures. CT revealed soft tissue neoplasms in the upper third of the left thigh (anterior, medial and dorsal regions). Large tumors with an isthmus along the inner surface had tuberous contours and internal septa. The last ones and walls accumulated contrast agent. No structural damage to femoral muscles was observed. The patient underwent resection of tumor and vascular ligation (great saphenous vein and its tributaries along the anterior surface, arteries from the deep femoral artery system along the posterior surface of tumor). There were no early postoperative complications. There are no complaints within 6 month after surgery. There is no lower limb dysfunction.

PMID:34480461 | DOI:10.17116/hirurgia202109193

International survey on the management of lumbosacral cutaneous stigmata in infants with suspected occult spinal dysraphism

Fetched: September 5th, 2021, 5:01am GMT by Konstantina Svokos

J Neurosurg Pediatr. 2021 Sep 3:1-8. doi: 10.3171/2021.5.PEDS2126. Online ahead of print.

ABSTRACT

OBJECTIVE: Occult spinal dysraphism (OSD) is a common pediatric neurosurgical diagnosis rife with controversy surrounding both the screening of asymptomatic infants and the threshold to offer a prophylactic detethering operation. The authors sought to clarify international practice patterns with a survey of pediatric neurosurgeons.

METHODS: A survey asked pediatric neurosurgeons whether they would perform imaging in patients with a variety of cutaneous stigmata associated with OSD and whether they would offer prophylactic detethering surgery for asymptomatic patients with a variety of imaging findings on the OSD spectrum.

RESULTS: Completed surveys were received from 141 pediatric neurosurgeons. Broad consensus was demonstrated on the need for obtaining images in sample patients with more severe stigmata ranging from large lipoma with a skin appendage to focal dysplastic skin in the lumbar midline. Ninety percent of respondents would perform MRI for these patients. In contrast, for patients with a low-sacral dimple, flat hemangioma, and symmetric (Y-shaped) splaying of the intergluteal cleft, opinion on the need for imaging varied considerably (between 57% and 89% recommended imaging). Respondents differed on the type of imaging that they would perform, with 31% to 38% recommending ultrasound screening. The responses reflected less consensus on when to offer surgery to patients with simple spinal tethering (low-lying conus medullaris and fatty filum terminale). Both a lower level of the conus and increased thickness of the filum terminale affected decision-making.

CONCLUSIONS: The results of this survey showed significant consensus on the recommendation for screening imaging in patients with more dramatic cutaneous stigmata, although these stigmata are the rarest. A significant variance in opinions was reflected in the recommendation for imaging of the most common cutaneous stigmata. Consensus was also lacking on which lesions deserve prophylactic detethering surgery. Significant equipoise exists for future study of screening imaging and of surgical decision-making in patients with asymptomatic OSD and associated cutaneous stigmata.

PMID:34479200 | DOI:10.3171/2021.5.PEDS2126

Vascular endothelial growth factor response with propranolol therapy in patients with infantile hemangioma

Fetched: September 4th, 2021, 5:01am GMT by S M Makkeyah

Pediatr Hematol Oncol. 2021 Sep 3:1-10. doi: 10.1080/08880018.2021.1961956. Online ahead of print.

ABSTRACT

Vascular endothelial growth factor-A (VEGF-A) is a master regulator of angiogenesis, with higher levels in infantile hemangioma (IH). The effects of propranolol on IH are not fully understood and may involve vasoconstriction, angiogenesis inhibition, and apoptosis induction. Therefore, we examined the effects of propranolol therapy on levels of VEGF-A in patients with IH in the proliferative phase and compared the VEGF-A levels to those in untreated patients in the involuting or involuted phases, as well as studied the consistency between the clinical and VEGF responses in patients receiving treatment. In a prospective study, we compared 24 patients with IH in the proliferative phase to 9 patients with IH in the involuting or involuted phase, assessing clinical responses to therapy and changes in VEGF-A levels after 3 months. The median VEGF level before treatment was 275 pg/ml; however, after 3 months, the level significantly decreased to 100 pg/ml (P = 0.007). Median VEGF was significantly higher in patients in the proliferative phase after 3 months of treatment (100 pg/ml) as compared to those in the involuting phase (50 pg/ml). We found no significant correlation between VEGF level and IH size reduction. Propranolol therapy induced a significant decline in VEGF levels at the 3-month evaluation in patients in the proliferative phase; however, this did not reach the levels of IH in the involuting phase. VEGF response was not translated to a clinical response in some patients with IH. These results put in uncertainty the clinical benefit of targeting VEGF pathway in IH.

PMID:34477031 | DOI:10.1080/08880018.2021.1961956

A hemangioma in the masseter muscle: a case report

Fetched: September 4th, 2021, 5:01am GMT by Daehwan Park

Arch Craniofac Surg. 2021 Aug;22(4):218-221. doi: 10.7181/acfs.2021.00283. Epub 2021 Aug 20.

ABSTRACT

Intramuscular hemangiomas of the masseter muscle are uncommon tumors and therefore can be difficult to accurately diagnose preoperatively, due to the unfamiliar presentation and deep location in the lateral face. A case of intramuscular hemangioma of the masseter muscle in a 66-yearold woman is presented. Doppler ultrasonography showed a 34× 15 mm hypoechoic and hypervascular soft tissue mass in the left masseter muscle, suggesting hemangioma. The mass was excised via a lateral cervical incision near the posterior border of the mandibular ramus. The surgical wound healed well without complications.

PMID:34474547 | PMC:PMC8413925 | DOI:10.7181/acfs.2021.00283

A national audit of oral propranolol for the treatment of Infantile Hemangiomas

Fetched: September 3rd, 2021, 5:01am GMT by C Cotter

Br J Dermatol. 2021 Sep 2. doi: 10.1111/bjd.20738. Online ahead of print.

ABSTRACT

Infantile haemangiomas (IH) affect up to 4% of infants and are the most common tumour of infancy. Although self-limiting, some lesions can lead to visual impairment, airway obstruction, ulceration or cosmetic disfigurement and require intervention. Oral propranolol, a nonselective beta-blocker was serendipitously identified as an effective treatment of IH in 2008 and is now recommended as first-line therapy for complicated lesions.

PMID:34473344 | DOI:10.1111/bjd.20738

A rare case of sclerosing hemangioma of the lung with a review of literature

Fetched: September 3rd, 2021, 5:01am GMT by Laxmi Niwas Tiwari

Lung India. 2021 Sep-Oct;38(5):474-476. doi: 10.4103/lungindia.lungindia_625_20.

ABSTRACT

A rare case of sclerosing hemangioma (SH) of the lung is described in a 23-year-old gentleman, who presented with cough with expectoration, breathlessness, and left-sided chest pain with recurrence. We present a brief review of SH, an uncommon but histologically distinctive neoplasm of the lung. SH of the lung is generally considered to be a benign lesion, and surgical excision is curative without the need for additional treatment.

PMID:34472527 | DOI:10.4103/lungindia.lungindia_625_20

Infantile Hemangioma Treated with Oral Propranolol: Case Presentation

Fetched: September 3rd, 2021, 5:01am GMT by Nahid Raufi

Clin Cosmet Investig Dermatol. 2021 Aug 25;14:1053-1055. doi: 10.2147/CCID.S326794. eCollection 2021.

ABSTRACT

Infantile hemangiomas (IHs) are the most common vascular tumors in childhood. We report the use of propranolol to treat the growth phase of IHs. Propranolol was given to a 6-month-old infant who presented with a 4-cm sharply demarcated hemangioma on his left gluteal region. After relevant evaluation, propranolol was prescribed with a starting dose of 1 mg/kg/day, given in 3 divided doses. Vital signs were monitored during the first 6 hours of treatment. In the absence of side effects, treatment was continued at home with 2 mg/kg/day for six months, and the child was reevaluated after 7 days of treatment and then monthly. After one month, effects on color and growth were noted. Complete healing occurred in less than 6 months. Side effects and relapse were not reported. Propranolol administered orally at 2 mg/kg/day was successful, leading to near resolution of the patient's hemangioma.

PMID:34471369 | PMC:PMC8403557 | DOI:10.2147/CCID.S326794

Measuring the impact of clinical practice guidelines on infantile hemangioma referrals

Fetched: September 2nd, 2021, 5:01am GMT by Michael T Barton

Pediatr Dermatol. 2021 Aug 31. doi: 10.1111/pde.14783. Online ahead of print.

ABSTRACT

High-risk infantile hemangiomas may be associated with significant patient comorbidity. The American Academy of Pediatrics published clinical practice guidelines with recommendations to refer high-risk hemangiomas early. The results of this study suggest that these guidelines may have resulted in an earlier referral age of patients with high-risk IH to hemangioma specialists.

PMID:34467544 | DOI:10.1111/pde.14783

A case report of mediastinal cavernous hemangioma

Fetched: September 2nd, 2021, 5:01am GMT by Davoud Ouladdameshghi

Clin Case Rep. 2021 Aug 25;9(8):e04698. doi: 10.1002/ccr3.4698. eCollection 2021 Aug.

ABSTRACT

When faced with a hypervascular mediastinal tumor, mediastinal hemangioma should be taken into consideration. Although it is uncommon, considering this important diagnosis may avoid a possible extensive surgery that is not necessary.

PMID:34466254 | PMC:PMC8385463 | DOI:10.1002/ccr3.4698

Adverse Drug Reactions Following Propranolol in Infantile Hemangioma

Fetched: September 2nd, 2021, 5:01am GMT by Vaibhav Pandey

Indian Pediatr. 2021 Aug 15;58(8):753-755.

ABSTRACT

OBJECTIVES: To assess the adverse effects of propranolol therapy in infantile hemangioma.

METHODS: An ambispective study was conducted from August 2011 to December 2019. In retrospective arm all children managed for infantile hemangioma with propranolol were included and case records were assessed for adverse reactions. In prospective arm the adverse reactions were identified on the basis of predefined criteria.

RESULTS: A total of 514 patients (358 retrospective records) were included. A majority, 378 (73.5%) patients had an excellent response, 75 (14.5%) had partial response and 61 (11.8 %) had no response. A total of 82 (15.9%) patients experienced at least one adverse effect. Diarrhea with weight loss (27, 32.9%) and irritability with decreased sleep (21, 25.6%) were the most common adverse effects. The adverse effects in 22 (4.2%) cases lead to the discontinuation of propranolol. Younger age, low body weight and early onset were risk factors for development of severe adverse reactions.

CONCLUSIONS: Young children with low body weight were at higher risk for adverse effects of propranolol.

PMID:34465658

Permalink for 'Nonselective Beta-Blockers for the Efficacious Healing of Ulcerated Infantile Hemangiomas in Unusual Locations of Two Female Infants'

Nonselective Beta-Blockers for the Efficacious Healing of Ulcerated Infantile Hemangiomas in Unusual Locations of Two Female Infants

Fetched: September 1st, 2021, 5:01am GMT by Divya Poulose

Cureus. 2021 Jul 28;13(7):e16683. doi: 10.7759/cureus.16683. eCollection 2021 Jul.

ABSTRACT

Infantile hemangiomas (IHs) are the most common vascular soft-tissue tumors of newborns and early childhood with a prevalence rate of approximately 4%-5% in infancy. Most of the IHs regress spontaneously after initial proliferation. Ulceration is one of the most common complications seen in IHs, which heal poorly without treatment. The prevalence of IHs is three times more in females compared to males. The most common location of IH is head and neck with the least being in extremities Oral propranolol and systemic corticosteroids are used as first-line therapy in the treatment. Topical timolol has been used as an alternative to counteract the long-term side effects of the prior modalities. In this study, we are reporting and presenting case reports of two female infants with ulcerated IH who were treated with oral propranolol and topical timolol, respectively, and comparing the efficacy of one over the other. we observed fast and efficacious healing with oral propranolol when used with proper monitoring, as compared to topical timolol, although the latter is more commonly used as it is safer with fewer potential side effects.

PMID:34462702 | PMC:PMC8389862 | DOI:10.7759/cureus.16683

Diagnostic challenge and management of intraosseous mandibular hemangiomas: a case report and literature review

Fetched: September 1st, 2021, 5:01am GMT by Maria Isabel Sánchez Jorge

J Korean Assoc Oral Maxillofac Surg. 2021 Aug 31;47(4):321-326. doi: 10.5125/jkaoms.2021.47.4.321.

ABSTRACT

Hemangioma is a benign tumor characterized by the proliferation of blood vessels. Although it often appears in soft tissues, its occurrence in bone tissue, particularly the mandible, is extremely rare. A 32-year-old female sought attention at the dental clinic complaining of a painless swelling in the posterior region of the left side of the mandible. A panoramic radiograph and computed axial tomography scan were taken, showing honeycomb and sunburst images, respectively, in the affected area. The patient underwent a biopsy, which led to the diagnosis of intraosseous hemangioma. Having assessed the characteristics of the lesion, it was decided to perform complete excision including safety margins, followed by an iliac crest bone graft to reconstruct the mandible. Awareness of the possible clinical and radiographic presentations of intraosseous hemangioma is considered important, as non-diagnosis could have severe consequences given its possible relation to dental structures.

PMID:34462389 | PMC:PMC8408642 | DOI:10.5125/jkaoms.2021.47.4.321

Childhood Vascular Tumors Treatment (PDQ®): Health Professional Version

Fetched: September 1st, 2021, 5:01am GMT by PDQ Pediatric Treatment Editorial Board

2021 Aug 27. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002–.

ABSTRACT

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood vascular tumors. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

PMID:26844334 | Bookshelf:NBK343452

Cavernous Malformations of the Optic Nerve and Optic Pathway: A Case Series and Systematic Review of the Literature

Fetched: August 31st, 2021, 5:01am GMT by Visish M Srinivasan

Oper Neurosurg (Hagerstown). 2021 Aug 30:opab284. doi: 10.1093/ons/opab284. Online ahead of print.

ABSTRACT

BACKGROUND: Although rare, cavernous malformations (CMs) of the optic nerve and anterior optic pathway (optic pathway cavernous malformations [OPCMs]) can occur, as described in several single case reports in the literature.

OBJECTIVE: To describe the technical aspects of microsurgical management of CMs of the optic pathway on the basis of an extensive single-center experience and review of the literature.

METHODS: A systematic literature review was performed to augment an earlier review, using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. In addition, an institutional database was searched for all patients undergoing surgical resection of OPCMs. Patient information, surgical technique, and clinical and radiographic outcomes were assessed.

RESULTS: Since the previous report, 14 CMs were resected at this institution or by the senior author at another institution. In addition, 34 cases were identified in the literature since the systematic review in 2015, including some earlier cases that were not discussed in the previous report. Most OPCMs were resected via pterional, orbital-pterional, and orbitozygomatic craniotomies. Visual outcomes were similar to those in earlier reports, with 70% of patients reporting stable to normal vision postoperatively.

CONCLUSION: OPCMs can occur throughout the anterior visual pathway and may cause significant symptoms. Surgery is feasible and should be considered for OPCMs presenting to a surface of the nerve. Favorable results can be obtained with resection, although optimal results are obtained with patients who present with milder symptoms without longstanding damage to the optic apparatus.

PMID:34460919 | DOI:10.1093/ons/opab284

Mediastinal and subglottic hemangioma in an infant: a case report and literature review

Fetched: August 31st, 2021, 5:01am GMT by Qiuyu Lin

J Int Med Res. 2021 Aug;49(8):3000605211039803. doi: 10.1177/03000605211039803.

ABSTRACT

We performed a retrospective analysis of the clinical manifestations, laboratory and imaging examinations, treatment, and prognosis of a male infant who was diagnosed with mediastinal and subglottic hemangioma in our hospital. The clinical features of this patient were coughing, wheezing, and dyspnea. Enhanced computed tomography of the neck and chest showed a diffuse abnormality in the right-upper mediastinum. He was diagnosed with a hemangioma after a physical examination combined with bronchoscopy. The clinical symptoms were relieved by oral propranolol. We also investigated the clinical characteristics, treatment, and prognosis of mediastinal and subglottic hemangioma in infants in the previous literature, and searched for case reports of this disease in China and in other countries. We only identified three previous cases of mediastinal and subglottic hemangioma in infants, indicating that this condition is rare. In the proliferative stage, surrounding organs and tissues are compressed, which can be life-threatening. Most of these children develop wheezing, shortness of breath, dyspnea, cyanosis, and other symptoms within 2 months. Enhanced computed tomography and magnetic resonance imaging combined with soft bronchoscopy can confirm the diagnosis of this disease, and oral propranolol achieves a favorable effect.

PMID:34459273 | PMC:PMC8408903 | DOI:10.1177/03000605211039803

Malignant Tumors Misdiagnosed as Liver Hemangiomas

Fetched: August 31st, 2021, 5:01am GMT by Murat Baki Yıldırım

Front Surg. 2021 Aug 11;8:715429. doi: 10.3389/fsurg.2021.715429. eCollection 2021.

ABSTRACT

Background and Aim: To derive lessons from the data of patients who were followed for various periods with the misdiagnosis of liver hemangioma and eventually found to have a malignancy. Material and Methods: The records of 23 patients treated between 2003 and 2018 were analyzed retrospectively. Results: Twelve patients were men and 11 were women; median (range) age was 55 (35-80). The principal diagnostic modality for the initial diagnosis was ultrasonography (n:8), magnetic resonance imaging (MRI) (n:13), and computed tomography (CT) (n:2). At our institution, MRI was performed in 16 patients; the diagnosis was made with the available MRI and CT studies in five and two patients, respectively. In other words, the ultrasonography interpretations were not confirmed on MRI; in others, the MRI or CT examinations were of low quality or they had not been interpreted properly. Fifteen patients underwent surgery; the other patients received chemotherapy (n:6) or chemoembolization (n:2). The misdiagnosis caused a median (range) 10 (0-96) months delay in treatment. The final diagnoses were hepatocellular carcinoma in 12 patients, cholangiocarcinoma in four patients, metastatic mesenchymal tumor, metastasis of colon cancer, metastatic neuroendocrine carcinoma, sarcomatoid hepatocellular carcinoma, angiosarcoma, thoracic wall tumor, and metastatic tumor of unknown primary in one patient each. Conclusions: High-quality MRI with proper interpretation and judicious follow up are vital for the accurate differential diagnosis of liver lesions.

PMID:34458317 | PMC:PMC8384961 | DOI:10.3389/fsurg.2021.715429

Surgical management of giant hepatic hemangioma: A 10-year single center experience

Fetched: August 31st, 2021, 5:01am GMT by Waad Farhat

Ann Med Surg (Lond). 2021 Jul 6;69:102542. doi: 10.1016/j.amsu.2021.102542. eCollection 2021 Sep.

ABSTRACT

BACKGROUND: Hepatic hemangiomas are the most typical benign mesenchymal lesions of the liver. Most of these lesions are asymptomatic. Giant hepatic hemangiomas (GHH) (>10 cm) are often symptomatic and require surgical intervention. This study aimed to describe the clinical findings, risk factors, diagnostic approach and management of GHH.

METHODS: We performed a retrospective analysis of patients with GHH treated at our hospital from January 2008 to December 2018. The medical records of each patient were reviewed to obtain the clinical and surgical data.

RESULTS: Twelve patients with GHH were treated during the study period. 9 were female and 3 were male. The mean age of diagnosis was 48,2 years. The most common presenting symptom was abdominal pain. Eight patients presented an abdominal mass. Indications for surgical resections were rupture (n = 2), Kasabach-Merritt syndrome (n = 1) and abdominal pain (n = 9). Right hepatectomy was done in four patients, left lobectomy in four patients, and enucleation in four patients. Embolization was performed in 4 patients, but due to the persistence of symptoms or bleeding, surgery was indicated. The mean operative time was 3.5 h, and median blood loss was 870 ml. The median hospital stay was 5.3 days. For four patients, we registered postoperative complications causing death in one case. All alive patients were asymptomatic at a median follow-up of 55 months.

CONCLUSION: Despite limitations and alternative modalities, surgery remains the only effective curative treatment for GHH.

PMID:34457247 | PMC:PMC8379432 | DOI:10.1016/j.amsu.2021.102542

An epithelioid haemangioma of the ulnar artery masquerading as an aneurysm

Fetched: August 29th, 2021, 5:01am GMT by Thilina Gunawardena

Ann Vasc Surg. 2021 Aug 24:S0890-5096(21)00518-5. doi: 10.1016/j.avsg.2021.05.040. Online ahead of print.

ABSTRACT

Epithelioid haemangiomas are rare benign vascular tumors that usually present as subcutaneous nodules in the head and neck area. Occasionally these tumors can arise in a peripheral artery. When it does so, it is often confused with an aneurysmal dilatation of the respective vessel. In these circumstances, surgical resection with vascular reconstruction is the preferred treatment option.

PMID:34450287 | DOI:10.1016/j.avsg.2021.05.040

Management of parapharyngeal space cavernous sinus haemangioma using transoral robotic surgery

Fetched: August 29th, 2021, 5:01am GMT by M Fleet

Ann R Coll Surg Engl. 2021 Aug 27. doi: 10.1308/rcsann.2021.0059. Online ahead of print.

ABSTRACT

Cavernous haemangiomas are a very rare occurrence in the parapharyngeal space (PPS). Here, we present a case of a 58-year-old woman with an incidentally identified left PPS mass thought to be a pleomorphic adenoma that underwent excision by transoral robotic surgery (TORS). Intraoperative findings demonstrated no solid mass present and histological assessment of resected tissue confirmed a cavernous haemangioma. We discuss our experience in the management of a radiological and surgical mismatch of a PPS mass in what we believe to be the first cavernous haemangioma to be excised by TORS in this region.

PMID:34448405 | DOI:10.1308/rcsann.2021.0059

Sirolimus treatment for neonate with blue rubber bleb nevus syndrome: A case report

Fetched: August 29th, 2021, 5:01am GMT by Si-Si Yang

World J Clin Cases. 2021 Aug 16;9(23):6929-6934. doi: 10.12998/wjcc.v9.i23.6929.

ABSTRACT

BACKGROUND: Blue rubber bleb naevus syndrome (BRBNS) is a rare disease that usually presents with multiple venous malformations in the skin and gastrointestinal tract. Lesions located in the gastrointestinal tract always result in chronic gastrointestinal bleeding and severe anemia. The successful management of BRBNS with sirolimus had been reported in many institutions, due to its impact on signaling pathways of angiogenesis. However, the experience in treatment of neonates with BRBNS was limited.

CASE SUMMARY: A 38-day-old premature female infant born with multiple skin lesions, presented to our center complaining of severe anemia and hematochezia. Laboratory examination demonstrated that hemoglobin was 5.3 g/dL and contrast-enhanced abdominal computed tomography showed multiple low-density space-occupying lesions in the right lobe of the liver. She was diagnosed as having BRBNS based on typical clinical and examination findings. The patient was treated by transfusions twice and hemostatic drugs but symptoms of anemia were difficult to alleviate. A review of BRBNS case reports found that patients had been successfully treated with sirolimus. Then the patient was treated with sirolimus at an average dose of 0.95 mg/m²/d with a target drug level of 10-15 ng/mL. During 28 mo of treatment, the lesion was reduced, hemoglobin returned to normal, and there were no adverse drug reactions.

CONCLUSION: This case highlights the dosing regimen and plasma concentration in neonates, for the current common empiric dose is high.

PMID:34447844 | PMC:PMC8362515 | DOI:10.12998/wjcc.v9.i23.6929

Thoracoscopic Surgery by Thin Thoracoscope with Fine Needlescopic Forceps for Cavernous Hemangioma in the Chest Wall

Fetched: August 29th, 2021, 5:01am GMT by Hideki Fujimori

Kyobu Geka. 2021 Sep;74(9):668-671.

ABSTRACT

A 27-year-old women was referred to our hospital because of abnormal subpleural nodule in her right thoracic cavity. Chest computed tomography demonstrated an 11 mm nodule with smooth and clear boundary adjacent to the right first rib. Chest magnetic resonance imaging revealed an iso-intensity area on T1-weighted images, a high-intensity on T2-weighted images, and enhanced homogeneously on contrast-enhanced images. Tumor extirpation was performed using a 2.7 mm grasp fine needlescopic forceps, a 3 mm thoracoscope and a 5 mm vascular sealing device. The histological diagnosis was cavernous hemangioma. Thoracoscopic surgery using fine needlescopic forceps and thin thoracoscope is useful in considering esthetic purposes.

PMID:34446619

Ultrasound-Guided Moving Shot Radiofrequency Ablation of Benign Soft Tissue Neoplasm

Fetched: August 29th, 2021, 5:01am GMT by Wei-Che Lin

Medicina (Kaunas). 2021 Aug 17;57(8):830. doi: 10.3390/medicina57080830.

ABSTRACT

Background and Objective: To evaluate the effectiveness of radiofrequency ablation (RFA) using the moving-shot technique for benign soft tissue neoplasm. Materials and Methods: This retrospective study reviewed eight patients with benign soft tissue neoplasm presenting with cosmetic concerns and/or symptomatic issues who refused surgery. Six patients had vascular malformation, including four with venous malformation and two with congenital hemangioma. The other two patients had neurofibroma. All patients underwent RFA using the moving-shot technique. Imaging and clinical follow-up were performed in all patients. Follow-up image modalities included ultrasound (US), computed tomography (CT), and magnetic resonance (MR) imaging. The volume reduction ratio (VRR), cosmetic scale (CS), and complications were evaluated. Results: Among the seven patients having received single-stage RFA, there were significant volume reductions between baseline (33.3 ± 21.2 cm³), midterm follow-up (5.1 ± 3.8 cm³, p = 0.020), and final follow-up (3.6 ± 1.4 cm³, p = 0.022) volumes. The VRR was 84.5 ± 9.2% at final follow-up. There were also significant improvements in the CS (from 3.71 to 1.57, p = 0.017). The remaining patient, in the process of a scheduled two-stage RFA, had a 33.8% VRR after the first RFA. The overall VRR among the eight patients was 77.5%. No complications or re-growth of the targeted lesions were noted during the follow-up period. Of the eight patients, two received RFA under local anesthesia, while the other six patients were under general anesthesia. Conclusions: RFA using the moving-shot technique is an effective, safe, and minimally invasive treatment for benign soft tissue neoplasms, achieving mass volume reduction within 6 months and significant esthetic improvement, either with local anesthesia or with general anesthesia under certain conditions.

PMID:34441036 | PMC:PMC8402204 | DOI:10.3390/medicina57080830

C-KIT Expression in Orbital Cavernous Venous Hemangiomas

Fetched: August 29th, 2021, 5:01am GMT by Mizhir Atallah

Biomolecules. 2021 Aug 12;11(8):1199. doi: 10.3390/biom11081199.

ABSTRACT

Orbital (slow flow) cavernous venous hemangiomas (OCVH) are the most common benign orbital tumors in adults. The c-KIT is a tyrosine kinase receptor, which is expressed on several types of cells, is thought to play a key role in tumor pathogenesis. The purpose of this study was to evaluate the presence of the receptor c-KIT in OCVH. Our retrospective study examined 16 orbital cavernous venous hemangiomas from 16 cases operated on between 2006-2016 at Emek Medical Center. The mean tumor size was 18.4 mm. Symptoms appeared between 6 months and 22 years before operation. All specimens were analyzed for the c-KIT receptor through immunohistochemistry. The c-KIT was expressed by the endothelium in all 16 preparates. Staining was strong in two cases, moderate in six, and weak in eight cases, with no statistically significant correlation between staining and tumor size (p = 0.69) or the symptom duration (p = 0.15). We conclude that c-KIT may play an important role in the pathogenesis of OCVH. This pilot study is significant in that tumor-targeted therapy such as Imatinib Mesylate and Sunitinib may have a role in treating surgically complicated cases located in the orbital apex. A large multicenter collaborative study is necessary to examine the role of c-KIT in OCVH.

PMID:34439864 | PMC:PMC8392613 | DOI:10.3390/biom11081199

Compressive vertebral hemangiomas with neurological deficits: diagnosis, surgical strategies and long term outcome

Fetched: August 27th, 2021, 5:01am GMT by Krishnakumar Kesavapisharady

Br J Neurosurg. 2021 Aug 26:1-11. doi: 10.1080/02688697.2021.1967878. Online ahead of print.

ABSTRACT

PURPOSE: Compressive vertebral hemangiomas with neurological deficits (CVHND) form a rare, unique subset of lesions comprising of differing clinico-imaging findings, pathologic behavior and treatment, when compared to the commoner and usually incidental intra-osseus vertebral hemangiomas (VH). Though various surgical strategies and a broad array of adjuncts have evolved and changed over the years, there is paucity of comprehensive data from sizeable series of such patients treated surgically with long term follow up. The purpose of this study is to device an optimum management strategy in CVHND based on our surgical experience.

MATERIALS AND METHODS: The data from electronic medical records of 26 consecutive patients operated in our department from 2009 to 2019 were retrospectively analyzed.

RESULTS: There were 11 males and 15 females with a mean age of 34.7 years. Neurological examination revealed paraparesis or paraplegia with myelopathy in all patients with Frankel score of B, C and D in 1 (3.9%), 11 (42.3%) and 14 (53.8%) patients respectively. Sixteen patients (61%) underwent laminectomy and gross total excision of extradural soft tissue component, 7 (27%) laminectomy with posterolateral fusion, three (12%) underwent additional anterior interbody support. The mean follow up was 72.4 months and at last follow-up 24 patients (92%) were Frankel E. Symptomatic recurrence was seen in two patients operated early in the series, they underwent re-surgery, gross total excision with posterolateral fusion, remaining neurologically intact at last follow up.

CONCLUSIONS: Pre-operative embolization, surgical excision of extradural component and intra-operative vertebroplasty form the mainstay of treatment for CVHND. Instrumented posterolateral fusion with optional anterior interbody support accomplished through the same approach is required only in a minority of cases. The long-term outcome following timely and appropriate treatment is excellent. Anterior or anterolateral approaches for intervertebral support and radical procedures like total en-bloc spondylectomy (TES) are not usually required.

PMID:34435527 | DOI:10.1080/02688697.2021.1967878

Prenatal diagnosis of umbilical cord hemangioma

Fetched: August 27th, 2021, 5:01am GMT by W Y Lok

Ultrasound Obstet Gynecol. 2021 Aug 25. doi: 10.1002/uog.24755. Online ahead of print.

NO ABSTRACT

PMID:34435409 | DOI:10.1002/uog.24755

Analysis of onset and clinical characteristics in Japanese patients with infantile hemangioma

Fetched: August 27th, 2021, 5:01am GMT by Aya Hashimoto

Drug Discov Ther. 2021 Aug 26. doi: 10.5582/ddt.2021.01066. Online ahead of print.

ABSTRACT

Infantile hemangioma (IH) is a common benign tumor during infancy, although the detailed mechanism behind it has not been fully elucidated. Based on previous studies, we hypothesized that formation of hemangioma might be triggered by secondary physiological events (perinatal hypoxia or mechanical stress during delivery) in patients carrying germline risk mutations. We aimed to clarify the mechanism by evaluating whether head and neck lesions were more frequent in patients in who IH appeared after birth compared with those in who it was present at birth. Clinical data of 62 lesions in 51 patients with IH were collected. All patients were analyzed for correlation of onset with gender, localization, family histories, gestational age, birth weight, and clinical subtypes. Distribution of lesions on the head and neck was slightly more frequent in the after-birth IH group, compared with those with IH present at birth, but without significant difference (47.6% vs. 40.0%, p = 0.32). On the other hand, the ratio of superficial and deep type IH at birth was significantly altered compared with that in IH after birth (19:0 vs. 26:7, p = 0.039). In addition, IHs appearing after birth tended to more commonly have multiple lesions than those with IH present at birth, with statistically significant difference (25.8% vs. 0%, p = 0.0164). There may therefore be different triggers for IHs at birth and IH after birth. Further studies with greater number of patients are necessary to validate these findings.

PMID:34433757 | DOI:10.5582/ddt.2021.01066

Propranolol inhibits infantile hemangioma by regulating the miR-424/vascular endothelial growth factor-A (VEGFA) axis

Fetched: August 27th, 2021, 5:01am GMT by Zhen-Bo Wu

Transl Pediatr. 2021 Jul;10(7):1867-1876. doi: 10.21037/tp-21-244.

ABSTRACT

BACKGROUND: Infantile hemangioma (IHA) is the most common tumor in infancy. We aimed to explore the effect of propranolol on the expression of microRNA (miR)-424 in IHA tissues and XPTS-1 cells, as well as its molecular mechanism of inhibiting XPTS-1 cell activity.

METHODS: Tumor tissues and peritumoral tissue were collected from 13 IHA patients in Lishui Municipal Central Hospital. The level of miR-424 were detected using real-time quantitative reverse transcription polymerase chain reaction (RT-PCR). Cell counting kit-8 (CCK-8) was used to measure XPTS-1 cell viability. Flow cytometry and transwell were used to detect the apoptosis level and invasion ability of XPTS-1 cells. Western blot was used to measure the protein level of vascular endothelial growth factor-A (VEGFA). The luciferase reporter gene assay detected the targeting relationship between miR-424 and VEGFA.

RESULTS: Compared with normal tissues and human umbilical vein endothelial cells, the expression level of miR-424 in IHA tissues and XPTS-1 cells was significantly reduced (P<0.05). As the concentration of propranolol increased, XPTS-1 cell viability gradually decreased (P<0.05), and the expression level of VEGFA decreased (P<0.05). The expression of miR-424 increased with the time of propranolol treatment (P<0.05). Compared with the control group, treatment with an miR-424 inhibitor resulted in a significant increase in XPTS-1 cell viability and invasion ability (P<0.05), and a decrease in apoptosis (P<0.05). However, both propranolol and miR-424 inhibitor treatment resulted in a partial decrease in XPTS-1 cell viability (P<0.05), and a partial increase in the level of apoptosis (P<0.05). MiR-424 directly targeted VEGFA; the overexpression of miR-424 resulted in a decrease in the VEGFA protein level (P<0.05), while inhibition of miR-424 resulted in an increase in the VEGFA protein level (P<0.05). Compared with the propranolol group, the XPTS-1 cell viability and invasion ability in the propranolol + VEGFA-si group were significantly decreased (P<0.05), while the level of apoptosis increased (P<0.05). Meanwhile, simultaneous miR-424 inhibitor treatment resulted in no difference in cell viability and apoptosis levels compared with the propranolol group, and the invasion ability was partially restored (P<0.05).

CONCLUSIONS: Propranolol affects the malignant biological behavior of IHA cells by regulating the miR-424/VEGFA axis.

PMID:34430434 | PMC:PMC8349971 | DOI:10.21037/tp-21-244

Large abdominal purpura of neonatal retroperitoneal kaposiform hemangioendothelioma

Fetched: August 27th, 2021, 5:01am GMT by Ryo Takemura

Clin Case Rep. 2021 Aug 16;9(8):e04600. doi: 10.1002/ccr3.4600. eCollection 2021 Aug.

ABSTRACT

Large abdominal purpuras may be caused by retroperitoneal kaposiform hemangioendothelioma with consumptive coagulopathy. Clinicians should perform serial ultrasonography studies to detect the sings of tumor until the final diagnosis is confirmed.

PMID:34429992 | PMC:PMC8365556 | DOI:10.1002/ccr3.4600

Permalink for 'Non-invasive multi-channel deep learning convolutional neural networks for localization and classification of common hepatic lesions'

Non-invasive multi-channel deep learning convolutional neural networks for localization and classification of common hepatic lesions

Fetched: August 27th, 2021, 5:01am GMT by Shubham Shah

Pol J Radiol. 2021 Jul 20;86:e440-e448. doi: 10.5114/pjr.2021.108257. eCollection 2021.

ABSTRACT

PURPOSE: Machine learning techniques, especially convolutional neural networks (CNN), have revolutionized the spectrum of computer vision tasks with a primary focus on supervised and labelled image datasets. We aimed to assess a novel method to segment the liver from the abdomen computed tomography (CT) image using the CNN network, and to train a unique method to locate and classify liver lesion pre-histological findings using multi-channel deep learning CNN (MDL-CNN).

MATERIAL AND METHODS: The post-contrast CT images of the liver with a resolution of 0.625 mm were chosen for the study. In a random method, 50 examples of each hepatocellular carcinomas, metastases tumours, haemangiomas, hepatic cysts were chosen and evaluated.

RESULTS: The dice score quantitatively analyses the similarity of segmentation results with the training dataset. In the first CNN model for segmenting the liver, the dice score was 96.18%. The MDL-CNN model yielded 98.78% accuracy in classification, and the dice score for locating liver lesions was 95.70%. Additionally, the performance of this model was compared to various other existing models.

CONCLUSIONS: According to our study, the machine learning approach can be successfully implemented to segment the liver and classify lesions, which will help radiologists impart better diagnosis.

PMID:34429791 | PMC:PMC8369821 | DOI:10.5114/pjr.2021.108257

Permalink for 'Utility of spinal angiography and arterial embolization in patients undergoing CT guided alcohol injection of aggressive vertebral hemangiomas'

Utility of spinal angiography and arterial embolization in patients undergoing CT guided alcohol injection of aggressive vertebral hemangiomas

Fetched: August 25th, 2021, 5:01am GMT by Gopinath Srinivasan

Neuroradiology. 2021 Aug 24. doi: 10.1007/s00234-021-02788-7. Online ahead of print.

ABSTRACT

PURPOSE: The purpose of this study is to evaluate the role of spinal angiography and arterial embolization in avoiding spinal cord ischemia in patients undergoing CT-guided alcohol injection of aggressive vertebral hemangiomas.

METHODS: In this retrospective study, patients with vertebral hemangioma who underwent CT-guided direct alcohol injection between January 2007 and October 2018 were identified. Of 28 such patients, 26 had neurological deficits, and 2 had only back pain or radiculopathy. Direct alcohol injection without prior arterial embolization was done in 17 patients. Direct alcohol injection with prior arterial embolization was done in 11 patients. Clinical outcome was assessed immediately after the intervention and at follow-up.

RESULTS: Three patients, who underwent alcohol injection without trans-arterial embolization, had worsening of neurological deficits in the post procedure period due to spinal cord ischemia. No complications related to spinal cord ischemia were noted in the embolization group. There was no significant difference in the outcomes between the two groups if the three patients with complications are excluded (p = 0.34).

CONCLUSION: While CT-guided direct alcohol injection is effective in the management of symptomatic and aggressive vertebral hemangiomas, spinal angiography and trans-arterial embolization of the blood supply to the vertebral body hemangioma, prior to the direct transpedicular alcohol embolization of the lesion, improves the safety of the procedure.

PMID:34427707 | DOI:10.1007/s00234-021-02788-7

Clinical Approaches to External Auditory Canal Hemangiomas: A Systematic Review

Fetched: August 25th, 2021, 5:01am GMT by Afrah Alshalan

Ear Nose Throat J. 2021 Aug 24:1455613211042449. doi: 10.1177/01455613211042449. Online ahead of print.

ABSTRACT

OBJECTIVES: Hemangioma is a common soft tissue tumor that can be categorized mainly into cavernous, capillary, and venous subtypes. It usually develops in the head and neck and rarely in the external auditory canal (EAC).

METHODS: A systematic review of patients with capillary hemangioma of the EAC was performed. A computerized systematic search was conducted in PubMed, Scopus, and Web of Science to extract relevant studies for a qualitative review.

RESULTS: A total of 38 patients were included in the systematic review, and half of them were men. Hearing loss was the most common presenting feature (n = 19; 50%), followed by aural fullness (n = 15; 39.4%). Cavernous hemangioma was the predominant type (n = 22, 57.8%). Endaural and transcanal surgical approaches were the most frequent, with 10 (26.3%) and 9 (23.6%) cases, respectively. Most of the patients (n = 31, 81.5%) had no recurrence.

CONCLUSIONS: Hemangioma of the EAC may not be uncommon, as health care professionals may expect. Hearing loss, aural fullness, and tinnitus are possible symptoms, but many patients remain asymptomatic. Surgical excision is the main effective management option, and complete resolution is the most prevalent outcome. Computed tomography assists in the diagnosis, but histopathological examination after resection is mandatory for the definitive diagnosis.

PMID:34427123 | DOI:10.1177/01455613211042449

Successful anesthetic management of a giant lower lip hemangioma patient using high flow nasal cannula: A case report

Fetched: August 25th, 2021, 5:01am GMT by Ji Yeon Kim

Korean J Anesthesiol. 2021 Aug 24. doi: 10.4097/kja.21231. Online ahead of print.

ABSTRACT

BACKGROUND: Giant lip hemangioma is a rare disease that may cause difficulty in preoxygenation and ventilation when using facemasks and intubation during general anesthesia induction.

CASE: A laparoscopic cholecystectomy was planned for a 77-year-old woman. The patient had a giant lower lip hemangioma that was 12 x 5 x 5 cm, which made preoxygenation and ventilation through a face mask impossible and put her at risk of hemangioma rupture. We preoxygenated her through a high-flow nasal cannula. Following propofol and succinylcholine administration, we intubated the patient with a video laryngoscope without desaturation, hemangioma rupture, or CO2 retention.

CONCLUSIONS: High-flow nasal cannula is a useful tool when difficult intubation is expected in patients who have problems using conventional facemasks.

PMID:34425640 | DOI:10.4097/kja.21231

Permalink for 'Clinical study of modified one stage posterior approach total en block spondylectomy and spinal reconstruction in the treatment of invasive thoracic vascular tumor'

Clinical study of modified one stage posterior approach total en block spondylectomy and spinal reconstruction in the treatment of invasive thoracic vascular tumor

Fetched: August 25th, 2021, 5:01am GMT by Zeng-Ping Wang

Zhongguo Gu Shang. 2021 Aug 25;34(8):759-63. doi: 10.12200/j.issn.1003-0034.2021.08.013.

ABSTRACT

OBJECTIVE: To explore the surgical method and safety of modified one stage posterior approach total en block spondylectomy combined with pedicle screw fixation and titanium mesh reconstruction for the treatment of invasive thoracic vascular tumor.

METHODS: The clinical data of 12 patients with invasive thoracic vasculay tumor from December 2012 to May 2015 was retrospectively analyzed. There were 8 males and 4 females, aged from 40 to 62 years with an average of 51.2 years, the course of disease was 2 months to 8 years with an average of 3.4 years. The lesions involved vertebral bodies:1 case of T2, 4 cases of T4, 1 case of T5, 2 cases of T6, 2 cases of T8, and 2 cases of T10. According to Tomita classification, there were 3 cases of typeⅠ, 3 cases of typeⅡ, 1 case of type Ⅲ, and 5 cases of type Ⅳ. The Japanese Orthopaedic Association (JOA) score was 8.0±2.7 before operation. One patient with T10 lesions had sensory dyskinesia below the umbilicus, and the muscle strength of both lower limbs was grade Ⅰ-Ⅱ, and the others 11 patients were grade Ⅲ-Ⅳ. All 12 patients underwent one stage posterior approach total en block spondylectomy and the pedicle screw fixation combined with titanium mesh reconstruction under general anesthesia and continuous motor evoked potential (MEP) spinal cord electrophysiological monitoring throughout the operation. The operation time, intraoperative blood loss and transfusion, postoperative pain and recovery of spinal cord function, bone graft fusion, tumor recurrence and other complications were followed up.

RESULTS: All the operations were successful. The average operation time, intraoperative blood loss and blood transfusion were 5.5 h (4.5 to 6.0 h), 1 850 ml (1 650 to 2 500 ml), 1 050 ml (600 to 1 500 ml), respectively. All 12 patients were followed up for 5 months to 2.5 years with an average of 21 months. Local pain and lower limb muscle strength were improved to varying, and the nerve compression symptoms disappeared. The JOA score at 6 months after operation was 12.0±3.4, which was statistically significant difference compared with the preoperative 8.0±2.7 (t=3.20, P<0.05). Titanium mesh bone grafts were all fused in phaseⅠ, with an average fusion time of 4.5 months (3 to 7 months). During the follow-up period, there was no tumor recurrence, loosening or breaking of nails, sinking and displacement of titanium mesh.

CONCLUSION: Modified one stage posterior approach total en block spondylectomy is an ideal surgical method for the treatment of invasive thoracic vascular tumors, which has a safe, reliable and long lasting efficacy.

PMID:34423621 | DOI:10.12200/j.issn.1003-0034.2021.08.013

Newly detected liver nodules with a history of colorectal cancer: are they metastatic? Review of 2,632 cases in a single center

Fetched: August 25th, 2021, 5:01am GMT by Kai Zhu

Ann Transl Med. 2021 Jul;9(13):1079. doi: 10.21037/atm-20-8153.

ABSTRACT

BACKGROUND: The diagnosis of newly detected liver nodules in patients with colorectal cancer (CRC) is crucial for determining prognosis and treatment. Accurate identification of benign nodules can help avoid unnecessary therapy. The aim of our study was to retrospectively review patients with CRC who underwent liver resection for benign liver nodules misdiagnosed as CRC metastasis (CRLM) in our institution.

METHODS: We reviewed all patients with a history of CRC who underwent liver resection from January 2012 to December 2019 in our institution. We specifically focused on nodules pathologically confirmed as benign. The pathology was rechecked by an independent pathologist. The clinicopathological characteristics of these patients were collected. Preoperative imaging examinations, including ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT) were reviewed.

RESULTS: From 2012 to 2019, a total of 2,632 patients with CRC who were preoperatively diagnosed CRLM received liver resection, among which 2,584 (98.2%) cases were proven to be malignant, and 48 (1.8%) cases were benign. Among these 48 cases, 24 were pathologically confirmed as focal nodular hyperplasia (FNH), 9 were peliosis, 10 were inflammatory lesions, and 5 were hemangioma. At least one preoperative imaging examination indicated CRLM, with a median size of 2.0 cm (range, 0.4-8.0 cm). Before liver resection, ten patients received chemotherapy after the discovery of liver nodules.

CONCLUSIONS: It should be noted that newly detected liver nodules in patients with a history of CRC could be benign. Accurate diagnosis of liver nodules in CRC is necessary to avoid overtreatment and to identify cost-effective medication.

PMID:34422991 | PMC:PMC8339815 | DOI:10.21037/atm-20-8153

Real-world data on the clinicopathological traits and outcomes of hospitalized liver hemangioma patients: a multicenter study

Fetched: August 25th, 2021, 5:01am GMT by Tengqian Tang

Ann Transl Med. 2021 Jul;9(13):1067. doi: 10.21037/atm-20-4684.

ABSTRACT

BACKGROUND: There is currently a lack of consensus regarding the clinical features, diagnosis, treatment indications and options, and risk assessment of hepatic hemangioma patients.

METHODS: This was a multicenter, real-world study that analyzed a large number of hepatic hemangioma cases in China and included patient data on epidemiology, diagnosis, treatment methods, and outcomes.

RESULTS: A total of 5,143 patients hospitalized for hepatic hemangioma were included, of whom 34.42% were male and 65.58% were female. The age distribution was concentrated between 30 and 60 years old, accounting for 87.41% of the patients. Among the hepatic hemangioma patients, 60.8% had only one tumor, with the most common pathological type being cavernous hemangioma (96.07% of cases). The treatment motivations and indications included anxiety, obvious clinical symptoms, rapid tumor growth, unclear diagnoses and acute emergencies. Overall, 41.4% of the patients were treated for psychological reasons, while 30.59% were treated because they presented obvious (primarily nonspecific) clinical symptoms. Hepatic resection was the main therapeutic method and was based on various indications. There were a small number of patients with Kasabach-Merritt syndrome, according to its generally recognized definition.

CONCLUSIONS: Most patients in this study who were hospitalized for hepatic hemangioma did not meet the indications for requiring treatment. Surveillance is the recommended course of action for definitively diagnosed hepatic hemangioma, and a new classification system is needed to standardize the diagnosis of this condition.

PMID:34422979 | PMC:PMC8339840 | DOI:10.21037/atm-20-4684

Case Report: High-Definition 4K-3D Exoscope for Removal of an Orbital Cavernous Hemangioma Using a Transpalpebral Approach

Fetched: August 25th, 2021, 5:01am GMT by Stefano Peron

Front Surg. 2021 Aug 6;8:671423. doi: 10.3389/fsurg.2021.671423. eCollection 2021.

ABSTRACT

Background: Cavernous hemangioma, also known as cavernous vascular malformation (CVM), is the most common primary lesion of the orbit in adults. The management of these lesions is challenging and is strongly dependent on their location, as well as the patient's symptoms and expectations. The trans-palpebral approach is currently used in surgery for orbital tumors, anterior skull base tumors, and even more, orbital reconstruction, because of its well-demonstrated esthetic advantages. Similarly, the use of magnification can be provided by surgical loupes, microscope, or more recently, endoscope, which is well-documented for its advantages in terms of minimal invasiveness and safety. In the last years, the use of exoscopes in microsurgery has been proposed due to their greater and sharper intraoperative magnification, but never for the removal of orbital tumors. Clinical Presentation: We describe a case of a 38-year-old woman with a right orbital intraconic CVM removed using an inferior transpalpebral approach performed under 4K-3-dimensional (4K-3D) exoscopic vision. Navigation and ultrasound were also used, with the former allowing better identification of the lesion within the orbit and the second overcoming the limitations of navigation, in terms of the retraction on the ocular globe before or just after periorbital incision. Conclusion: The use of a 4K-3D exoscope allowed us to perform the surgery safely, thanks to the high magnification and definition of anatomical details, with the surgeon operating in an upright, comfortable position. The CVM was completely removed with excellent results from both functional and esthetic points of view.

PMID:34422890 | PMC:PMC8377276 | DOI:10.3389/fsurg.2021.671423

Crotoxin Inhibits Endothelial Cell Functions in Two- and Three-dimensional Tumor Microenvironment

Fetched: August 25th, 2021, 5:01am GMT by Ellen Emi Kato

Front Pharmacol. 2021 Aug 4;12:713332. doi: 10.3389/fphar.2021.713332. eCollection 2021.

ABSTRACT

Antitumor property of Crotoxin (CTX), the major toxin from Crotalus durissus terrificus snake venom, has been demonstrated in experimental animal models and clinical trials. However, the direct action of this toxin on the significant events involved in neovascularization, which are essential for tumor growth and survival, has not been confirmed. This study investigated the effects of CTX on the key parameters of neovascularization in two- and three-dimensional culture models. Murine endothelial cell lines derived from thymus hemangioma (t.End.1) were treated at different concentrations of CTX (6.25-200 nM). Endothelial cell proliferation, cell adhesion, and actin cytoskeletal dynamics on laminin (10 µg/ml), type I collagen (10 µg/ml), and fibronectin (3 µg/ml) were evaluated along with the endothelial cell migration and formation of capillary-like tubes in 3D Matrigel. CTX concentration of 50 nM inhibited tube formation on 3D Matrigel and impaired cell adhesion, proliferation, and migration under both culture medium and tumor-conditioned medium. These actions were not accountable for the loss of cell viability. Inhibition of cell adhesion to different extracellular matrix components was related to the reduction of αv and α2 integrin distribution and cytoskeletal actin polymerization (F-actin), accompanied by inhibition of focal adhesion kinase (FAK), Rac1 (GTPase) signaling proteins, and actin-related protein 2/3 (Arp 2/3) complex. This study proved that CTX inhibits the major events involved in angiogenesis, particularly against tumor stimuli, highlighting the importance of the anti-angiogenic action of CTX in inhibition of tumor progression.

PMID:34421610 | PMC:PMC8371242 | DOI:10.3389/fphar.2021.713332

Intra-articular leiomyoma of the knee mimicking a ganglion cyst in a child: A case report and review of literature

Fetched: August 23rd, 2021, 5:01am GMT by Karim Khezami

Int J Surg Case Rep. 2021 Aug 18;86:106320. doi: 10.1016/j.ijscr.2021.106320. Online ahead of print.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Angioleiomyoma is a rare benign soft tissue tumor of smooth muscle getting its origin from the muscular layer of vessel walls. The localized type of leiomyoma occurs on the skin, deep soft tissues, in the genitourinary, gastrointestinal, and respiratory tracts.

CASE PRESENTATION: We report a case of a 16-year-old patient who presented with an intra-articular leiomyoma developed in the anterior cruciate ligament and treated with arthroscopy.

CLINICAL DISCUSSION: Angioleiomyoma can occur anywhere in the body and is most often seen in the extremities, particularly the lower limbs, but is very rare in the knee joint. Angioleiomyoma should be included in the differential diagnosis of nodular lesions mimicking loose body, such as lipomas, inclusion cysts, ganglion, pigmented villonodular synovitis (PVNS), fibroma, nodular synovitis, hemangioma, synovial sarcoma, myopericytoma, leiomyosarcomas, glomus tumor.

CONCLUSION: Angioleiomyoma related to the cruciate ligaments of the knee should be considered in the differential diagnosis of a painful knee especially when associated with a decreased of range of motion of knee. The arthroscopic debridement should be considered the treatment of choice in order to reliably restore active ROM.

PMID:34419722 | PMC:PMC8379635 | DOI:10.1016/j.ijscr.2021.106320

Infantile hemangioma. Part 1: Epidemiology, pathogenesis, clinical presentation and assessment

Fetched: August 23rd, 2021, 5:01am GMT by Ana Isabel Rodríguez Bandera

J Am Acad Dermatol. 2021 Aug 19:S0190-9622(21)02351-3. doi: 10.1016/j.jaad.2021.08.019. Online ahead of print.

ABSTRACT

Infantile hemangioma (IH) is the most common pediatric vascular tumor. Its pathogenesis is poorly understood but thought to represent an aberrant response of pluripotent stem cells to stimuli such as hypoxia and the renin-angiotensin-system. IH usually appears during the first few weeks of life and follows a characteristic natural trajectory of proliferation and involution. Their clinical appearance depends on their depth and distribution. Classification comprises superficial, mixed and deep IH as well as IH with minimal or arrested growth. Multifocal IHs are more likely to be associated with infantile hepatic hemangioma, and although the need for screening based on a specific number of IH has been recently debated, 5 remains the most widely acceptable cut off point. Large facial or lumbar IH warrants investigation for PHACE and LUMBAR syndrome, respectively. Complications of IH include ulceration, obstruction or functional impairment, hypothyroidism, and cosmetic sequelae. Differential diagnoses mostly consist of other vascular tumors and vascular malformations, though IH may sometimes mimic non-vascular tumors or developmental anomalies. Diagnosis is usually clinical and biopsy rarely indicated. High-frequency ultrasonography may help with the differential diagnosis, particularly with subcutaneous lesions. Referral to other specialists may be required in specific cases.

PMID:34419524 | DOI:10.1016/j.jaad.2021.08.019

Infantile hemangioma. Part 2: Management

Fetched: August 23rd, 2021, 5:01am GMT by Deshan Sebaratnam

J Am Acad Dermatol. 2021 Aug 19:S0190-9622(21)02352-5. doi: 10.1016/j.jaad.2021.08.020. Online ahead of print.

ABSTRACT

The majority of infantile hemangioma (IH) can be managed conservatively but for those requiring active treatment, management has been revolutionized in the last decade by the discovery of propranolol. Patients that may require active intervention should receive specialist review, ideally before five weeks of age to mitigate the risk of sequelae. The majority of infants can be commenced on propranolol in the outpatient setting and the most frequently employed dosing regimen is 1mg/kg twice daily. In the future, β-blockers with a more selective mechanism of action, such as atenolol, show some promise. In recalcitrant lesions, systemic corticosteroids or sirolimus may be considered and for small, superficial IH, topical timolol maleate or pulsed dye laser may be considered. Where IH involute with cutaneous sequelae a range of interventions have been reported including surgery, laser and embolization. IH have a well described clinical trajectory and avail themselves to diagnosis and management via telemedicine. Algorithms have been constructed to stratify those patients who can be managed remotely from those who warrant in-person review during the COVID19 pandemic.

PMID:34419523 | DOI:10.1016/j.jaad.2021.08.020

Oral Hemangiomas

Fetched: August 22nd, 2021, 5:01am GMT by Lauren A. Lyssy

2021 Aug 14. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

Oral hemangiomas (OHs) are benign tumors that develop due to endothelial cell proliferation and occur in and around the oral cavity. While 60 to 70 percent of hemangiomas occur in the head and neck region, OHs are relatively rare and most frequently involve the lips, tongue, buccal mucosa, and palate. OHs have also been noted in the mandible and maxilla (central hemangiomas) and within the masseter and other muscles of mastication (intramuscular hemangiomas).

The term “hemangioma” and “vascular malformation” have often been used interchangeably, creating significant confusion in both the clinical setting and in the literature. As Mulliken and Glowacki proposed in their 1982 classification system, “hemangiomas” are true neoplasms characterized by proliferation and increased rates of endothelial cell turnover, while “vascular malformations” are localized anomalies due to defects in vascular morphogenesis with normal rates of cell turnover.

Clinically, hemangiomas can be classified as infantile (formerly called juvenile or strawberry) or congenital. Infantile hemangiomas (IHs) develop during the first 2 months of life and demonstrate rapid proliferation between 6 and 12 months of age, followed by a period of slow involution. Most IHs will spontaneously regress between 6 and 9 years of age. In contrast, congenital hemangiomas (CHs) are present at birth, do not exhibit a proliferative phase, and either rapidly involute or not at all. The majority of hemangiomas will completely involute, with 10%-20% persisting into adolescence or adulthood. While medical, interventional, and surgical regimens are available, there is no standardized treatment for OHs. Due to myriad potential complications, treatment of OHs is typically not pursued unless functional impairment exists.

PMID:32809603 | Bookshelf:NBK560768

Focal Nodular Hyperplasia

Fetched: August 22nd, 2021, 5:01am GMT by Sammy Hamad

2021 Aug 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

The liver is the only self-regenerative internal organ in the human body. This regenerative capability places the liver at an inherent risk for developing atypical masses. While the majority of liver masses present as predominantly solid or cystic masses, the etiologies are broad. In 1958, pathologist, Hugh Edmondson, MD, first described focal nodular hyperplasia (FNH) as a solid, benign hepatic mass of non-vascular origin. Unlike the most common liver mass which is the hemangioma, focal nodular hyperplasia is thought to be the result of increased hepatocyte number caused by hypoperfusion or hyperperfusion from anomalous arteries within the hepatic lobule. Focal nodular hyperplasia often is confounded by comorbid conditions making establishing a diagnosis and management difficult.

Previously, focal nodular hyperplasia was referred to by a variety of synonyms including pedunculated adenoma, solitary hyperplastic nodule, focal cirrhosis, and hepatic hamartoma. Because of the indeterminate classification, a standard diagnosis needed to be established. In 1994, the International Working Party of the World Congresses of Gastroenterology standardized the diagnosis term as follicular nodular hyperplasia and categorized it as a regenerative liver nodule. After that, follicular nodular hyperplasia was distinguished from neoplastic hepatic conditions.

PMID:30335277 | Bookshelf:NBK532244

An Unusual Case of Meyerson Phenomenon Around Infantile Hemangioma

Fetched: August 22nd, 2021, 5:01am GMT by Giulia Veronesi

Dermatol Pract Concept. 2021 Jul 8;11(3):e2021039. doi: 10.5826/dpc.1103a39. eCollection 2021 Jul.

NO ABSTRACT

PMID:34414001 | PMC:PMC8337032 | DOI:10.5826/dpc.1103a39

Isolated Focal Infantile Hemangioma of the Alveolar Ridge with Glucose Transporter-1 Reactivity: An Aberrant Presentation

Fetched: August 22nd, 2021, 5:01am GMT by Divya Natarajan

Int J Clin Pediatr Dent. 2021 Mar-Apr;14(2):319-322. doi: 10.5005/jp-journals-10005-1923.

ABSTRACT

AIM AND OBJECTIVE: To give dental practitioners an insight into the unusual presentation of focal infantile hemangioma on the alveolar ridge showing immunoreactivity to glucose transporter-1 (GLT1). We also recommend the importance of using standardized terminologies to describe the type of hemangioma.

BACKGROUND: One of the most commonly seen and misdiagnosed vascular tumors of the skin and soft tissues of the head and neck region in children are infantile hemangiomas. Infantile hemangiomas developing at extracutaneous locations such as the oral cavity are rare, especially without cutaneous involvement.

CASE DESCRIPTION: A 9-month-old infant girl presented to the Pediatric Dental Department with a swelling over the maxillary alveolar ridge. The solitary exophytic mass appeared after birth and gradually enlarged in size with episodes of bleeding during feeding. Clinical examination revealed a reddish-pink pedunculated mass that bled profusely on palpation. The lesion was excised by electrocautery and was found to be express glucose transporter-1 confirming the diagnosis of infantile hemangioma. The patient was followed up for 1 year and showed no recurrence.

CONCLUSION: Hemangiomas are usually misinterpreted to be pyogenic granulomas, vascular malformation, reactive granulation tissue, or congenital granular cell tumor. This report highlights the use of glucose transporter-1 when there is suspicion of hemangioma for immunohistochemical distinction from other histologically similar vascular lesions of the oral cavity.

CLINICAL SIGNIFICANCE: Dental surgeons treating vascular lesions of the oral cavity should advocate for surgical management techniques that also produce hemostasis due to the risk of intraoperative bleeding.

HOW TO CITE THIS ARTICLE: Natarajan D, Muthukali S, Nachiappan N, et al. Isolated Focal Infantile Hemangioma of the Alveolar Ridge with Glucose Transporter-1 Reactivity: An Aberrant Presentation. Int J Clin Pediatr Dent 2021;14(2):319-322.

PMID:34413614 | PMC:PMC8343689 | DOI:10.5005/jp-journals-10005-1923

Cytologic features of hepatic YAP1-TFE3 rearranged epithelioid hemangioendothelioma

Fetched: August 20th, 2021, 5:01am GMT by Melanie Bourgeau

Diagn Cytopathol. 2021 Aug 19. doi: 10.1002/dc.24853. Online ahead of print.

ABSTRACT

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of intermediate malignancy, often with indolent behavior. Though most cases have a characteristic WWTR1-CAMTA1 gene fusion, a subtype of EHE with YAP1-TFE3 fusions and a distinct morphology has recently been described histologically, but no cases of YAP1-TFE3 EHE have been described in the cytology literature. We herein report on a case of YAP1-TFE3 fusion associated EHE diagnosed on fine-needle aspiration and core biopsy of a liver mass in an 18-year-old male patient who presented with synchronous lung and liver involvement. We also discuss the differential diagnosis of EHE on cytology specimens.

PMID:34411462 | DOI:10.1002/dc.24853

Short-term efficacy of superficial X-ray treatment for infantile maxillofacial hemangioma

Fetched: August 20th, 2021, 5:01am GMT by Li-Na Tang

Hua Xi Kou Qiang Yi Xue Za Zhi. 2021 Aug 1;39(4):464-468. doi: 10.7518/hxkq.2021.04.014.

ABSTRACT

OBJECTIVES: This study aimed to explore the short-term clinical efficacy and factors influencing low-dose superficial X-ray for treating infantile maxillofacial hemangioma.

METHODS: Retrospective analysis was conducted on 161 cases of infants with maxillofacial hemangioma treated with superficial X-ray in the Laser Center of Dermatology Department of Sichuan Provincial People's Hospital from January 2015 to December 2017. Clinical efficacy was analyzed by comparing the photos before and after treatment. Patients were further divided into groups according to different genders, age at the start of treatment, preterm birth or low birth weight, hemangioma site, longest diameter of hemangioma, and type of hemangioma to analyze whether differences existed in clinical efficacy and therapeutic dose between different groups.

RESULTS: Twelve months after the end of treatment, the overall cure rate was 93.8%, and the significant efficiency was 97.5%. The clinical efficacy was related to the age of children at the beginning of treatment and the type of hemangioma (P<0.05). The clinical efficacy of children aged less than or equal to 12 months and superficial hemangioma group was better than that of children aged more than 12 months and deep subtype or mixed hemangioma group, respectively. Therapeutic doses associa-ted with hemangioma treatment with diameter, category, age (P<0.05), diameter greater than or equal to 4 cm hemangioma group, the mixed type or deep in the group, the children older than 12 months hemangioma group, respectively, the dia-meter is less than 4 cm hemangioma, superficial hemangioma group and age less than or equal to 12 months hemangioma total treatment group exposure dose is greater.

CONCLUSIONS: Low-dose superficial X-ray is safe and effective for the treatment of infantile maxillofacial hemangioma. Age and type of hemangioma at the time of treatment are the factors influencing therapeutic dose and clinical efficacy.

PMID:34409804 | PMC:PMC8381126 | DOI:10.7518/hxkq.2021.04.014

Dermoscopic features of infantile hemangioma during treatment with topical propranolol

Fetched: August 20th, 2021, 5:01am GMT by Manuel Valdebran

JAAD Int. 2020 Aug 7;1(2):121-123. doi: 10.1016/j.jdin.2020.07.005. eCollection 2020 Dec.

ABSTRACT

PMID:34409332 | PMC:PMC8361867 | DOI:10.1016/j.jdin.2020.07.005

International heterogeneity in admission criteria and monitoring for the initiation of propranolol in infantile hemangioma

Fetched: August 20th, 2021, 5:01am GMT by Samuel Antranig Der Sarkissian

JAAD Int. 2020 Jul 31;1(2):111-113. doi: 10.1016/j.jdin.2020.06.005. eCollection 2020 Dec.

NO ABSTRACT

PMID:34409330 | PMC:PMC8361929 | DOI:10.1016/j.jdin.2020.06.005

Serous Retinal Detachment Resolution following Trabeculectomy in a Patient with Sturge-Weber Syndrome

Fetched: August 20th, 2021, 5:01am GMT by Gabriel Castilho Sandoval Barbosa

J Curr Ophthalmol. 2021 Jul 5;33(2):209-211. doi: 10.4103/joco.joco_176_20. eCollection 2021 Apr-Jun.

ABSTRACT

PURPOSE: To report a case of Sturge-Weber syndrome (SWS) complicated with uncontrolled glaucoma and serous retinal detachment (SRD) in the left eye that evolved with complete resolution after trabeculectomy.

METHODS: We report the case of a 10-year-old boy with SWS complicated with uncontrolled glaucoma and SRD in the left eye. In primary evaluation, he presented with a left-sided nevus flammeus affecting upper eyelid and best corrected visual acuity of 20/50 on the affected eye. Fundus examination revealed glaucomatous optic nerve neuropathy and diffuse choroidal hemangioma with overlying SRD, which were confirmed with spectral domain optical coherence tomography. Right eye was unremarkable.

RESULTS: The patient underwent trabeculectomy with mitomycin-C on the affected eye. Two weeks later, he presented with normalization of the intraocular pressure and substantial resolution of subretinal fluid (SRF), which improved to complete resolution of the SRD at 2 months of follow-up. In addition, there was an improvement of visual acuity from 20/50 to 20/40.

CONCLUSION: This is the first report to describe a case of SWS associated with SRD and resolution of SRF after trabeculectomy.

PMID:34409235 | PMC:PMC8365587 | DOI:10.4103/joco.joco_176_20

Expression of Cathepsins B, D, and G in Extracranial Arterio-Venous Malformation

Fetched: August 20th, 2021, 5:01am GMT by Lauren Hansen

Front Surg. 2021 Aug 2;8:676871. doi: 10.3389/fsurg.2021.676871. eCollection 2021.

ABSTRACT

Objectives: We have previously identified a population of cells that expressed stemness-associated markers in extracranial arterio-venous malformation (AVM) and demonstrated expression of cathepsins B, D, and G on embryonic stem cell (ESC)-like populations in other vascular anomalies. This study investigated the expression of cathepsins B, D, and G, and their localization in relation to this primitive population in extracranial AVM. Methods: Immunohistochemical staining was performed on AVM tissue samples from 13 patients to demonstrate expression of cathepsins B, D, and G. Western blotting was performed on four AVM tissue samples and three AVM-derived primary cell lines to confirm protein expression of cathepsins B and D proteins. RT-qPCR was performed on three AVM-derived primary cell lines to demonstrate transcript expression of cathepsins B, D, and G. Enzymatic activity assays were performed on three AVM-derived primary cell lines to investigate if cathepsins B and D were active. Localization of the cathepsins was investigated using immunofluorescence dual-staining of the cathepsins with the ESC markers OCT4 and SOX2, and mast cells marker chymase on two of the 13 AVM tissue samples. Results: Immunohistochemical staining demonstrated expression of cathepsins B, D, and G in all 13 AVM tissue samples. Western blotting showed expression of cathepsins B and D proteins in all four AVM tissue samples and all three AVM-derived primary cell lines. RT-qPCR demonstrated transcripts of cathepsins B, D, and G in all three AVM-derived primary cell lines. Enzymatic activity assays showed that cathepsins B and D were active. Immunofluorescence staining showed expression of cathepsins B and D on the OCT4+/SOX2+ endothelium and media of the lesional vessels and cells within the stroma in AVM nidus. Cathepsin G was expressed on the chymase+ phenotypic mast cells. Conclusions: This study demonstrated the novel finding of the expression of cathepsins B, D, and G in AVM. Cathepsins B and D were expressed by the primitive population, and cathepsin G was localized to mast cells, within the AVM nidus.

PMID:34409065 | PMC:PMC8367294 | DOI:10.3389/fsurg.2021.676871

Spinal Dysraphism And Myelomeningocele

Fetched: August 20th, 2021, 5:01am GMT by Waleed Iftikhar

2021 Aug 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

Spinal dysraphism encompasses congenital problems that result in an abnormal bony formation of the spine and/or the spinal cord. It is caused by the maldevelopment of the ectodermal, mesodermal, and neuroectodermal tissues. The two major types of spinal dysraphism are based on the appearance, i.e., aperta (open) if the lesion is visible and occulta (closed) if the lesion is not visible on the surface. Common manifestations are meningocele, myelomeningocele, lipomeningocele, lipomyelomenigocele, myeloschisis, and rachischisis.

This condition also correlates with cutaneous conditions including port-wine stain, hemangioma, hypertrichosis, fibroma pendulum, pigmentary nevus, lipoma, dermal sinus, and deviation of the gluteal furrow. Myelomeningocele is a spinal dysraphism in which the spinal cord and its contents herniate through a congenital bony defect at the posterior elements of the spine, in most cases, the spinous process. It falls under the aperta category and is also known as an open neural tube defect. This is a serious malformation and is associated with a high mortality rate. Myelomeningocele is the most common presentation of spinal dysraphism and constitutes about 80% of the cases.

PMID:32491654 | Bookshelf:NBK557722

Cerebral Cavernous Malformations

Fetched: August 20th, 2021, 5:01am GMT by Michael T. Caton

2021 Aug 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

Cerebral cavernous malformations (CCMs) are abnormally large collections of "low flow" vascular channels without brain parenchyma intervening between the sinusoidal vessels. McCormick (1966) recognized CCMs as one of the four classes of cerebral vascular malformations which include arteriovenous malformations (AVM), developmental venous anomalies (DVA), and capillary telangiectasia. Clinically, CCMs are highly variable in both symptomatic presentation and natural history. Adding to the confusion, CCM has assumed a variety of names in the medical literature including cavernomas, cavernous angiomas, and cavernous hemangiomas, though CCM is the preferred nomenclature. CCMs range in size from punctate to several centimeters in diameter and may occur anywhere in the central nervous system with up to 20% located in the brainstem.

CCM may be diagnosed in both young children and adults and may develop de novo or even regress spontaneously during a patient’s lifetime. A thorough understanding of the natural history of this entity is of paramount importance to avoid unnecessary and potentially morbid interventions. Given the heterogeneity of this condition, the ontogenesis, diagnosis, management strategies for CCMs are subjects of ongoing debate among neuroscientists and treatment paradigms continue to evolve.

PMID:30844171 | Bookshelf:NBK538144

Angiodysplasia

Fetched: August 20th, 2021, 5:01am GMT by Maryam Aghighi

2021 Aug 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.

ABSTRACT

The gastrointestinal (GI) tract is the most frequent site in the body for several types of abnormal blood vessels, which represent irregularities in the normal form of the affected arteries, veins, or capillaries. Vascular abnormalities can be benign or malignant such as hemangiomas or angiosarcomas, respectively. Also, they can be congenital, such as hereditary hemorrhagic telangiectasia or mostly acquired, such as angiodysplasia. Angiodysplasia is an abnormal, tortuous, dilated small blood vessel in the mucosal and submucosal layers of the GI tract. The abnormal vessels consist of endothelium with scant or no smooth muscle. Angiodysplasia is the most frequent etiology of small bowel bleeding or formerly called obscure gastrointestinal bleeding (OGIB) in patients more than 60 years of age. Angiodysplasia is not related to any hereditary, skin, or systemic disease and can affect any part of the GI tract.

PMID:31747184 | Bookshelf:NBK549777

Treatment and management of orbital tumors

Fetched: August 19th, 2021, 5:01am GMT by Michael Zimbelmann

Ophthalmologe. 2021 Aug 18. doi: 10.1007/s00347-021-01471-9. Online ahead of print.

ABSTRACT

BACKGROUND: There are various options for the conservative treatment of the most frequent orbital tumors. These can delay, complement or be superior to the surgical approach, which is often prone to complications.

OBJECTIVE: This article gives a summary of the possible treatment options for the most common orbital tumors in childhood and adulthood.

METHODS: A literature search was carried out and the possible treatment pathways are presented.

RESULTS: 1. Frequent orbital tumors in childhood: a systemic treatment with noncardioselective beta blockers is the primary treatment for capillary orbital hemangiomas. In cases of no response, steroids, interferon alpha or cyclophosphamide are treatment options. Observation is a possible option for smaller dermoid cysts, in cases of progression excision can become necessary. Symptomatic optic nerve gliomas can also be observed and in cases of progression treated with chemotherapy, mTOR/MEK inhibitors or radiotherapy (children > 5 years). Rhabdomyosarcomas are biopsied and subsequently treated by radiotherapy and chemotherapy. 2. Frequent orbital tumors in adulthood: asymptomatic cases of cavernous hemangiomas of the orbit can just be observed. Symptomatic hemangiomas can be surgically excised or treated with radiotherapy. For meningiomas of the optic nerve sheath radiotherapy is a very effective treatment. Surgical excision should be reserved for cases with no prognosis of visual acuity. There is also the option to treat with antiprogesterone. Orbital lymphomas with purely orbital involvement can be treated with radiotherapy, chemotherapy or the application of rituximab.

CONCLUSION: There are now very effective conservative treatment options for many orbital tumors. In some cases a surgical procedure can be avoided and a good visual function can be retained.

PMID:34406460 | DOI:10.1007/s00347-021-01471-9

Permalink for 'Therapeutic Evaluation and Management Strategy of Transarterial Embolization for Giant Liver Hemangiomas Exceeding 10 cm in Diameter'

Therapeutic Evaluation and Management Strategy of Transarterial Embolization for Giant Liver Hemangiomas Exceeding 10 cm in Diameter

Fetched: August 19th, 2021, 5:01am GMT by Xin Li

Cardiovasc Intervent Radiol. 2021 Aug 18. doi: 10.1007/s00270-021-02897-z. Online ahead of print.

NO ABSTRACT

PMID:34406427 | DOI:10.1007/s00270-021-02897-z

Fundus Autofluorescence in Juxtapapillary Choroidal Hemangioma

Fetched: August 19th, 2021, 5:01am GMT by Kuan-Jen Chen

JAMA Ophthalmol. 2021 Aug 1;139(8):e211948. doi: 10.1001/jamaophthalmol.2021.1948. Epub 2021 Aug 18.

NO ABSTRACT

PMID:34406337 | DOI:10.1001/jamaophthalmol.2021.1948

Is endoscopic resection a useful technique for a cavernous sinus sellar cavernoma? A case report and literature review

Fetched: August 19th, 2021, 5:01am GMT by Franco Rubino

Br J Neurosurg. 2021 Aug 18:1-8. doi: 10.1080/02688697.2021.1958154. Online ahead of print.

ABSTRACT

BACKGROUND: Cavernous sinus cavernous malformations (CSCMs) is a vascular malformation of the cavernous sinus. Nowadays, there is an increasing preference to withhold using the terms cavernoma or cavernous hemangioma in order to stop considering these lesions as vascular neoplasms. These lesions are highly vascularized making surgical resection a challenge, mainly in endoscopic approaches. We present a case of this tumor treated in our institution with an endoscopic endonasal approach and incomplete resection. Because of the strenuous resection through this approach, we systematically reviewed the reported endoscopic cases of CSCMs to determine their intraoperative complications, results and tumor features.

METHODS: Using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, one database (PubMed) and crossed references were queried for CSCMs from 1948 to 2020. Data regarding demographic features, clinical presentation, MRI features, surgical results and overall pathology features extracted.

RESULTS: Eighteen patients were selected (including our case). The mean age was 50.4 ± 14 years. Pituitary dysfunction and cavernous sinus nerve compression were the most reported symptoms. Only five cases (27%) reported a gross total resection (GTR) through endoscopic endonasal approach. Intraoperative bleeding was the most frequent intraoperative complication.

CONCLUSION: We present a comprehensive analysis of every reported CSCM treated through endoscopic approach. Partial or subtotal resection are the most used techniques because of the intraoperative bleeding and the adherence to surrounding structures. Radiotherapy is a very good option for patients with incomplete resections.

PMID:34406095 | DOI:10.1080/02688697.2021.1958154

Port-wine stains and Sturge-Weber syndrome: clinicopathological analysis and molecular characterization

Fetched: August 19th, 2021, 5:01am GMT by Y Z Kan

Zhonghua Bing Li Xue Za Zhi. 2021 Jul 8;50(7):802-804. doi: 10.3760/cma.j.cn112151-20210425-00316.

NO ABSTRACT

PMID:34405619 | DOI:10.3760/cma.j.cn112151-20210425-00316

A retrospective study of hemophilic pseudotumor in maxillofacial region

Fetched: August 19th, 2021, 5:01am GMT by G X Yu

Zhonghua Kou Qiang Yi Xue Za Zhi. 2021 Aug 9;56(8):785-790. doi: 10.3760/cma.j.cn112144-20210325-00141. Online ahead of print.

ABSTRACT

Objective: To provide clinical references for the diagnosis and treatment of hemophilic pseudotumor (HPT) in maxillofacial region. Methods: Fourteen cases of HPT in maxillofacial region from the Department of Stomatology, Beijing Children's Hospital from Jan 2009 to Jan 2019 were collected. Two cases were lost for follow-up and 12 patient,all boys, were finally followed up and included in the study. The patients aged from 13 months to 10 years old. The medical history, clinic manefestitions and the features of the radiology examination were recorded. The patients were treated by using replacement treatment first. If the conservative treatment was not effective, the patients then received operation combined with pereoperation replacement thearapy. The patients were followed up for 13 months to 10 years.There were 11 cases of hemophilia A, and 1 case of hemophilia B. Two cases were severe type, the others (10/12) were mild and moderate types. Only 1 case was diagnosed as hemophilia initially. Nine cases (9/12) were misdiagnosed as malignant tumors, 1 case was misdiagnosed as osteomyelitis and 1 case was misdiagnosed as hemangioma. Only 3 cases had identified history of trauma before. Results: All cases were treated with replacement therapy first, among which 10 cases were effective, 8 cases were cured by conservative therapy, 1 case had residual soft tissue fistula after conservative treatment and 1 case recurrented after conservative treatment for 8 months. Two patients with poor efficacy to the replacement treatment were performed operations and finally were cured. Conclusions: The misdiagnosis rate of HPT in maxillofacial region was high. The conservative factor replacement therapy could achieve good results in most children and could be used as the preferred treatment. If the conservative treatment was not effective, the surgical treatment was also a safe option.

PMID:34404145 | DOI:10.3760/cma.j.cn112144-20210325-00141

Permalink for 'Percutaneous Sclerotherapy with Bleomycin and Ethiodized Oil: A Welcomed Minimally Invasive Treatment for Giant Liver Hemangiomas'

Percutaneous Sclerotherapy with Bleomycin and Ethiodized Oil: A Welcomed Minimally Invasive Treatment for Giant Liver Hemangiomas

Fetched: August 18th, 2021, 5:01am GMT by John P McGahan

Radiology. 2021 Aug 17:211594. doi: 10.1148/radiol.2021211594. Online ahead of print.

NO ABSTRACT

PMID:34402673 | DOI:10.1148/radiol.2021211594

Percutaneous Sclerotherapy with Bleomycin and Ethiodized Oil: A Promising Treatment in Symptomatic Giant Liver Hemangioma

Fetched: August 18th, 2021, 5:01am GMT by Niloofar Ayoobi Yazdi

Radiology. 2021 Aug 17:204444. doi: 10.1148/radiol.2021204444. Online ahead of print.

ABSTRACT

Background Percutaneous sclerotherapy with bleomycin has been proven to have a potential benefit in the management of low-flow venous malformations. Liver hemangiomas are considered low-flow venous malformations. Thus, percutaneous sclerotherapy could potentially have a promising result in their management. Purpose To investigate the feasibility, efficacy, and safety of percutaneous sclerotherapy with bleomycin in the management of symptomatic giant liver hemangioma (GLH). Materials and Methods This single-institute prospective study was conducted between September 2018 and July 2020. Percutaneous sclerotherapy was performed using a mixture of bleomycin and ethiodized oil under guidance of US and fluoroscopy in participants with GLH who were experiencing related abdominal pain or fullness. Technical success was recorded. Change in symptom severity, according to visual analog scale (VAS), was considered the primary outcome of the study. Volume change, based on the lesion volume at CT, and complications, based on the classification of the Society of Interventional Radiology, were regarded as secondary outcomes. The primary and secondary outcomes were recorded 6 and 12 months after the procedure. Comparison was performed by using the Wilcoxon signed-rank test or paired t test. Results Twenty-eight participants (mean age, 45 years ± 9; 25 women) were evaluated. Technical success was 100%. The mean VAS score was 8.3 before the procedure, which decreased to 1.4 (84.7% reduction) and 1.5 (83.5% reduction) at 6- and 12-month follow-ups, respectively (P < .001 for both). All participants reported relief of symptoms (17 of 28 participants [61%] with complete relief; 11 [39%] with partial relief) at 12-month follow-up. Mean GLH volumes dropped from 856.3 cm³ to 309.8 cm³ (65.7% reduction) and 206.0 cm³ (76% reduction) at 6- and 12-month follow-ups, respectively (P < .001 for both). No major complications were detected. Conclusion Percutaneous sclerotherapy is a safe and feasible method with promising results in the treatment of patients with symptomatic giant liver hemangioma. Clinical trial registration no. NCT03649113 © RSNA, 2021 See also the editorial by McGahan and Goldman in this issue.

PMID:34402664 | DOI:10.1148/radiol.2021204444

Surgical treatment of conjunctival hemangioma and hemangiosarcoma: A retrospective study of 52 dogs

Fetched: August 18th, 2021, 5:01am GMT by Sarah Richardson

Vet Ophthalmol. 2021 Aug 17. doi: 10.1111/vop.12921. Online ahead of print.

ABSTRACT

OBJECTIVE: To review cases of canine conjunctival hemangioma (HA) and hemangiosarcoma (HSA) treated surgically at a referral center to establish success of surgical management, recurrence rates, and long-term outcomes for patients.

ANIMALS STUDIED: Retrospective record review of dogs that underwent surgery to remove histologically diagnosed conjunctival HA or HSA between April 2004 and April 2020 to collect data on signalment, tumor location, interval between initial presentation and surgery, tumor diagnosis, surgical dose, surgical margins, tumor size, recurrence and survival times.

RESULTS: A total of 52 dogs (60 tumors) were included. The mean age of affected dogs was 8.69 years; the most affected breed was the Border collie (n = 13, 25%). 28 tumors were HA (46.67%) and 32 HSA (53.33%). Tumors occurred in three locations: the lateral bulbar conjunctiva (n = 37, 61.67%), the third eyelid margin (n = 19, 31.67%), and the ventral conjunctival fornix (n = 4, 6.67%). There was no site predilection for HA versus HSA. 97% of tumors occurred in non-pigmented tissue. Corneal invasion was more likely to be a feature of malignant tumors. Five tumors were incompletely excised, one of which recurred. There was no statistical difference in likelihood of incomplete excision between HSA and HA. Six tumors (10%) recurred. HSA was not statistically more likely to recur than HA. Recurrence times ranged from 5 weeks to 1 year.

CONCLUSION: Surgical treatment of conjunctival HA and HSA is likely to be curative. There is a recurrence rate of 10% regardless of tumor type, and recurrence may be late in the course of the disease.

PMID:34402571 | DOI:10.1111/vop.12921

Kaposiform Hemangioendothelioma further broadens the phenotype of PIK3CA-related overgrowth spectrum

Fetched: August 18th, 2021, 5:01am GMT by Diana Carli

Clin Genet. 2021 Aug 17. doi: 10.1111/cge.14047. Online ahead of print.

ABSTRACT

Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive mixed vascular tumor, with typical onset in early childhood and characterized by progressive angio- and lymphangiogenesis. Its etiopathogenesis and molecular bases are still unclear. Here, we report the first case of congenital KHE harboring a PIK3CA mosaic pathogenic variant (c.323G > A, p.Arg108His) in a boy with very subtle PIK3CA-related overgrowth spectrum (PROS) features. This finding provides insights into the pathophysiology of KHE, offering targeted therapeutic options by inhibition of the PI3K/Akt/mTOR pathway. We propose the inclusion of this mixed lymphatic and vascular anomaly within the PROS.

PMID:34402524 | DOI:10.1111/cge.14047

A Rare Presentation of Lisegang Rings in Adrenal Cavernous Hemangioma : Case Report and Literature Review

Fetched: August 18th, 2021, 5:01am GMT by Wejdan Almotairi

Case Rep Med. 2021 Aug 3;2021:9998729. doi: 10.1155/2021/9998729. eCollection 2021.

ABSTRACT

BACKGROUND: Adrenal cavernous hemangiomas (AH) are benign nonfunctional vascular tumors rarely discovered as incidental findings on imaging studies or autopsies. This study presents a single case report of AH with another rare finding of the Liesegang ring. Also, we reviewed 73 case reports of cavernous adrenal hemangioma to provide an overview of AH's clinical characteristics. Case Report. A nonfunctional AH was incidentally discovered in a 59-year-old morbidly obese female patient with a 10-year history of hypertension and thyroidectomy. An abdominal computed tomography (CT) scan showed a left suprarenal mass of ∼16 cm in diameter. While the patient had no clinical manifestations from the hemangioma, all laboratory tests were within the normal values with no indication of a functional adrenal tumor. The mass was removed by open left adrenalectomy. The microscopic histological examination revealed a laminated structure with wide blood-filled spaces with a central core of necrotic and hemorrhagic changes, characteristic of a cavernous AH with the presence of a rare Liesegang ring.

CONCLUSION: Although rare, AH should be considered as a differential diagnosis for adrenal masses. This is the first reported case of a cavernous AH with rare microscopic findings of the Liesegang ring.

PMID:34400913 | PMC:PMC8364425 | DOI:10.1155/2021/9998729

Permalink for '15,16-dihydrotanshinone I inhibits EOMA cells proliferation by interfering in posttranscriptional processing of hypoxia-inducible factor 1'

15,16-dihydrotanshinone I inhibits EOMA cells proliferation by interfering in posttranscriptional processing of hypoxia-inducible factor 1

Fetched: August 18th, 2021, 5:01am GMT by Peiwen Duan

Int J Med Sci. 2021 Jul 11;18(14):3214-3223. doi: 10.7150/ijms.60774. eCollection 2021.

ABSTRACT

Infantile hemangioma (IH), which threatens the physical and mental health of patients, is the most common benign tumor in infants. Previously, we found that 15,16-dihydrotanshinone I (DHTS) was significantly more effective at inhibiting hemangioma proliferation in vitro and in vivo than the first-line treatment propranolol. To investigate the underlying mechanism of DHTS, we used EOMA cells as a model to study the effect of DHTS. We compared the transcriptomes of control and DHTS-treated EOMA cells. In total, 2462 differentially expressed genes were detected between the groups. Kyoto Encyclopedia of Genes and Genomes pathway analysis revealed downregulated activity of the hypoxia-inducible factor 1 alpha (HIF-1α) signaling pathway in EOMA cells following treatment with DHTS. Thus, we investigated HIF-1α expression at protein and mRNA levels. Our results revealed that DHTS downregulated HIF-1α expression by interfering in its posttranscriptional processing, and the RNA-binding protein HuR participated in this mechanism. Our findings provide a basis for clinical transformation of DHTS and insight into pathogenic mechanisms involved in IH.

PMID:34400891 | PMC:PMC8364454 | DOI:10.7150/ijms.60774

Extradural lumbar nerve root and ganglion capillary hemangioma: case report

Fetched: August 18th, 2021, 5:01am GMT by Sophia Peng

Spinal Cord Ser Cases. 2021 Aug 17;7(1):74. doi: 10.1038/s41394-021-00438-x.

ABSTRACT

INTRODUCTION: Extradural spinal hemangiomas without vertebral body or intradural involvement are rare and often misdiagnosed. When present in the lumbar spine, they typically present with radiculopathy and weakness. CT imaging is helpful in assessing for bony involvement and temporal involvement while MRI imaging can be helpful in distinguishing hemangiomas from other mass lesions, however current reports on imaging features are limited. Diagnosis remains primarily dependent on tissue pathology with surgery as the mainstay of treatment.

CASE PRESENTATION: We present a unique case report in which we obtain additional DSA imaging to not only visualize the vascular anatomy associated with a L4-5 neuroforaminal capillary hemangioma involving the L4 nerve root and ganglion, but to also embolize the feeding artery prior to surgical resection. Patient initially underwent a CT-guided biopsy that was suspicious for a hemangioma and confirmed on final pathology to be the capillary subtype.

DISCUSSION: When diagnosed appropriately, patients avoid unnecessary additional testing and avoid erroneous treatment of this rare lesion. Based on our experience, we propose initial MRI imaging to characterize the hemangioma and evaluate for intradural involvement, DSA to assess vascularity followed by embolization of the lesion when able in order to minimize intra-operative hemorrhage risk, and ultimately surgery to achieve a gross total resection.

PMID:34400608 | DOI:10.1038/s41394-021-00438-x

Permalink for 'Isolated juxtapapillary retinal capillary haemangioblastoma and exudative maculopathy in an elderly patient without von Hippel-Lindau syndrome: combined proton beam radiotherapy and intravitreal anti-VEGF'

Isolated juxtapapillary retinal capillary haemangioblastoma and exudative maculopathy in an elderly patient without von Hippel-Lindau syndrome: combined proton beam radiotherapy and intravitreal anti-VEGF

Fetched: August 18th, 2021, 5:01am GMT by Ahmed Adan

BMJ Case Rep. 2021 Aug 16;14(8):e244077. doi: 10.1136/bcr-2021-244077.

ABSTRACT

A 71-year-old Caucasian man presented with an isolated juxtapapillary retinal capillary haemangioblastoma on the superior and temporal left optic disc with active exudation resulting in macular intraretinal and subretinal fluid, reduced vision, scotoma and distortion with progression over 6 weeks. He did not have von Hippel-Lindau syndrome. After proton beam radiotherapy (PBR), the tumour size remained unchanged, but did not stop the exudation. Three anti-vascular endothelial growth factor (VEGF) (ie, bevacizumab) injections at monthly intervals resulted in reduced macular oedema. Combined therapy with PBR and anti-VEGF injections sustained our patient's vision at 12 months follow-up.

PMID:34400427 | DOI:10.1136/bcr-2021-244077

Aberrant PTEN, PIK3CA, pMAPK, and TP53 expression in human scalp and face angiosarcoma

Fetched: August 17th, 2021, 5:01am GMT by Huiying Wan

Medicine (Baltimore). 2021 Jul 30;100(30):e26779. doi: 10.1097/MD.0000000000026779.

ABSTRACT

Angiosarcoma is a rare, highly aggressive malignant tumor originating from endothelial cells that line the lumen of blood or lymphatic vessels. The molecular mechanisms of scalp and face angiosarcoma still need to be elucidated. This study aimed to investigate the expression of phosphatase and tensin homolog (PTEN), phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA), phosphorylated mitogen-activated kinase-like protein (pMAPK), and tumor protein p53 (TP53) in scalp and face angiosarcoma and to assess tumor tissue apoptosis.The expression and intracellular distribution of PTEN, PIK3CA, pMAPK, and TP53 proteins in 21 specimens of human scalp and face angiosarcoma and 16 specimens of human benign hemangioma were evaluated using immunohistochemistry. Tumor cell apoptosis was assessed by terminal deoxyribonucleotide transferase-mediated dUTP nick end-labeling staining.Significantly lower PTEN but higher PIK3CA, pMAPK, and TP53 immunostaining were detected in the angiosarcoma specimens than in the benign hemangioma specimens(P < .01). The angiosarcoma tissues exhibited significantly higher apoptosis indices than the benign hemangioma tissues (P < .01). The positive expression rates of PIK3CA, pMAPK, and TP53 were correlated with the degree of tumor differentiation in the human scalp and face angiosarcoma.The PI3K, MAPK, and TP53 pathways might be involved in angiosarcoma tumorigenesis in humans and may serve as therapeutic targets for the effective treatment of this malignancy.

PMID:34397726 | PMC:PMC8322557 | DOI:10.1097/MD.0000000000026779

Infantile Hemangiomas Cleared by Combined Therapy With Pulsed Dye Laser and Propranolol

Fetched: August 17th, 2021, 5:01am GMT by Atsuko Sugimoto

Dermatol Surg. 2021 Aug 1;47(8):1052-1057. doi: 10.1097/DSS.0000000000003018.

ABSTRACT

BACKGROUND: Oral propranolol is the first-line therapy for infantile hemangioma. Combining it with pulse dye laser (PDL) (595nm-long PDL) could reduce treatment duration and sequelae incidence and severity.

OBJECTIVE: To determine the effect of PDL-propranolol treatment on duration to cure and sequelae.

METHODS: All consecutive patients with infantile hemangioma who were cured by PDL-propranolol treatment were identified.

RESULTS: In the 27 cases, average age at treatment start was 4.3 ± 3.8 months, mean tumor diameter was 11.1 ± 14.0 cm2, and tumor-type was most common (72.4% of lesions). The patients received 9.8 ± 10.5 PDL sessions. After ensuring patients had no physical contraindications, including heart disease, oral propranolol was started at 1 mg/kg/d, increased up to 3 mg/kg/d as a maintenance dose. Mean propranolol treatment duration was 11.1 ± 4.9 months. Total treatment duration was 15.3 ± 10.8 months.

CONCLUSION: Our data in the context of recent literature suggest combining propranolol with PDL may reduce propranolol duration without increasing harms.

PMID:34397540 | DOI:10.1097/DSS.0000000000003018

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