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Hemangioendothelioma (127 unread)

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Efficacy and Safety of Different Concentrations of Sirolimus in the Treatment of Kaposiform Hemangioendothelioma.

Posted: March 1st, 2021, 3:00pm GMT

Condition: Kaposiform Hemangioendothelioma
Intervention: Drug: Sirolimus
Sponsor: West China Hospital
Recruiting

Tacrolimus for the Treatment of Superficial Kaposiform Hemangioendothelioma and Tufted Angioma

Posted: August 14th, 2019, 2:00pm GMT

Condition: Tacrolimus, Kaposiform Hemangioendothelioma, Tufted Angioma
Intervention: Drug: Tacrolimus ointment
Sponsor: West China Hospital
Recruiting

Permalink for 'Case report: Intraosseous hemangioma of the lateral clavicle treated by surgical resection and reconstructed by three-dimensional-printed personalized prosthesis'

Case report: Intraosseous hemangioma of the lateral clavicle treated by surgical resection and reconstructed by three-dimensional-printed personalized prosthesis

Fetched: January 27th, 2023, 5:01am GMT by Zhuangzhuang Li

Front Bioeng Biotechnol. 2023 Jan 9;10:1085674. doi: 10.3389/fbioe.2022.1085674. eCollection 2022.

ABSTRACT

Background: Intraosseous hemangiomas occurring the clavicle is uncommon. Reconstruction of the clavicle is suggested to maintain the normal shoulder joint function and prevent adverse outcomes. Complex anatomy shape of the clavicle remains a great challenge for prosthetic reconstruction of the clavicle. Case presentation: A 37-year-old female with no conclusive history of trauma presented with progressive mass at the right lateral clavicle for 5 years. The patient was treated by surgical resection and reconstructed by three-dimensional-printed personalized prosthesis. Postoperatively radiographic examinations revealed a good position of the prosthesis, neither breakage nor loosening was detected. The right shoulder mobility returned to approximate level of preoperative shoulder 2 months after surgical reconstruction, with the range of motion of flexion 80°, extension 40°, abduction 80°, adduction 30°, external rotation 55°, and internal rotation 60°. The patient maintained the normal shoulder function during the 48 months follow-up period. There was no pain during shoulder motion. The Musculoskeletal Tumor Society Score (MSTS) score was 29 and the Functional Evaluation Form recommended by the American Shoulder and Elbow Surgeons (ASES) score was 95. Conclusion: 3D-printed personalized prosthesis is a good option to reconstruct the lateral clavicle bone defect and restore the shoulder support structure. It maintains the normal shoulder joint function and avoids adverse effects on daily activities after claviculectomy.

PMID:36698635 | PMC:PMC9868392 | DOI:10.3389/fbioe.2022.1085674

Poor response to sintilimab plus chemotherapy in a pulmonary epithelioid hemangioendothelioma patient: a case report

Fetched: January 26th, 2023, 5:01am GMT by Hao Zeng

Immunotherapy. 2023 Jan 25. doi: 10.2217/imt-2022-0073. Online ahead of print.

ABSTRACT

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with no established treatment protocol. The authors report the case of a young woman diagnosed with PEH. DNA and RNA analysis by next-generation sequencing was performed on the tumor tissue. A novel germline PALB2 mutation and classical WWTR1-CAMTA1 fusion were identified. She experienced a poor response to sintilimab (a PD-1 inhibitor) plus platinum-based chemotherapy as the first-line treatment. PEH patients harboring a germline PALB2 mutation and WWTR1-CAMTA1 gene fusion may respond poorly to treatment with PD-1 inhibitors plus chemotherapy.

PMID:36695105 | DOI:10.2217/imt-2022-0073

Pyogenic granuloma of the colon observed by magnifying endoscopy and treated by endoscopic submucosal dissection

Fetched: January 25th, 2023, 5:01am GMT by Keisuke Kawasaki

Gastrointest Endosc. 2023 Jan 21:S0016-5107(23)00046-9. doi: 10.1016/j.gie.2023.01.032. Online ahead of print.

NO ABSTRACT

PMID:36693500 | DOI:10.1016/j.gie.2023.01.032

Childhood Liver Cancer Treatment (PDQ®): Health Professional Version

Fetched: January 25th, 2023, 5:01am GMT by PDQ Pediatric Treatment Editorial Board

2023 Jan 19. 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 liver cancer. It is intended as a resource to inform and assist clinicians in the care of their 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:26389232 | Bookshelf:NBK65790

Permalink for 'Study protocol for a multicentre nationwide prospective cohort study to investigate the natural course and clinical outcome in benign liver tumours and cysts in the Netherlands: the BELIVER study'

Study protocol for a multicentre nationwide prospective cohort study to investigate the natural course and clinical outcome in benign liver tumours and cysts in the Netherlands: the BELIVER study

Fetched: January 24th, 2023, 5:01am GMT by Alicia Furumaya

BMJ Open. 2022 Sep 8;12(9):e055104. doi: 10.1136/bmjopen-2021-055104.

ABSTRACT

INTRODUCTION: Benign liver tumours and cysts (BLTCs) comprise a heterogeneous group of cystic and solid lesions, including hepatic haemangioma, focal nodular hyperplasia and hepatocellular adenoma. Some BLTCs, for example, (large) hepatocellular adenoma, are at risk of complications. Incidence of malignant degeneration or haemorrhage is low in most other BLTCs. Nevertheless, the diagnosis BLTC may carry a substantial burden and patients may be symptomatic, necessitating treatment. The indications for interventions remain matter of debate. The primary study aim is to investigate patient-reported outcomes (PROs) of patients with BLTCs, with special regards to the influence of invasive treatment as compared with the natural course of the disease.

METHODS AND ANALYSIS: A nationwide observational cohort study of patients with BLTC will be performed between October 2021 and October 2026, the minimal follow-up will be 2 years. During surveillance, a questionnaire regarding symptoms and their impact will be sent to participants on a biannual basis and more often in case of invasive intervention. The questionnaire was previously developed based on PROs considered relevant to patients with BLTCs and their caregivers. Most questionnaires will be administered by computerised adaptive testing through the Patient-Reported Outcomes Measurement Information System. Data, such as treatment outcomes, will be extracted from electronic patient files. Multivariable analysis will be performed to identify patient and tumour characteristics associated with significant improvement in PROs or a complicated postoperative course.

ETHICS AND DISSEMINATION: The study was assessed by the Medical Ethics Committee of the University Medical Center Groningen and the Amsterdam UMC. Local consultants will provide information and informed consent will be asked of all patients. Results will be published in a peer-reviewed journal.

STUDY REGISTRATION: NL8231-10 December 2019; Netherlands Trial Register.

PMID:36691222 | PMC:PMC9462085 | DOI:10.1136/bmjopen-2021-055104

Vascular Malformations: A Histopathologic and Conceptual Appraisal

Fetched: January 24th, 2023, 5:01am GMT by A Fernandez-Flores

Actas Dermosifiliogr. 2023 Jan 20:S0001-7310(23)00044-3. doi: 10.1016/j.ad.2022.07.032. Online ahead of print.

ABSTRACT

In the field of vascular anomalies, distinguishing between vascular malformations and tumors has become crucial for a correct therapeutic approach. However, the differential diagnosis between these two groups is not always well explained in classical texts, mainly because many vascular malformations are still known with old names that suggest a tumoral nature. Also, genetic and pathogenic knowledge of these entities has greatly increased in recent decades, so researchers and clinicians now have a better understanding of vascular malformations. In this paper, we present the main histopathological tips to recognize and identify a vascular malformation as such. We also contextualize such information in the clinical and pathogenic knowledge for a better understanding of these entities.

PMID:36690149 | PMC:PMC9854208 | DOI:10.1016/j.ad.2022.07.032

Vascular malformations: a histopathologic and conceptual appraisal

Fetched: January 24th, 2023, 5:01am GMT by A Fernandez-Flores

Actas Dermosifiliogr. 2023 Jan 20:S0001-7310(23)00045-5. doi: 10.1016/j.ad.2022.10.039. Online ahead of print.

ABSTRACT

PMID:36690143 | DOI:10.1016/j.ad.2022.10.039

Progressive kaposiform hemangioendothelioma and sirolimus-related severe thrombocytopenia

Fetched: January 24th, 2023, 5:01am GMT by Hanru Ying

Indian J Dermatol Venereol Leprol. 2022 Dec 13:1-6. doi: 10.25259/IJDVL_799_19. Online ahead of print.

ABSTRACT

Kaposiform hemangioendothelioma is a locally invasive tumor and we were unable to find any previous reports of multifocal progression. Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. Herein, we report a case of multifocal progressive kaposiform hemangioendothelioma, wherein sirolimus treatment caused severe thrombocytopenia. A 12-year-old East Asian girl presented with indurated dark-purple masses on her back. The patient had received three surgical interventions following the first appearance of the masses in 2012 and subsequent reappearances in 2014 and 2016. Kaposiform hemangioendothelioma was diagnosed based on radiological and pathological findings. Two more masses appeared in the following year. The patient was treated with oral sirolimus (2.5 mg/ m2/day) and developed grade 3 thrombocytopenia 8 days later. The patient was uneventfully relieved 5 days later after the withdrawal of sirolimus and the administration of appropriate medications. This rare case indicated that kaposiform hemangioendothelioma could be progressive with local metastatic characteristics in children. Besides, the severe sirolimus-induced complication highlights the importance of serum drug level monitoring during treatment. Physicians should be extremely cautious while treating kaposiform hemangioendothelioma patients with sirolimus.

PMID:36688881 | DOI:10.25259/IJDVL_799_19

Permalink for 'Letter to editor regarding "Clinical practice guidelines on management of infantile hemangioma: a systematic quality appraisal using the AGREE II instrument"'

Letter to editor regarding "Clinical practice guidelines on management of infantile hemangioma: a systematic quality appraisal using the AGREE II instrument"

Fetched: January 24th, 2023, 5:01am GMT by Ilona J Frieden

Pediatr Hematol Oncol. 2023 Jan 23:1-2. doi: 10.1080/08880018.2023.2166632. Online ahead of print.

NO ABSTRACT

PMID:36688573 | DOI:10.1080/08880018.2023.2166632

Permalink for 'Anastomosing Hemangioma of Ovary with Stromal Luteinization Masquerading as Sex Cord-Stromal Tumor on Intraoperative Consultation'

Anastomosing Hemangioma of Ovary with Stromal Luteinization Masquerading as Sex Cord-Stromal Tumor on Intraoperative Consultation

Fetched: January 24th, 2023, 5:01am GMT by Shivani Jha

J Microsc Ultrastruct. 2022 Aug 4;10(4):208-210. doi: 10.4103/jmau.jmau_92_21. eCollection 2022 Oct-Dec.

ABSTRACT

Anastomosing hemangiomas (AHs) are benign vascular tumor with rare occurrence in the ovary and the majority being asymptomatic. We report one such case of AH with stromal luteinization. A 35-year-old female had complaints of abdominal pain and heavy menstrual bleeding for 2 years. Her lactate dehydrogenase was markedly raised. Following the clinical suspicion of germ cell tumor, exploratory laparotomy and right salpingo-oophorectomy were done. On frozen section due to extensive stromal luteinization, diagnosis of sex cord-stromal tumor was suggested. However, the case was finally diagnosed with AH with extensive stromal luteinization. This case highlighted the potential mimics of AH due to coexistent raised biomarkers and secondary changes, thereby posing a diagnostic dilemma on intraoperative consultation.

PMID:36687324 | PMC:PMC9846922 | DOI:10.4103/jmau.jmau_92_21

The case of a liver adenoma that looked like a hemangioma

Fetched: January 24th, 2023, 5:01am GMT by Andrea Boccatonda

Radiol Case Rep. 2023 Jan 5;18(3):1024-1028. doi: 10.1016/j.radcr.2022.11.073. eCollection 2023 Mar.

ABSTRACT

Benign focal liver lesions are among the most frequent findings on ultrasound. Liver hemangiomas are often easily recognizable on ultrasound examination and are characterized by a clear benign prognosis. In some cases, hemangiomas display an atypical appearance both on B-mode and on contrast-enhanced ultrasound, thus raising concerns for differential diagnosis both with adenomas and with lesions of malignant nature. We report here the case of a patient who presented with a liver lesion with all signs suggestive for hemangioma on B-mode, but it showed atypical features on contrast-enhanced ultrasound examination and final diagnosis of liver adenoma.

PMID:36684629 | PMC:PMC9849955 | DOI:10.1016/j.radcr.2022.11.073

Initial experience with robotic-assisted thoracic surgery for superior mediastinal masses

Fetched: January 24th, 2023, 5:01am GMT by Bo Yang

Front Surg. 2023 Jan 6;9:1043525. doi: 10.3389/fsurg.2022.1043525. eCollection 2022.

ABSTRACT

OBJECTIVE: Minimally invasive surgery is challenging for masses located in the superior mediastinum, especially for those close to the chest outlet. This study aimed to evaluate the feasibility and safety of robotic-assisted thoracic surgery (RATS) for these masses.

METHODS: From June 2015 to January 2020, 35 patients (19 males, 16 females), with a mean age of 41.6 (range, 13-66) years, underwent RATS for the treatment of superior mediastinal masses. Data regarding the operation time, blood loss, pathology, conversion rate, morbidity, mortality, and cost were collected and analyzed.

RESULTS: The mean (±standard deviation) operation time, blood loss, chest tube use duration, and postoperative hospital day were 117 ± 45.2 (range, 60-270) min, 59.7 ± 94.4 (range, 10-500) ml, 4.1 ± 2.1 (range, 1-10) days, and 5.1 ± 2.1 (range, 2-11) days, respectively. The pathological diagnoses included schwannoma (26 cases), ganglioneuroma (4 cases), bronchogenic cysts (3 cases), ectopic nodular goiter (1 case), and cavernous hemangioma (1 case). The mean diameter of the resected tumor was 4.6 ± 2.0 (range, 2.5-10) cm. No conversion or mortality occurred. Postoperative complications included Horner's syndrome (18 cases: 6 patients with preoperative Horner's syndrome), weakened muscular power (2 cases), and chylothorax (2 cases). The mean cost was $ 8,868.7 (range, $ 4,951-15,883).

CONCLUSIONS: Our experience demonstrated that RATS is safe and feasible for superior mediastinal mass resection. However, the high incidence of postoperative Horner's syndrome requires further research.

PMID:36684169 | PMC:PMC9852329 | DOI:10.3389/fsurg.2022.1043525

$Permalink for 'Refractory Chylothorax Secondary to Sizeable Azygos Vein Hemangioma: Tailored Multimodal Treatment of a Challenging Case Report'$

Refractory Chylothorax Secondary to Sizeable Azygos Vein Hemangioma: Tailored Multimodal Treatment of a Challenging Case Report

Fetched: January 22nd, 2023, 5:01am GMT by Paolo Albino Ferrari

Medicina (Kaunas). 2022 Dec 31;59(1):91. doi: 10.3390/medicina59010091.

ABSTRACT

BACKGROUND: Mediastinal hemangiomas are rare, and their etiology remains unclear. Most patients affected have no pathognomonic clinical symptoms, and the diagnosis is often incidental. Due to the paucity of the available literature regarding the management of this disease, the choice and timing of treatment remains controversial.

CASE PRESENTATION: Herein, we report the case of a hemangioma of the azygos vein arch in a 66-year-old woman who presented with dyspnea, chest discomfort, dysphagia, and weight loss. A simultaneous right chylothorax refractory to conservative management was found. A CT-guided biopsy of the mass was performed, and it confirmed the vascular nature of the lesion. Therefore, the patient underwent an angiography followed by endo-vascular embolization. Three days later, thoracoscopic surgical resection of the mass and the repair of the chyle leakage were performed safely. The patient was discharged uneventfully on postoperative day seven, with complete resolution of all the presenting symptoms.

CONCLUSIONS: Treatment of symptomatic mediastinal hemangiomas could be mandatory, but a thorough multidisciplinary approach to these rare malformations is essential. Despite the risk of intraoperative bleeding, selective endovascular embolization followed by thoracoscopic surgery allowed for a complete and safe resection with a good outcome.

PMID:36676715 | PMC:PMC9863118 | DOI:10.3390/medicina59010091

Choroidal Neovascular Membranes in Retinal and Choroidal Tumors: Origins, Mechanisms, and Effects

Fetched: January 22nd, 2023, 5:01am GMT by Federico Di Staso

Int J Mol Sci. 2023 Jan 5;24(2):1064. doi: 10.3390/ijms24021064.

ABSTRACT

Choroidal neovascularizations are historically associated with exudative macular degeneration, nonetheless, they have been observed in nevus, melanoma, osteoma, and hemangioma involving the choroid and retina. This review aimed to elucidate the possible origins of neovascular membranes by examining in vivo and in vitro models compared to real clinical cases. Among the several potential mechanisms examined, particular attention was paid to histologic alterations and molecular cascades. Physical or biochemical resistance to vascular invasion from the choroid offered by Bruch's membrane, the role of fibroblast growth factor 2 and vascular endothelial growth factor, resident or recruited stem-like/progenitor cells, and other angiogenic promoters were taken into account. Even if the exact mechanisms are still partially obscure, experimental models are progressively enhancing our understanding of neovascularization etiology. Choroidal neovascularization (CNV) over melanoma, osteoma, and other tumors is not rare and is not contraindicative of malignancy as previously believed. In addition, CNV may represent a late complication of either benign or malignant choroidal tumors, stressing the importance of a long follow-up.

PMID:36674579 | PMC:PMC9865148 | DOI:10.3390/ijms24021064

Management of a Rare Case of Cavernous Medullary Intraosseous Hemangioma in Proximal Tibia of a 38-year-old Female

Fetched: January 21st, 2023, 5:01am GMT by Amyn M Rajani

J Orthop Case Rep. 2022;12(5):96-100. doi: 10.13107/jocr.2022.v12.i05.2834.

ABSTRACT

INTRODUCTION: Intraosseous hemangiomas (IH) are one of the rarest bone tumors that an orthopedic surgeon comes across, more so in the long bones. It most often affects adult females, severely blunting their activities of daily living, coupled with potential debilitating complications such as pathological fracture.

CASE PRESENTATION: We present a case of a 38-year-old female who presented with long standing pain and swelling in the left knee. Radiology, in the form of plain radiographs and magnetic resonance imaging, narrowed the diagnosis down to a benign form of osteoblastoma or hemangioma. With this in mind, the patient underwent radical excision of the lytic lesion, along with a margin of the surrounding normal bone. The defect was filled with impaction bone grafting and supplemented by a hydroxyapatite block and a poly ethyl ether ketone plate. The patient had excellent clinical, functional, and radiological outcomes at 6 months follow-up.

CONCLUSION: With the inherent rarity of IH, especially when it affects the long bones, it has barely been reported in orthopedic literature. Subsequently, management protocols for such lesions are ill-defined, which can be deleterious to the patient as well as the surgeon. Through this case report, we show how to approach a patient presenting with associated complaints, and show a detailed outline of an efficacious management regime that gave excellent outcomes in our patient.

PMID:36660152 | PMC:PMC9826562 | DOI:10.13107/jocr.2022.v12.i05.2834

Custom Mega Prosthesis in a Rare case of Epithelioid Hemangioendothelioma of Proximal Tibia - A Case Report

Fetched: January 21st, 2023, 5:01am GMT by Sathish Muthu

J Orthop Case Rep. 2022 Jul;12(7):5-9. doi: 10.13107/jocr.2022.v12.i07.2892.

ABSTRACT

INTRODUCTION: Lytic lesions of the proximal tibia include a plethora of differential diagnoses. The most common ones are the Giant cell tumor, fibrous dysplasia, adamantinoma, chondromyxoid fibroma, and osteoblastoma. The rarer ones include vascular tumors such as hemangioma and hemangioendothelioma. A systematic line of investigations is essential to pick up the right diagnosis especially in case of rarer conditions. In this background, we present a case of lytic lesion of the proximal tibia which turned out to be epitheloid hemangioendothelioma (EHE).

CASE REPORT: A 37-year-old female presented with pain and swelling in the left knee for 2 years. On examination, the patient had a 3 × 4 cm firm, non-tender, and well-defined swelling on the anterolateral aspect of the proximal tibia. X-ray showed a lytic lesion of the proximal tibia. Magnetic resonance imaging was suggestive of a giant cell tumor. However, the biopsy revealed a rare diagnosis of epithelioid hemangioendothelioma.

CONCLUSION: The patient was managed with wide excision. In a young adult, arthrodesis may not be the best option; hence, we reconstructed the joint with a custom mega prosthesis (CMP). At 3 years follow-up, our patient had no signs of recurrence. To our best knowledge, this is the first report on the use of CMP in a case of EHE.

PMID:36659885 | PMC:PMC9826681 | DOI:10.13107/jocr.2022.v12.i07.2892

Early morphea appearing as a port-wine stain: A clinical challenge

Fetched: January 20th, 2023, 5:02am GMT by C Matucci-Cerinic

Ann Dermatol Venereol. 2023 Jan 17:S0151-9638(22)00117-X. doi: 10.1016/j.annder.2022.11.003. Online ahead of print.

NO ABSTRACT

PMID:36658067 | DOI:10.1016/j.annder.2022.11.003

Common benign melanocytic and non-melanocytic skin tumors among the elderly. Results of the Graz Study on Health & Aging (GSHA)

Fetched: January 20th, 2023, 5:02am GMT by Viktoria Gruber

Dermatology. 2023 Jan 19. doi: 10.1159/000529219. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Benign skin tumors are the most common skin findings and contribute to the major reasons for consulting a dermatologist. However, the frequency of benign skin tumors concerning different body areas and their relationship with sun-related behavior have not been sufficiently characterized. We aimed to improve knowledge about the anatomic distribution of the most frequently occurring benign skin tumors among a healthy predominant elderly population. Furthermore, we investigated associations with sun-related habits.

METHODS: In total, 100 participants, 37 men, and 63 women (mean age: 67.2 years; range: 46-86 years) were enrolled in the Graz Study on Health & Aging (GSHA) cohort. Full body images were investigated for melanocytic nevi, seborrheic keratoses, hemangiomas, dermatofibromas, and lentigines. Information on the phenotypic trait, sun exposure, use of sun-protective measures, and history of sunburns was collected in a questionnaire.

RESULTS: Common melanocytic nevi were the most frequently encountered skin lesions. Male sex was associated with multiple common nevi on the abdomen and the presence of atypical nevi. High sun exposure in central European latitudes during adolescence was positively associated with multiple common nevi on the total body. Multiple common nevi and atypical nevi on the back correlated with frequent use of sunscreens with SPF during young adulthood and adolescence, respectively.

CONCLUSION: Our study adds new knowledge about the most frequently occurring benign skin tumors, considering all visible body areas. This research may serve as a reference basis for following epidemiological studies.

PMID:36657431 | DOI:10.1159/000529219

Hemangioma-related gene polymorphisms in the pathogenesis of intraventricular hemorrhage in preterm infants

Fetched: January 20th, 2023, 5:02am GMT by Katarzyna Kosik

Childs Nerv Syst. 2023 Jan 19. doi: 10.1007/s00381-023-05824-4. Online ahead of print.

ABSTRACT

PURPOSE: The aim of this study was to evaluate the possible relationship between four single nucleotide polymorphisms of hemangioma-linked genes encoding for anthrax toxin receptor 1 (ANTXR1 G976A), R kinase insert domain receptor (KDR T1444C), adrenoceptor beta 2 (ADRB C79CG), and insulin-like growth factor 1 receptor (IGF-1R G3174A) and the occurrence of IVH in a population of preterm infants.

METHODS: The study includes a population of 105 infants born from 24 + 0 to 32 + 0 weeks of gestation and hospitalized at the Department of Neonatology (III level hospital) of Poznan University of Medical Science. Intraventricular hemorrhage was diagnosed with the use of cranial ultrasound. The classification of intraventricular bleeding was based on the Papile IVH classification.

RESULTS: The incidence of IVH was higher in infants with lower birth weight, lower APGAR scores, and low birth weight. The study revealed that IVH was approximately two times less likely to occur in infants with the allele G of IGF-1R 3174G > A.

CONCLUSION: Identifying susceptible premature infants through genetic analysis could be a potential way to alleviate severe IVH and its subsequent consequences. Further research examining a wider range of relevant gene polymorphisms could help highlight any genetic patterns in this deleterious bleeding complication.

PMID:36656337 | DOI:10.1007/s00381-023-05824-4

Testicular hemangioma in a child: A rare case report and review of literature

Fetched: January 20th, 2023, 5:02am GMT by Kaiping Zhang

Indian J Pathol Microbiol. 2023 Jan-Mar;66(1):222-225. doi: 10.4103/ijpm.ijpm_881_21.

NO ABSTRACT

PMID:36656253 | DOI:10.4103/ijpm.ijpm_881_21

Pediatric intranasal lobular capillary hemangioma

Fetched: January 20th, 2023, 5:02am GMT by P Naina

Indian J Pathol Microbiol. 2023 Jan-Mar;66(1):202-204. doi: 10.4103/ijpm.ijpm_592_21.

NO ABSTRACT

PMID:36656243 | DOI:10.4103/ijpm.ijpm_592_21

Multiply Recurrent Composite Hemangioendothelioma of Penis with Histologic Progression to High-Grade Features

Fetched: January 18th, 2023, 5:01am GMT by Chau M Bui

Dermatopathology (Basel). 2023 Jan 11;10(1):41-45. doi: 10.3390/dermatopathology10010005.

ABSTRACT

Composite hemangioendothelioma (CHE) is a very rare low-grade malignant vascular neoplasm. Here, we present the first case of it occurring on a penis with two local recurrences over a 9 year span and its progression to a high-grade morphology.

PMID:36648783 | PMC:PMC9844429 | DOI:10.3390/dermatopathology10010005

Cardiac hemangioma presenting as a primary cardiac tumor

Fetched: January 17th, 2023, 5:01am GMT by Leart Berdica

Cardiooncology. 2023 Jan 17;9(1):3. doi: 10.1186/s40959-023-00154-5.

ABSTRACT

Cardiac hemangiomas are very rare benign cardiac tumors. They can present at any age and clinical presentation varies according to location and size. We encountered an 87-year-old woman with a left atrial hemangioma clinically diagnosed as cardiac myxoma. Histopathological examination revealed that it was a cavernous-type hemangioma Left atrial hemangiomas, especially those attached to the left atrium wall, may be mistakenly diagnosed as myxomas. Furthermore, a comprehensive review of atrial hemangioma was conducted for the diagnosis and treatment of this uncommon entity.

PMID:36647178 | PMC:PMC9843832 | DOI:10.1186/s40959-023-00154-5

Single-port thoracoscopic removal of an azygos vein aneurysm: a case report and literature review

Fetched: January 17th, 2023, 5:01am GMT by Yi Yao

J Cardiothorac Surg. 2023 Jan 16;18(1):29. doi: 10.1186/s13019-023-02143-2.

ABSTRACT

BACKGROUND: Azygos vein aneurysms (AVAs) are extremely rare. The majority of patients have no obvious clinical symptoms, so they are found by physical examination or by chance. There is limited clinical treatment experience that can be referred to, and there are no clear guidelines or research evidence standardizing the surgical and interventional therapy. Here, we report a patient with idiopathic AVA whose three-dimensional reconstruction of the tumor was completed before surgery. On the basis of three-dimensional reconstruction, single-port thoracoscopic resection of the AVA was successfully completed and reported for the first time. The previously reported cases are summarized to provide guidance for the diagnosis and treatment of patients with AVAs.

CASE PRESENTATION: A 56-year-old man was transferred to our hospital due to "dysphagia". The diagnosis of AVA was made after enhanced computed tomography, gastroscopy, fiberoptic bronchoscopy, and three-dimensional reconstruction. Congenital weakness or degenerative changes causes the vein walls to be extremely thin that the AVA had the risk of ruptur. Furthermore, the patient had symptoms of dysphagia, he received single-port thoracoscopic surgery. After the operation, his dysphagia disappeared. The postoperative pathology confirmed hemangioma. The patient was discharged 3 days after surgery without any complications.

CONCLUSIONS: AVAs are rare. Preoperative three-dimensional reconstruction can greatly help surgeons clarify the disease diagnosis, formulate the surgical plan, avoid damage to the surrounding vital organs, and reduce intraoperative bleeding. Thoracoscopic surgery to remove AVAs is difficult and has a high risk of bleeding, while more minimally invasive single-port thoracoscopic surgery is also safe and effective for the treatment of AVAs.

PMID:36647165 | PMC:PMC9844024 | DOI:10.1186/s13019-023-02143-2

Prevalence and clinical features of orbital vascular anomalies in children

Fetched: January 17th, 2023, 5:01am GMT by Laura A Torrado-Cobian

Orbit. 2023 Jan 16:1-5. doi: 10.1080/01676830.2023.2166081. Online ahead of print.

ABSTRACT

PURPOSE: To report the prevalence, clinical characteristics, and natural history of orbital vascular anomalies diagnosed among children over a 50-year period.

METHODS: The medical records of all patients <19 years diagnosed with any form of an orbital vascular anomaly (OVA) at Mayo Clinic, Rochester, Minnesota from January 1 1966, through December 31 2015, were retrospectively reviewed.

RESULTS: A total of 109 children were diagnosed with OVA during the 50-year period, of which 24 were from Olmsted County, MN, yielding a birth prevalence of 1 in 4,305 live births. The median age at diagnosis for the 109 patients was 1.2 years (range, 0-17.9 years) and 67 (61.5%) were female. Common presenting symptoms included proptosis in 80 (73.4%) patients, abnormalities in skin color in 45 (41.3%) patients, and pain in 18 (16.5%) patients. There were 55 (50.5%) vascular malformations [50 (91%) low-flow lymphatic malformations, 3 (5.5%) high-flow arteriovenous malformations, and 2 (3.5%) low-flow venous malformations] and 54 (49.5%) vascular tumors [53 (98%) capillary hemangiomas and 1 (2%) kaposiform hemangioendothelioma]. During a mean follow-up of 5.95 years (range 0-27.7 years), amblyopia and/or strabismus were diagnosed in 46 (43.4%) patients.

CONCLUSIONS: Capillary hemangiomas and low-flow lymphatic malformations comprise most of the orbital vascular anomalies in this cohort of children. Amblyopia and strabismus are common sequelae, highlighting the importance of early diagnosis and appropriate management.

PMID:36644978 | DOI:10.1080/01676830.2023.2166081

Case report: Sclerosed hemangioma of the liver: A diagnostic challenge

Fetched: January 17th, 2023, 5:01am GMT by M Poras

Front Surg. 2022 Dec 29;9:985849. doi: 10.3389/fsurg.2022.985849. eCollection 2022.

ABSTRACT

Hemangiomas are the most common noncystic benign hepatic tumors and are usually incidentally discovered during routine radiological examinations. The diagnosis of hepatic hemangiomas with a typical presentation is generally easy with plain and cross-sectional imaging; however, it can be complicated when hemangiomas undergo histological changes such as fibrosis. Sclerosed hepatic hemangioma (SHH) is the extreme presentation of this fibrotic process. These atypical lesions can be misdiagnosed as primary hepatic malignancies or metastasis. Their diagnosis is established by histological examination. We report the case of a patient with an SHH, which was misdiagnosed as an intrahepatic cholangiocarcinoma. This article's aim is to draw attention to this infrequent pathology and underline the features of this benign tumor that could suggest its diagnosis prior to surgery to avoid unnecessary hepatic resections.

PMID:36644528 | PMC:PMC9834279 | DOI:10.3389/fsurg.2022.985849

Permalink for 'Thoracic Percutaneous Vertebroplasty for the Treatment of Vertebral Hemangioma in a Patient With Forestier's Disease: A Case Report'

Thoracic Percutaneous Vertebroplasty for the Treatment of Vertebral Hemangioma in a Patient With Forestier's Disease: A Case Report

Fetched: January 17th, 2023, 5:01am GMT by Renat Nurmukhametov

Cureus. 2022 Dec 13;14(12):e32466. doi: 10.7759/cureus.32466. eCollection 2022 Dec.

ABSTRACT

Percutaneous vertebroplasty consists of an injection of polymethylmethacrylate in the vertebral body, with the aim of reinforcing the bone structure, preventing vertebral collapse, and achieving analgesic and antitumor effects. It is used in the treatment of patients with aggressive vertebral hemangiomas, as well as compression fractures of traumatic etiology and pathological fractures. Forestier's disease is also known as senile ankylosing hyperostosis of the spine. It is characterized by hypertrophy of the anterior longitudinal ligament. Depending on the most prominent place of ossification of this ligament, its clinical symptoms vary, with intense pain being the most relevant. Here, we present the case of a 73-year-old female with complaints of intense, constant pain that did not improve with conservative treatment, located at the level of the Th4Th10 vertebrae, radiating along the intercostal spaces, with eight months of evolution with muscular hypertonism. Magnetic resonance imaging of the thoracic spine showed osteochondritis of the thoracic spine and right-sided scoliosis. For hemangioma of the Th6 vertebral body, the patient was referred to the vertebrology department, where she was admitted to undergo percutaneous vertebroplasty of the affected level under fluoroscopic control. In this study, we report the use of percutaneous vertebroplasty as a minimally invasive treatment in a patient with Forestier's disease, obtaining excellent results, rapid recovery, and minimal hospitalization time, without having to subject the patient to major surgery.

PMID:36644075 | PMC:PMC9835017 | DOI:10.7759/cureus.32466

The Role of Computed Tomography Perfusion in Various Focal Liver Lesions

Fetched: January 17th, 2023, 5:01am GMT by Vignesh Gadupudi

Cureus. 2022 Dec 11;14(12):e32420. doi: 10.7759/cureus.32420. eCollection 2022 Dec.

ABSTRACT

Background This study aims to identify the potential advantages of quantitative determination of various focal liver pathologies, identify lesion hemodynamics, and distinguish benign and malignant pathologies based on CT perfusion (CTP) parameters. Methodology In this study, we examined 36 patients using contrast-enhanced CT (CECT) and proposed inclusion and exclusion criteria. Of the 36 patients, 18 had malignant lesions and 14 had benign lesions. CTP was performed on patients comprising cases of hepatocellular carcinoma (HCC), metastasis, hemangiomas, hepatic cysts, and hepatic abscess. Images were post-processed and analyzed to calculate various perfusion parameters such as blood flow (BF), blood volume (BV), permeability surface (PS), mean transit time (MTT), the hepatic arterial fraction (HAF), and induced residue fraction time of onset (IRFTO). Parameters were compared between benign and malignant lesions, and descriptive analysis was performed for individual lesions. Results Data were analyzed with IBM SPSS Statistics (IBM Corp., Armonk, NY, USA). IRFTO showed the area of the curve (AOC) = 0.659, P-value = 0.040, sensitivity 66.7%, and specificity 64.3%. BV showed AOC = 0.659, P-value = 0.040, with a cutoff value of 1.26, sensitivity of 66.7%, and specificity of 64.3%. BF showed AOC = 0.786 and P-value = 0.006, with a cutoff value of 171.2, sensitivity of 83.3%, and specificity of 78.6%. MTT showed AOC = 0.778 and P-value = 0.008, with a cutoff value of 6.94, sensitivity of 77.8%, and specificity of 78.6%. Statistically significant changes were observed in the perfusion parameters in the BV, BF, MTT, and IRFTO. Conclusions The noninvasive CT liver perfusion technique makes it possible to compare the hemodynamic changes in healthy and sick liver tissues, identify focal liver lesions, and evaluate the effectiveness of tumor therapy.

PMID:36644059 | PMC:PMC9833639 | DOI:10.7759/cureus.32420

Orbital hemangioma extirpation via a transnasal endoscopic surgical approach: A case report

Fetched: January 15th, 2023, 5:01am GMT by Ratih Finisanti

Int J Surg Case Rep. 2023 Jan 11;103:107898. doi: 10.1016/j.ijscr.2023.107898. Online ahead of print.

ABSTRACT

INTRODUCTION: Orbital tumors are heterogeneous lesions originating from various structures in the eyeball, including the extraconal, conal, and intraconal spaces. One orbital tumor type is a vascular tumor, such as a hemangioma. Hemangiomas are most common in women aged 20-64 years. They are painless and slow-growing, leading to proptosis. The diagnosis can generally be established by clinical examination and computed tomography (CT) or magnetic resonance imaging scans. Orbital hemangioma management can involve regular observation of small and asymptomatic tumors or surgery for large symptomatic tumors.

PRESENTATION OF CASE: We report a 20-year-old Asian woman who presented with swelling on the medial side of her right eyelid that had increased over the last three months. A non-contrast head CT scan showed a mass in the right eye's medial wall. The patient underwent a joint operation with an ophthalmologist to remove the median orbital tumor by transnasal endoscopic orbital surgery. Anatomical pathology examination of the tumor tissue identified hemangiomas.

CONCLUSION: An endoscopic transnasal approach is a safe and effective way to access and manage an orbital tumor medial to the optic nerve. It is essential to have a multidisciplinary team with experience in endoscopic procedures. This patient had satisfactory results at their three-month postoperative follow-up. They reported no symptoms, and their CT scan did not show a relapsed mass in the right oculi region.

PMID:36640470 | PMC:PMC9846022 | DOI:10.1016/j.ijscr.2023.107898

Permalink for 'Forty-five-months follow-up of a minimally invasive, interdisciplinary treated hemangioma of the mandible with a high risk of severe bleeding - a case report'

Forty-five-months follow-up of a minimally invasive, interdisciplinary treated hemangioma of the mandible with a high risk of severe bleeding - a case report

Fetched: January 14th, 2023, 5:01am GMT by Michelle Alicia Ommerborn

Head Face Med. 2023 Jan 14;19(1):1. doi: 10.1186/s13005-022-00346-x.

ABSTRACT

BACKGROUND: Hemangiomas are benign tumours, mostly seen in the soft tissues. The intraosseous appearance is rare, in particular in the jaws they represent a very seldom malformation.

AIM: To present a combined endodontic and surgical management report of a clinical case with a rare intraosseous hemangioma diagnosis in the mandible.

CASE PRESENTATION: This well-documented case report describes the interdisciplinary treatment approach of an intraosseous hemangioma in the left mandible of a 70-year-old male patient. This incidental finding was detected through a routine dental examination. The panoramic radiograph revealed an asymptomatic, apical translucency approximately 15 mm diameter with contact to the mesial root of the tooth 36. The clinical examinations showed no abnormalities. The multifaceted specialized treatments started with the endodontic treatment of the tooth prior to the surgical removal of the lesion and were followed by the histological assessment. As derived from the histologically verified diagnosis, this rare case included the risk of severe bleeding complications during therapy.

PMID:36639813 | PMC:PMC9840336 | DOI:10.1186/s13005-022-00346-x

Diagnosis and management of extramedullary plasmacytoma in nasal cavity: Clinical experience and literature review

Fetched: January 14th, 2023, 5:01am GMT by Hongyu Hu

Medicine (Baltimore). 2023 Jan 13;102(2):e32647. doi: 10.1097/MD.0000000000032647.

ABSTRACT

Nasal extramedullary plasmacytoma (EMP) is a rare plasma cell tumor that occurs in the soft tissue of the nasal cavity, and its imaging characteristics are still unclear. The purpose of this study was to investigate the clinical features, imaging findings, treatment, survival analysis, and prognosis of nasal EMP, and to provide a systematic review of the patients we treated and the published literature. A 45-year-old female patient who presented with epistaxis with nasal obstruction was recommended for magnetic resonance imaging to assess the nature of the lesion. On magnetic resonance imaging, abnormal signal shadow can be seen in the right nasal cavity. Diffusion weighted imaging showed signal of the lesion was significantly limited, presenting high signal, with a low apparent dispersion coefficient, and the lesion was significantly enhanced on contrast-enhanced scan. Combined with the clinical manifestations of the patient, who was initially considered to have a hemangioma. She underwent endoscopic nasal surgery under general anesthesia to remove the mass, and the final pathology confirmed it was EMP. However, the final pathology confirmed EMP. Five months later, the patient came to our hospital for follow-up and underwent fluorine-18-fluorodeoxyglucose/positron emission tomography/computed tomography scan, which showed no recurrence of the lesion and no transformation of multiple myeloma. The nasal EMP imaging findings were mostly soft tissue masses with uniform density or signal, which were significantly enhanced by enhancement scan, high signal on diffusion weighted imaging and low signal on apparent dispersion coefficient. Immunohistochemical staining for CD38, CD138, and CD79a was positive in most of the cases evaluated, while CD20 and CD10 were negative. The absence of dilated features, infiltrative features and the presence of significant contrast enhancement may be relatively specific imaging findings of nasal EMP. The prognosis of nasal EMP is good, and recurrence, metastasis, and transformation into multiple myeloma are rare. Because the lesions are sensitive to radiotherapy, surgical resection combined with radiotherapy is a more effective treatment.

PMID:36637932 | PMC:PMC9839280 | DOI:10.1097/MD.0000000000032647

Epithelioid hemangioendothelioma mimicking aortic intramural haematoma

Fetched: January 13th, 2023, 4:19pm GMT by Lei Zhang

Eur Heart J Cardiovasc Imaging. 2023 Jan 13:jead003. doi: 10.1093/ehjci/jead003. Online ahead of print.

NO ABSTRACT

PMID:36637898 | DOI:10.1093/ehjci/jead003

Permalink for 'Endoscopic Endonasal Translamina Terminalis Approach for Resection of Hemorrhaged Third Ventricle Cavernous Hemangioma: 2-Dimensional Operative Video'

Endoscopic Endonasal Translamina Terminalis Approach for Resection of Hemorrhaged Third Ventricle Cavernous Hemangioma: 2-Dimensional Operative Video

Fetched: January 13th, 2023, 4:19pm GMT by Sumin Geng

Oper Neurosurg (Hagerstown). 2023 Feb 1;24(2):e120-e121. doi: 10.1227/ons.0000000000000486. Epub 2022 Dec 5.

NO ABSTRACT

PMID:36637319 | DOI:10.1227/ons.0000000000000486

A Giant Mixed Infiltrative Angiolipoma of the Back with Venous Malformation- A Case Report and Related Gene Mutation Detection

Fetched: January 13th, 2023, 2:07pm GMT by Fangning Zhao

Clin Cosmet Investig Dermatol. 2023 Jan 6;16:53-58. doi: 10.2147/CCID.S389178. eCollection 2023.

ABSTRACT

We report a case of a middle-aged male patient with a diffuse large mixed infiltrating angiolipoma on the back with venous malformation that grew from the age of 3 years. We discussed the design of the surgical flap for this large lipoma, observed its blood supply using SPY, and screened for possible causative genes, FCGR2A, BMP5, MUC2, and KRTAP4-9. To date, no invasive lipomas of this size and duration have been reported.

PMID:36636636 | PMC:PMC9831088 | DOI:10.2147/CCID.S389178

The complex pediatric middle ear hemangioma

Fetched: January 13th, 2023, 5:02am GMT by Miray-Su Yılmaz Topçuoğlu

HNO. 2023 Jan 12. doi: 10.1007/s00106-022-01261-y. Online ahead of print.

ABSTRACT

Hemangiomas in the pediatric middle ear are a rare occurrence. We present the case of a 3-year-old boy with unilateral hearing impairment and recurrent otitis media with effusion due to a hemangioma in the middle ear. This caused a venous sinus thrombosis, osteomyelitis, and mastoiditis. Interdisciplinary treatment was performed. This case shows that even benign lesions in the middle ear can cause severe, life-threatening complications due to the close proximity of important anatomic structures in the middle ear. Every case of asymmetric hearing loss should thus be examined in detail.

PMID:36635394 | DOI:10.1007/s00106-022-01261-y

A clinicopathological classification of space-occupying lesions of the orbit in 1 913 patients from 2000 to 2021

Fetched: January 12th, 2023, 5:01am GMT by L Y Wang

Zhonghua Yan Ke Za Zhi. 2023 Jan 11;59(1):20-25. doi: 10.3760/cma.j.cn112142-20220802-00373.

ABSTRACT

Objective: To investigate the histopathological classification of orbital space-occupying lesions. Methods: This is a retrospective case series study. The clinical and pathological data of 1 913 tissue specimens from 1 913 patients with space-occupying lesions of the orbit which were examined in the Second Affiliated Hospital, Zhejiang University School of Medicine from January 2000 to December 2021 were collected. The mass lesions were classified based on histogenesis, pathological nature and age. Results: There were 913 males (47.7%) and 1 000 females (52.3%). The lesions were benign in 1 489 patients (77.8%) and malignant in 424 patients (22.2%). Based on histogenesis, there were 521 vasculogenic lesions (27.2%), which rancked first, 407 cystoid lesions (21.3%), 277 lymphoproliferative lesions (14.5%), 182 lacrimal gland lesions (9.5%) and 121 inflammatory lesions (6.3%). By pathological nature, there were 1 489 benign lesions, including cavernous hemangioma (275, 14.4%), dermoid cyst (225, 11.8%), other hemangiomas (199, 10.4%), epidermoid cyst (136, 7.1%) and benign mixed tumor of the lacrimal gland (134, 7.0%), and 257 malignant lesions, including lymphoma (210, 11.0%) and sebaceous gland carcinoma (47, 2.5%). The age of all patients ranged from 0 to 90 years, while 247 lesions (12.9%) occurred in patients aged 0 to18 years, 1 270 lesions (66.4%) in patients aged 19 to 59 years, and 396 lesions (20.7%) in patients aged 60 to 90 years. Conclusions: In 22 years, almost 2/3 benign orbital lesions in the Second Affiliated Hospital, Zhejiang University School of Medicine occurred in young and middle-aged patients, and males were fewer than females. The most common benign orbital tumors was cavernous hemangioma, followed by dermoid cyst and epidermoid cyst. And the most common malignant orbital tumor was lymphoma, which occurred more frequently in older patients.

PMID:36631053 | DOI:10.3760/cma.j.cn112142-20220802-00373

Giant hepatic mesenchymal hamartoma in a preterm newborn: a case report and literature review

Fetched: January 12th, 2023, 5:01am GMT by Zhiru Wang

Am J Transl Res. 2022 Dec 15;14(12):8782-8787. eCollection 2022.

ABSTRACT

Mesenchymal hamartoma of the liver (MHL) often presents as a painless right upper abdominal mass in young children. However, MHL is rarely reported in the neonatal period. We presented the case of a preterm newborn with a huge MHL. The boy was delivered at 30 weeks weighing 1750 g. Abdominal distention was the initial presentation. Ultrasound and computed tomography showed a highly vascularized mass originating from the left lobe. Liver hemangioma was initially suspected and oral propranolol was administered. However, the tumor was rapidly enlarging, resulting in compromised respiratory status and severe anemia. Surgical resection and neonatal management were successful. The patient required cardiopulmonary resuscitation in the operating room and received packed red blood cells. The histopathological result was mesenchymal hamartoma. The baby recovered well after one-year follow-up. We also reviewed the clinical courses and treatment strategies of preterm MHL cases in published English literature from 1990 to 2021.

PMID:36628252 | PMC:PMC9827322

Permalink for 'Multimodal Imaging Characteristics of Circumscribed Choroidal Hemangioma Accompanying with Central Serous Chorioretinopathy: A Case Report'

Multimodal Imaging Characteristics of Circumscribed Choroidal Hemangioma Accompanying with Central Serous Chorioretinopathy: A Case Report

Fetched: January 12th, 2023, 5:01am GMT by Irmak Karaca

Beyoglu Eye J. 2022 Nov 15;7(4):329-333. doi: 10.14744/bej.2022.47113. eCollection 2022.

ABSTRACT

A 42-year-old man presented with a temporal visual field defect in his right eye. His history revealed systemic steroid use before onset of his complaints. Multimodal imaging techniques including ultrasonography, fluorescein angiography, indocyanine green angiography, and optical coherence tomography angiography (OCTA) suggested the presence of circumscribed choroidal hemangioma (CCH) accompanying with central serous chorioretinopathy (CSCR) in the right eye, which might have worsened with systemic steroid treatment. CCH may rarely present with accompanying CSCR. Besides, OCTA is a non-invasive reliable method for the diagnosis of CCH in terms of visualizing vascular features of tumor.

PMID:36628075 | PMC:PMC9794509 | DOI:10.14744/bej.2022.47113

Permalink for 'A Case Series of 18 Congenital Haemangiomas: Clinical, Histological and Ultrasound Features, and their Relationship with Complications and Atypical Behaviour'

A Case Series of 18 Congenital Haemangiomas: Clinical, Histological and Ultrasound Features, and their Relationship with Complications and Atypical Behaviour

Fetched: January 11th, 2023, 5:01am GMT by Gloria Juan-Carpena

Acta Derm Venereol. 2023 Jan 10;103:adv00849. doi: 10.2340/actadv.v103.3983.

ABSTRACT

Recent studies have advanced our understanding of the clinical, histological and imaging characteristics of congenital haemangiomas (CHs), and have reported possible complications and atypical behaviour. The aim of this study is to describe the clinical, histological and ultrasound features of a series of CHs and to analyse their association with complications and atypical behaviour, with a view to providing diagnostic and management recommendations. The medical records, histology results and ultrasound images of all patients with CH diagnosed in the Dermatology Department of Alicante University General Hospital between 2006 and 2021 were retrospectively reviewed. A total of 18 patients were included, of whom 4 (22.2%) had complications. The most severe was 1 case with heart failure. There was a significant association between large CH size (> 5 cm) and the occurrence of complications (p = 0.019). The study identified 3 different lobule patterns, but found no relationship with CH subtype or other findings. The associations of venous ectasia, venous lakes and arteriovenous microshunts with occurrence of complications was borderline significant (p = 0.055). Study limitations were the small sample and the retrospective analysis. To conclude, haematological and cardiological assessment is indicated in large CHs and should be considered in CHs with ultrasound findings of venous ectasia, venous lakes or arteriovenous microshunts, as these cases present a greater risk of complications.

PMID:36625212 | DOI:10.2340/actadv.v103.3983

Permalink for 'Early Phase Functional Recovery after Spinal Intramedullary Tumor Resection Could Predict Ambulatory Capacity at 1 Year after Surgery'

Early Phase Functional Recovery after Spinal Intramedullary Tumor Resection Could Predict Ambulatory Capacity at 1 Year after Surgery

Fetched: January 11th, 2023, 5:01am GMT by Tetsuya Suzuki

Asian Spine J. 2023 Jan 10. doi: 10.31616/asj.2022.0068. Online ahead of print.

ABSTRACT

STUDY DESIGN: This is a single-center retrospective cohort study with a university hospital setting.

PURPOSE: This study aims to evaluate the short-term course of physical function and walking ability after intramedullary spinal cord tumor (ISCT) resection and predict walking independence 1 year after surgery.

OVERVIEW OF LITERATURE: Although several reports have shown the postoperative functional prognosis of spinal intramedullary tumors with long-term follow-up, no reports have identified the predictors associated with the functional outcome at an early stage.

METHODS: A total of 79 individuals who underwent ISCT resection at our institute between 2014 and 2019 were enrolled in the study, whose preoperative walking state was independent ambulator regardless of cane support with the Functional Independence Measure Locomotor Scale (FIM-L) score of ≥6. The FIM-L, the American Spinal Injury Association (ASIA) motor and sensory scores in the lower extremities, and the Walking Index for Spinal Cord Injury II (WISCI II) were assessed for walking independence, lower-limb function, and walking ability, respectively. These evaluations were performed at 4 time points: preoperatively, 1 week (1W), 2 weeks (2W), and 1 year after surgery.

RESULTS: In the early phase after surgery, 71% and 43% of the participants were nonindependent ambulators at 1W and 2W, respectively. Histopathology indicated that patients with solid tumors (ependymoma, astrocytoma, or lipoma) showed significantly lower indices at 1W and 2W than those with vascular tumors (hemangioblastoma or cavernous hemangioma). Regarding tumor location, thoracic cases exhibited poorer lower-limb function at 1W and 2W and poorer walking ability at 2W than cervical cases. According to the receiver operating characteristic (ROC) analysis, 2 WISCI II points at 2W had the highest sensitivity (100%) and specificity (92.2%) in predicting the level of walking independence at 1 year postoperatively (the area under the ROC curve was 0.99 (95% confidence interval, 0.93-1.00).

CONCLUSIONS: The higher the lower-limb function scores in the early phase, the better the improvement in walking ability is predicted 1 year after ISCT resection.

PMID:36625019 | DOI:10.31616/asj.2022.0068

Diagnostic challenge and management of thymic cavernous hemangioma: a case report and review of the literature

Fetched: January 11th, 2023, 5:01am GMT by Li Liu

Curr Med Imaging. 2023 Jan 9. doi: 10.2174/1573405619666230109121128. Online ahead of print.

ABSTRACT

Objective Thymic cavernous hemangioma (CH) as a benign lesion is extremely rare, and it is very difficult to distinguish it from other anterior mediastinal tumors on imaging. Case Presentation We treated a 63-year-old woman with thymic CH that was incidentally found by chest computed tomography (CT). Contrast-enhanced chest CT described an anterior superior mediastinal mass, approximately 2.5cm in diameter, with centripetal enhancement and an increased range of enhancement in the delayed period. The tumor and thymus were completely resected by video-assisted thoracic surgery (VATS) to provide a definitive diagnosis and precise treatment. Postoperative pathology suggested that the tumor was a thymus cavernous hemangioma. Conclusion We reported an extremely uncommon case of CH in the thymus and reviewed the previously published literature. CH can occur at any age and body tissue, and there is no significant difference between males and females. Most patients are asymptomatic, often found by chest CT. Enhanced chest CT suggested that an increased area of venous enhancement at a prolonged duration may be a feature of CH. Complete surgical resection is a safe and effective method for CH in the thymus.

PMID:36624647 | DOI:10.2174/1573405619666230109121128

Giant cavernous hemangioma of the left atrial appendage: a case description and literature review

Fetched: January 10th, 2023, 5:01am GMT by Qing Duan

Quant Imaging Med Surg. 2023 Jan 1;13(1):538-545. doi: 10.21037/qims-22-460. Epub 2022 Oct 18.

NO ABSTRACT

PMID:36620148 | PMC:PMC9816720 | DOI:10.21037/qims-22-460

Primary YAP1-TFE3-fused epithelioid hemangioendothelioma of bone: a clinicopathological analysis of two cases

Fetched: January 10th, 2023, 5:01am GMT by W Zhang

Zhonghua Bing Li Xue Za Zhi. 2023 Jan 8;52(1):43-45. doi: 10.3760/cma.j.cn112151-20221005-00830.

ABSTRACT

目的： 探讨骨原发YAP1-TFE3融合上皮样血管内皮瘤（epithelioid hemangioendothelioma，EHE）的临床病理学特征、诊断、鉴别诊断及分子特征。 方法： 收集北京积水潭医院病理科2009年1月至2021年9月骨内原发YAP1-TFE3融合EHE 2例，进行HE染色、免疫组织化学染色、荧光原位杂交（FISH）及二代测序，分析其组织形态、免疫组织化学表达及分子遗传学特征，并复习相关文献。 结果： 2例YAP1-TFE3融合EHE，男性1例，女性1例，年龄分别为26、16岁，均为多发骨病变。例1位于右侧髂骨、髋臼及股骨大转子，例2位于左股骨远端、髌骨、胫腓骨、距骨及跟骨。2例除均可见到典型的EHE形态外，还可观察到丰富、具有嗜酸性胞质的细胞，实性片状生长，伴血管腔结构形成，细胞核具有轻-中度异型性。免疫组织化学染色2例标本均表达CD31、CD34、ERG、Fli1、F8等血管标志物，例2个别细胞EMA阳性，均不表达其余上皮标志物，CAMTA1均不表达，TFE3均为核表达，FISH检测2例均检测到TFE3分离信号，无CAMTA1分离信号。二代测序例1检测到YAP1（第1号外显子）-TFE3（第4号外显子）融合。 结论： 骨内原发YAP1-TFE3融合上皮样血管内皮瘤是一种低-中度恶性肿瘤，极为罕见，特殊的组织学形态结合TFE3核表达及分子检测有助于该肿瘤的诊断及鉴别诊断。.

PMID:36617905 | DOI:10.3760/cma.j.cn112151-20221005-00830

Preoperative embolization of intraosseous hemangioma arised in zygoma: A case report

Fetched: January 9th, 2023, 5:01am GMT by Hang Goo Yun

Asian J Surg. 2023 Jan 6:S1015-9584(22)01783-3. doi: 10.1016/j.asjsur.2022.12.071. Online ahead of print.

NO ABSTRACT

PMID:36610901 | DOI:10.1016/j.asjsur.2022.12.071

Hemangioma of Conjunctiva and Eyelid

Fetched: January 9th, 2023, 5:01am GMT by Girendra Yadav

Ophthalmology. 2023 Jan 4:S0161-6420(22)00931-9. doi: 10.1016/j.ophtha.2022.11.025. Online ahead of print.

NO ABSTRACT

PMID:36609022 | DOI:10.1016/j.ophtha.2022.11.025

Nasal lobular capillary hezmangioma: Report of a case managed by endoscopic excision and pre-operative angio-embolization

Fetched: January 9th, 2023, 5:01am GMT by Meshal B Albesher

Int J Surg Case Rep. 2023 Jan;102:107866. doi: 10.1016/j.ijscr.2022.107866. Epub 2022 Dec 31.

ABSTRACT

INTRODUCTION: Lobular capillary hemangiomas are fast-growing benign vascular lesions with distinctive histopathological characteristics. The head and neck region is a common location for lobular capillary hemangiomas. However, the presence of such lesions in the nasal cavity is rare. Although several contributing factors have been identified in literature, the exact pathophysiology is not yet well understood. Predisposing factors include nasal trauma, pregnancy, and the use of contraceptive pills. Thus, the disease is more prevalent in females, with variable peak incidence in pediatric patients. Unilateral nasal obstruction and recurrent epistaxis are the most common symptoms of nasal lobular capillary hemangiomas. Radiological evaluation using contrast-enhanced computed tomography and magnetic resonance imaging is often required for large lesions.

CASE PRESENTATION: We present a 30-year old female who presented to ENT clinics with two month complaint of left-sided nasal obstruction and epistaxis with left facial pain and headache. She had no predisposing risk factors. Imaging with CT and MRI revealed a large hypervascular mass in left nasal cavity. Surgical excision preceded by pre-operative embolization was done.

DISCUSSION: Endoscopic endonasal excision is the standard of treatment. While some authors believe that pre-operative embolization is not required, others advocate its use. Based on literature, recurrence rate is variable.

CONCLUSION: We believe that use of pre-operative embolization for large nasal lobular capillary hemangioma would have an impact on perioperative morbidity.

PMID:36608636 | PMC:PMC9829804 | DOI:10.1016/j.ijscr.2022.107866

Permalink for 'Solitary pulmonary capillary hemangioma mimicking a preinvasive malignant lesion in an asymptomatic middle-aged female patient'

Solitary pulmonary capillary hemangioma mimicking a preinvasive malignant lesion in an asymptomatic middle-aged female patient

Fetched: January 7th, 2023, 5:02am GMT by Yasuyuki Kanamoto

Int Cancer Conf J. 2022 Aug 3;12(1):14-18. doi: 10.1007/s13691-022-00570-x. eCollection 2023 Jan.

ABSTRACT

Pulmonary capillary hemangiomatosis (PCH) is a rare disease characterized by a proliferation of capillaries in the alveolar septa, bronchial and venous walls, pleura, and regional lymph nodes. However, the etiology of the disease remains unknown due to its rarity. Therefore, we present a case of a solitary PCH lesion without symptoms in a 38-year-old female patient. According to computed tomography, she was diagnosed with lung carcinoma, indicated by a tiny nodule with ground-glass opacity detected in her right upper lung. However, no other lesions were detected on systemic examination. Consequently, partial lung resection was conducted, since the lesion was suspected of lung adenocarcinoma. Pathologic results showed that the thick alveolar septa were caused by capillary growth without cellular atypia and hardly any infiltration of inflammatory cells. Finally, we diagnosed the pulmonary lesion as PCH, although solitary PCH has previously been reported in a few case reports. Therefore, further case studies are essential to clarify the causes of PCH.

PMID:36605833 | PMC:PMC9807707 | DOI:10.1007/s13691-022-00570-x

Cavernous mediastinal hemangioma presenting with persistent cough: a rare case report and review of literature

Fetched: January 6th, 2023, 5:02am GMT by Parviz Mardani

J Cardiothorac Surg. 2023 Jan 5;18(1):3. doi: 10.1186/s13019-023-02130-7.

ABSTRACT

BACKGROUND: Cavernous hemangioma is a rare benign tumor which can sometimes mimic the clinical presentation and radiological findings of malignant tumors. Here we present a rare presentation of cavernous hemangioma in the mediastinum (CHM), along with a literature review among the main databases.

CASE PRESENTATION: We present a 48-year-old male who had suffered from persistent cough as the sole symptom of an anterior CHM. Computed tomography scan demonstrated a 12.5 × 10.8 cm mass in the anterior mediastinum. The mass was surgically resected, and histopathological evaluation established the diagnosis of CHM. The patient was discharged in good condition, in which during his four-month follow-up period, no recurrence of the tumor has been observed.

CONCLUSION: Although cavernous hemangioma rarely present in the mediastinum, it should be considered in the differential diagnosis of mediastinal tumors. However, our review of literature demonstrated a female dominance and average age of 40 years, with a 52% mortality rate based on previous reports.

PMID:36604701 | PMC:PMC9817268 | DOI:10.1186/s13019-023-02130-7

Kaposiform hemangioendothelioma and tufted angioma: two entities of the same clinicopathological spectrum

Fetched: January 6th, 2023, 5:02am GMT by Lula María Nieto-Benito

An Bras Dermatol. 2023 Jan 3:S0365-0596(22)00318-X. doi: 10.1016/j.abd.2021.07.010. Online ahead of print.

NO ABSTRACT

PMID:36604299 | DOI:10.1016/j.abd.2021.07.010

Permalink for 'Clinical study on application of 3D Slicer software assisted domestic frameless stereotactic robot in biopsy of intracranial lesions'

Clinical study on application of 3D Slicer software assisted domestic frameless stereotactic robot in biopsy of intracranial lesions

Fetched: January 6th, 2023, 5:02am GMT by H Chen

Zhonghua Wai Ke Za Zhi. 2023 Jan 1;61(1):61-65. doi: 10.3760/cma.j.cn112139-20220610-00264.

ABSTRACT

Objective: To examine the application value of 3D Slicer software assisted domestic frameless stereotactic robot in biopsy of intracranial lesions. Methods: A retrospective analysis was performed on 80 patients who admitted consecutively and underwent intracerebral lesions biopsy with the domestic frameless stereotactic robot at Department of Neurosurgery, Aerospace Central Hospital from January 2019 to December 2021. There were 36 males and 44 females, with a mean age of (38.5±18.0) years (range: 6 to 71 years). Before surgery only enhanced T1-weighted three-dimensional magnetization prepared gradient echo sequences and diffusion tensor imaging scans were performed. Self-reconstruction of intracranial lesions, cerebral cortex and blood vessels was carried out using 3D Slicer software system after the DICOM format imaging data of 80 patients were collected. These imaging data were merged to the workstation of the domestic frameless stereotactic robot for preoperative surgical planning and the surgical puncture path was designed to avoid blood vessels in the brain functional area, cerebral cortex and sulcus. Results: All frameless stereotactic biopsy were successfully performed. Postoperative pathological diagnosis included 50 cases of diffuse astrocytic and oligodendroglioma, 15 cases of lymphoma, 5 cases of metastatic tumors, 5 cases of inflammatory demyelinating disease, 2 cases of inflammatory granuloma, 1 case of hemangioma, 1 case of acute lymphoblastic leukemia intracranial invasion and 1 case of seminoma. The positive diagnosis rate was 100% (80/80). Postoperative imaging confirmed that the puncture path and target were accurately implemented according to the preoperative planning, and the target error was (1.32±0.44) mm (range: 0.55 to 1.99 mm). One case of puncture-related bleeding occurred at the target after surgery and improved after treatment. Conclusion: The three-dimensional multimodal images reconstructed by the 3D Slicer software before operation could help the surgeons make the preoperative planning and reduce the risk of stereotactic brain biopsy.

PMID:36603886 | DOI:10.3760/cma.j.cn112139-20220610-00264

Surgical Resection of Primary Cardiac Cavernous Hemangioma: A Case Report

Fetched: January 6th, 2023, 5:02am GMT by Yulin Wen

Heart Surg Forum. 2022 Nov 30;25(5):E753-E755. doi: 10.1532/hsf.4969.

ABSTRACT

Cardiac tumors are rare. They were found in only 0.001%-0.300% of cases in a relatively recently reported autopsy series. Among cardiac tumors, primary hemangioma accounted for approximately 2.8% of all primary resected tumors, indicating this is a particularly rare benign neoplasm. We present a patient with a 5×3×2 cm cavernous hemangioma, arising from the right atrial roof and occupying the atrial septum and inseparable from the aortic root. We successfully accomplished a complete surgical resection of a cardiac cavernous hemangioma and reconstructed the cardiac atrium by a bovine pericardial patch.

PMID:36602391 | DOI:10.1532/hsf.4969

Surgical Approach and Further Management of Intracranial Hemangioendothelioma With Double Location: A Case Report

Fetched: January 6th, 2023, 5:02am GMT by Alan Hernández-Hernández

Cureus. 2022 Nov 30;14(11):e32072. doi: 10.7759/cureus.32072. eCollection 2022 Nov.

ABSTRACT

Hemangioendotheliomas are highly vascularized lesions, and their intracranial presentation is extremely rare. We present the case of a 65-year-old female patient who was evaluated for cranial deformity, headache, and left hemiplegia. Two bone lesions that were destroying and expanding the bone diploe with intracranial extension were identified in the fronto-temporal and parietal regions. Both lesions were multilobed and showed heterogeneous behavior. Mixed hemangioendotheliomas were identified after the successful resection of both tumors in two separate surgical procedures. The prognosis of this type of tumor with an intracranial location is not well-defined because there are too few reported cases.

PMID:36600818 | PMC:PMC9803365 | DOI:10.7759/cureus.32072

Permalink for 'A Rare Co-Existence of Multiple Cerebral Cavernous Malformations and Pleomorphic Adenoma of the Parotid Gland in Myotonic Dystrophy'

A Rare Co-Existence of Multiple Cerebral Cavernous Malformations and Pleomorphic Adenoma of the Parotid Gland in Myotonic Dystrophy

Fetched: January 6th, 2023, 5:02am GMT by Sadaf Iftikhar

J Coll Physicians Surg Pak. 2022 Dec;32(12):SS187-SS189. doi: 10.29271/jcpsp.2022.Supp0.SS187.

ABSTRACT

Myotonic dystrophy is an autosomal dominant inherited disorder primarily affecting muscle function. Myotonia, progressive muscle weakness and wasting, and associated systemic involvement, i.e., cataracts, cardiac conduction defects and endocrine abnormalities especially insulin resistance, are the characteristic features. Recent evidence has shown an increased risk of developing benign as well as malignant tumours in such patients. We report a 39-year male of myotonic dystrophy who presented with multiple cerebral cavernous malformations in addition to pleomorphic adenoma of the parotid gland. Though the association of myotonic dystrophy with salivary gland neoplasms has been sparsely documented in the literature, but the co-existence with multiple cerebral cavernous malformations has not been reported so far. Our case is the first of its kind. Key Words: Cerebral cavernous malformations, Myotonic dystrophy, Parotid gland, Pleomorphic adenoma.

PMID:36597334 | DOI:10.29271/jcpsp.2022.Supp0.SS187

Permalink for 'CT and MRI features of hepatic epithelioid haemangioendothelioma: a multi-institutional retrospective analysis of 15 cases and a literature review'

CT and MRI features of hepatic epithelioid haemangioendothelioma: a multi-institutional retrospective analysis of 15 cases and a literature review

Fetched: January 5th, 2023, 5:01am GMT by Lianmei Luo

Insights Imaging. 2023 Jan 5;14(1):2. doi: 10.1186/s13244-022-01344-y.

ABSTRACT

OBJECTIVE: To improve the current imaging understanding of MRI or CT for hepatic epithelioid haemangioendothelioma (HEHE) to aid in its successful preoperative diagnosis.

METHODS: The imaging features of 15 patients (median age 38.6, range 20-71; 7 M/8 F) from eight institutions with pathologically confirmed HEHE were retrospectively analysed. Additionally, the CT/MR imaging features of 180 patients in 15 literature publications were collected, analysed and compared with our case series.

RESULTS: Fifteen patients underwent CT and MRI (n = 2), CT (n = 9) or MR (n = 8) scans. A total of 92.9% (13/14) of the patients were initially diagnosed with other lesions on imaging. A total of 86.7% (13/15) were multifocal. Nodules (11/15, 73.3%) were predominantly peripheral in distribution (12/15, 80.0%). Some cases were associated with hepatic capsular retraction (13/15, 86.7%), "target signs" (8/15, 53.3%) and "lollipop signs" (5/15, 33.3%). Peripheral enhancement of various shapes in the early phase with a progressive centripetal filling was the most common pattern of enhancement (12/15, 80.0%). Abnormal vascularity was seen in 50.7% (6/15) of the patients. Suspicious tumour thromboses in the inferior vena cava were seen in 3 (20.0%) of the patients. Two of the 15 patients (13.3%) had a history of smoking.

CONCLUSIONS: HEHEs have common distinctive features, including multifocal lesions that are predominantly peripheral, "target signs", "lollipop signs", hepatic capsular retraction and peripheral enhancement of various shapes in the early phase with progressive centripetal filling. Additional aggressive imaging features that may be valuable clues to the diagnosis can be identified by CT or MRI.

PMID:36600110 | PMC:PMC9813315 | DOI:10.1186/s13244-022-01344-y

Loss of CDKN2A cooperates with WWTR1(TAZ)-CAMTA1 gene fusion to promote tumor progression in epithelioid hemangioendothelioma

Fetched: January 5th, 2023, 5:01am GMT by Caleb N Seavey

Clin Cancer Res. 2023 Jan 4:CCR-22-2497. doi: 10.1158/1078-0432.CCR-22-2497. Online ahead of print.

ABSTRACT

PURPOSE: Epithelioid hemangioendothelioma (EHE) is a vascular sarcoma caused by the WWTR1(TAZ)-CAMTA1 (TC) gene fusion. This fusion gene has been observed in almost all reported EHE cases and functions as a constitutively activated TAZ. Sequencing of human tumors has, however, identified additional secondary mutations in approximately 50% of EHE, most commonly the loss of tumor suppressor CDKN2A. In this study, the effect of loss of CDKN2A in EHE tumorigenesis was evaluated.

DESIGN: Mice bearing a conditional TC allele were paired with a conditional Cdkn2a knockout allele and an endothelial-specific Cre. Histological characterization and single-cell RNA sequencing of the resultant tumors was performed. EHE cell lines were established through ex vivo culture of tumor cells and evaluated for sensitivity to TEAD inhibition and trametinib.

RESULTS: Loss of Cdkn2a within EHE was associated with more aggressive disease, as displayed by earlier tumor-related morbidity/mortality and enhanced tumor cell proliferation. As no previous EHE cell lines exist, we attempted, successfully, to expand EHE tumor cells ex vivo and produced the first EHE cell lines. These cell lines are "addicted" to the TC oncoprotein, replicate the EHE transcriptional profile, and generate EHE tumors when injected into immunodeficient mice.

CONCLUSION: CDKN2A loss enhances the tumorigenicity of EHE in vivo and enabled the generation of. the first cell lines of this disease. These cell line replicate key facets of the human disease phenotype. Therefore, these cell lines and allograft tumors generated after implantation serve as robust model systems for therapeutic testing of compounds either directed at EHE or at other TAZ-driven cancers.

PMID:36598859 | DOI:10.1158/1078-0432.CCR-22-2497

Permalink for 'Role of magnetic resonance imaging in differentiating intrasellar cavernous hemangioma and pituitary adenoma: A case report-Decipit frons prima multos'

Role of magnetic resonance imaging in differentiating intrasellar cavernous hemangioma and pituitary adenoma: A case report-Decipit frons prima multos

Fetched: January 5th, 2023, 5:01am GMT by Giacomo Bertolini

Neuroradiol J. 2023 Jan 4:19714009221150854. doi: 10.1177/19714009221150854. Online ahead of print.

ABSTRACT

BACKGROUND: Cavernous hemangioma represents a rare vascular malformation usually located in the cavernous sinus that could be exceptionally found purely in the intrasellar region. The clinical presentation of intrasellar cavernous hemangioma (ICH), frequently variable and unspecific, poses the patient at risk for misdiagnosis and the clinical consequences of suboptimal treatment. We present a case of ICH and describe the advanced magnetic resonance imaging (MRI) features that should direct toward the clinical suspicion of ICH.

CASE PRESENTATION: An illustrative case of a 61-year-old man complaining of recurrent headaches and diagnosed with a sellar and parasellar lesion was reported and used as a cue to discuss MRI imaging sequences that may aid in the distinction of ICH from pituitary adenoma and other skull base lesions. Heterogeneous enhancement followed by intense homogeneous enhancement at the dynamic contrast-enhanced sequences ("fill-in" phenomenon), absence of blooming signs at the gradient recalled echo (GRE) T2*-weighted and/or susceptibility-weighted imaging (SWI) MRI sequences, and elevated apparent diffusion coefficient (ADC) values usually characterize ICH instead of pituitary adenoma.

CONCLUSION: Advanced MRI imaging plays an invaluable role in the pre-operative characterization of skull base lesions. Although rare, skull base surgeons should be aware of the ICH in the differential diagnosis process in case of the intrasellar lesion, and a tailored MRI examination should be performed to direct the patient toward the safest and optimal treatment.

PMID:36598406 | DOI:10.1177/19714009221150854

A Case Report of Rare Vocal Cord Hemangioendothelioma in a Paediatric Larynx

Fetched: January 5th, 2023, 5:01am GMT by Aneesh P Azeez

Indian J Otolaryngol Head Neck Surg. 2022 Dec 29:1-4. doi: 10.1007/s12070-022-03418-1. Online ahead of print.

ABSTRACT

Hemangioendothelioma (HEM) is a vascular tumour which is locally aggressive with a low-grade malignant potential. We present a rare case of HEM arising from the vocal cord of a 10-year-old female child. Video laryngoscopy clearly showed a large vocal cord polyp-like mass attached to middle one-third of left cord. Histopathological examination showed fragments of tissue with a neoplasm composed of spindle cells arranged diffusely admixed with numerous small vascular channels. These specific cells were positive for CD31, CD34, vimentin and ERG. Flexiblescopy and narrow band imaging were performed 3 weeks after surgery which showed no residual mass. Treatment of HEM is hampered by lack of proper guidelines and protocol. In this case of localized vocal cord lesion, a wide excision with regular follow up is considered ideal.

PMID:36597551 | PMC:PMC9798936 | DOI:10.1007/s12070-022-03418-1

Un signe précurseur d’hémangiome infantile segmentaire

Fetched: January 4th, 2023, 5:02am GMT by Yi-Teng Hung

CMAJ. 2022 Dec 5;194(47):E1637-E1638. doi: 10.1503/cmaj.220336-f.

NO ABSTRACT

PMID:36588177 | DOI:10.1503/cmaj.220336-f

Non-invasive detection technology in port-wine stain treatment

Fetched: January 4th, 2023, 5:02am GMT by Xue Wang

Chin Med J (Engl). 2022 Nov 5;135(21):2535-2537. doi: 10.1097/CM9.0000000000002124.

NO ABSTRACT

PMID:36583915 | DOI:10.1097/CM9.0000000000002124

An adult case of small bowel intussusception caused by hemangioma presenting with intestinal obstruction: A case report

Fetched: January 4th, 2023, 5:02am GMT by Chengyu Shi

Medicine (Baltimore). 2022 Dec 23;101(51):e32268. doi: 10.1097/MD.0000000000032268.

ABSTRACT

INTRODUCTION: Both small bowel intussusception in adults and small bowel hemangioma are rare benign mass lesions. Moreover, a secondary effect of intussusception caused by hemangioma is extremely rare.

PATIENT CONCERNS: A 87-year-old female suffered from intussusception and intestinal obstruction caused by hemangioma located in the small bowel (February 14, 2020), reporting abdominal distention without nausea and vomiting.

DIAGNOSIS: Emergency abdominal and pelvic computed tomography showed an intussusception with the evidence of associated small bowel obstruction. Histological analysis revealed as small intestinal hemangioma accompanied by mesenteric ulcer.

INTERVENTIONS: The patient underwent segmental resection of intussusception of intestine instead of invalid conservative treatment.

OUTCOMES: Although the postoperative pathological results were inconsistent with preoperative imaging examination, the old woman recovered well.

CONCLUSION: The literature on intussusception of small intestine has described several possible causes including hemangioma, which more likely results in gastrointestinal bleeding or abdominal pain. Yet we experienced a rare case presenting as abdominal distention without nausea and vomiting, Therefore, preoperative diagnosis and localization of these lesions is of great importance. We recommend high resolution contrast-enhanced computed tomography and magnetic resonance imaging should be considered in diagnosis while capsule endoscopy is not available owing to the intestinal obstruction, as long as in facilitating surgical excision.

PMID:36595798 | PMC:PMC9794260 | DOI:10.1097/MD.0000000000032268

Management of the Hand Tumors

Fetched: January 4th, 2023, 5:02am GMT by N K Datta

Mymensingh Med J. 2023 Jan;32(1):135-143.

ABSTRACT

Tumors in the hand are relatively uncommon but 95% are benign. Tumors occurring in the hand, forearm and arm often have unique growth patterns and potential for metastasis that may be different from those seen elsewhere in the body. Secondary metastatic tumors in the hand are very rare (0.1%). Diagnosis is mainly clinical, but X-ray, USG and MRI help as a diagnostic aid. The aim of the study was to early diagnosis, see the pattern and proper management of the hand tumor and ensure good hand function. This prospective study was done from January 2004 to July 2019. We found 220 hand tumors in the hand unit, Department of Orthopaedic Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka Bangladesh. Among 220 patients, male were 114(51.81%) and female were 106 (48.19%). Out of 220 patients we found 190(86.36%) benign tumor and tumor like lesions and 30(13.64%) was malignant hand tumors. Out of 190 benign lesions, benign tumor was 158(83.15%) and 32(16.85%) was tumor like lesions. Among 158 benign tumor, bone tumors were 40(25.31%) and soft tissue tumours were 138(74.69%). Out of soft tissue tumor, peripheral nerve tumor was 20(12.66%). Enchondroma and Giant cell tumors are the most common among the benign bone tumors, on the other hand giant cell tumors of tendon sheath, Glomus tumor, haemangioma, neurofibroma, schwanoma are the common soft tissue tumors. Compound palmar ganglion, fibromatosis and tuberculosis of phalanx are the most common tumor like lesions. Synovial sarcoma 10(33.33%), osteosarcoma 03(10%), chondrosarcoma 03(10%), ewings sarcoma 02(6.66%), fibrosarcoma 04 (13.33%), Malignant fibrous histocytoma 01(3.33%), soft tissue sarcoma 01(3.33%), Merkel cell tumor 01(3.33%), pleomorphic Rabdomyosarcoma 01(3.33%), malignant melanoma 01(3.33%), clear cell sarcoma of tendon and aponeurosis 01(3.33%), undifferentiated carcinoma 01(3.33%) and extra skeletal chondro sarcoma 01(3.33%) were the malignant tumors. Most of the benign lesions recovered fully after excision except neurofibroma and malignant tumors were treated with excision (including amputation) and chemo-radiotherapy successfully, but 4 patients were refereed to higher center due to recurrence and deteriation of hand function and one patient died due to metastasis. Malignant hand tumor management is very difficult even after amputation with multidisciplinary approach. Hand tumor is uncommon and malignant tumors are rare but any abnormal lump or bump in the hand or wrist is considered as tumor. Early detection and intervention are essential for better prognosis and survival for malignant tumors of hands and upper limbs.

PMID:36594313

Homozygous familial hypobetalipoproteinemia caused by APOB gene variations: a case report and review of literature

Fetched: January 4th, 2023, 5:02am GMT by Y Q Zhang

Zhonghua Er Ke Za Zhi. 2023 Jan 2;61(1):70-75. doi: 10.3760/cma.j.cn112140-20220926-00838.

ABSTRACT

Objective: To summarize the genotypes and clinical characteristics of homozygous family hypobetalipoproteinemia (Ho-FHBL) caused by apolipoprotein B (APOB) gene variations. Methods: The clinical, laboratory, genetic, and liver histology data of a boy with Ho-FHBL managed in the hepatology ward of the Children's Hospital of Fudan University in May 2021 were retrospectively analyzed. The literature was searched from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, China VIP database, China Biology Medicine disc and PubMed database (up to May 2022) with "familial hypobetalipoproteinemia" or "hypobetalipoproteinemias" or "hypo beta lipoproteinemia" or "hypolipoproteinemias" as the search terms. All relevant literatures were reviewed to summarize the clinical and genetic features of Ho-FHBL caused by APOB gene variations. Results: The male patient was admitted to the hospital due to abnormal liver function tests for 8 months at the age of 4 years and 6 months. Blood biochemistry showed transaminitis and abnormally low serum levels of lipids. Liver biopsy revealed fatty liver with inflammation and early cirrhosis (Brunt score was F3G2S4). Whole exome sequencing revealed two novel variants of APOB gene (c.3745C>T, p.Q1249 * from the father and c.4589_4592delinsAGGTAGGAGGTTTAACTCCTCCTACCT, p.T1530Kfs * 12 from the mother). He was diagnosed as Ho-FHBL caused by APOB gene compound heterozygous variations. Literature search retrieved 36 English literatures and 0 Chinese literature. A total of 55 (23 males and 32 females) Ho-FHBL cases, including this one, were caused by 54 APOB gene pathogenic variants (23 frameshift, 15 nonsense, 7 missense, 8 splice and 1 gross deletions). The age of the last follow-up was between 1 month and 75 years. Among them, 28 cases had lipid malabsorption, 19 cases had early dysplasia, 12 cases had no symptoms. Twenty-one patients had symptoms related to fat soluble vitamin deficiency, including 14 cases of acanthocytosis, 10 cases of neurological symptoms, and 6 cases of ocular lesions. Thirty-four patients had liver involvement, including 25 cases of elevated transaminase, 21 cases of fatty liver, 15 cases of hepatomegaly, 9 cases of liver fibrosis, 3 cases of liver cirrhosis, 1 case of hepatic hemangioma and 1 case of liver neoplastic nodule. Conclusions: The variants of APOB gene in Ho-FHBL are mainly frameshift and nonsense variations. Patients may have lipid malabsorption and (or) early dysplasia, or symptom-free. Liver involvement is common.

PMID:36594125 | DOI:10.3760/cma.j.cn112140-20220926-00838

Permalink for 'A transfersomes hydrogel patch for cutaneous delivery of propranolol hydrochloride: formulation, in vitro, ex vivo and in vivo studies'

A transfersomes hydrogel patch for cutaneous delivery of propranolol hydrochloride: formulation, in vitro, ex vivo and in vivo studies

Fetched: January 4th, 2023, 5:02am GMT by Changzhao Jiang

J Liposome Res. 2023 Jan 2:1-10. doi: 10.1080/08982104.2022.2162539. Online ahead of print.

ABSTRACT

OBJECTIVE: In this work, a propranolol hydrochloride (PRH) transfersomes loaded cutaneous hydrogel patch was developed for topical drug delivery in the affected area of infantile haemangioma.

METHODS: Sodium cholate was used as the edge activator to prepare the transfersomes. Based on the central composite design, transfersomes hydrogel patch formulation was optimised with 48 h cumulative penetration and time lag as response values. Particle sizes and morphology of the prepared transfersomes were assessed. They were loaded in a cutaneous hydrogel patch, after which their skin permeation abilities were evaluated, and histopathological effects were investigated using guinea pigs. Moreover, in vivo pharmacokinetics studies were performed in rats.

RESULTS: The transfersomes system had a encapsulation efficiency of 81.84 ± 0.53%, particle size of 186.8 ± 3.38 nm, polydispersity index of 0.186 ± 0.002, and a zeta potential of -28.6 ± 2.39 mV. Transmission electron microscopy images revealed sphericity of the particles. The ex vivo drug's penetration of the optimised transfersomes hydrogel patch was 111.05 ± 11.97 μg/cm² through rat skin within 48 h. Assessment of skin tissue did not reveal any histopathological alterations in epidermal and dermal cells. Pharmacokinetic studies showed that skin Cmax (68.22 μg/cm²) and AUC0-24 (1007.33 μg/cm² × h) for PRH transfersomes hydrogel patch were significantly higher than those of commercially available oral dosage form and hydrogel patch without transfersomes. These findings imply that the transfersomes hydrogel patch can prolong drug accumulation in the affected skin area, and reduce systemic drug distribution via the blood stream.

CONCLUSIONS: The hydrogel patch-loaded PRH transfersomes is a potentially useful drug formulation for infantile haemangioma.

PMID:36594110 | DOI:10.1080/08982104.2022.2162539

Case report: Rare epithelioid hemangioendothelioma occurs in both main bronchus and lung

Fetched: January 3rd, 2023, 5:01am GMT by Jiuyu Gong

Front Med (Lausanne). 2022 Dec 15;9:1066870. doi: 10.3389/fmed.2022.1066870. eCollection 2022.

ABSTRACT

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of endothelial origin with low- to intermediate-grade malignant potentials. Since there is no characteristic clinical or biological marker available for PEH, most cases require a surgical lung biopsy for diagnosis. To date, although some patients with PEH reported in the literature were diagnosed through bronchoscopic biopsy, most of the patients still underwent surgical lung biopsy for confirmation. In this case report, we present a rare case diagnosed as PEH through endobronchial biopsies due to the presence of an intraluminal mass that blocked the trachea and caused atelectasis in the right upper lobe. Moreover, since surgery was not appropriate for this patient with unresectable bilateral multiple nodules, we adopted genetic analysis using NGS to provide a guide for personalized treatment. Then, based on the NGS results, the patient was treated with anti-PD-1 mAb and sirolimus for 1 year and has been stable in a 1-year follow-up examination.

PMID:36590968 | PMC:PMC9799331 | DOI:10.3389/fmed.2022.1066870

Vascular neoplasia masquerading as cellulitis and persistent hemorrhagic pericardial effusion

Fetched: January 3rd, 2023, 5:01am GMT by Sharan Thangaraju

Ann Pediatr Cardiol. 2022 May-Jun;15(3):304-307. doi: 10.4103/apc.apc_140_21. Epub 2022 Nov 16.

ABSTRACT

Tufted angioma and kaposiform hemangioendothelioma are considered to represent two ends of the spectrum of benign vascular neoplasms that predominantly present during infancy or early childhood. We report a rare case of a 5-month-old infant with complicated vascular neoplasm involving the pericardial cavity and skin over cervical region, masquerading as infective pericarditis with cellulitis. The patient responded dramatically to therapy with oral prednisolone and sirolimus, with a significant reduction of size of skin lesions and complete resolution of pericardial effusion over 8 weeks. The report also highlights the importance of a multidisciplinary team in managing such complicated cases.

PMID:36589655 | PMC:PMC9802615 | DOI:10.4103/apc.apc_140_21

Spindle cell haemangioma - Report of a rare entity

Fetched: January 3rd, 2023, 5:01am GMT by Vindhya Savithri

J Oral Maxillofac Pathol. 2022 Jul-Sep;26(3):424. doi: 10.4103/jomfp.jomfp_490_20. Epub 2022 Oct 17.

ABSTRACT

Spindle cell haemangioma is a rare benign vascular neoplasm. Its occurrence in the oral cavity is uncommon and may be mistaken for other common oral lesions such as mucoceles or fibromas. Microscopically, it has features which are similar to those of other vascular neoplasms and may be a cause for potential mis-diagnosis. In this article, we report a case in the lower lip of a young male patient.

PMID:36588825 | PMC:PMC9802532 | DOI:10.4103/jomfp.jomfp_490_20

Recurrent posterior fossa intracranial capillary hemangioma in a pregnant woman: A case report and review of literature

Fetched: December 31st, 2022, 5:02am GMT by Mohammad Ali Abouei Mehrizi

Ann Med Surg (Lond). 2022 Nov 15;84:104913. doi: 10.1016/j.amsu.2022.104913. eCollection 2022 Dec.

ABSTRACT

INTRODUCTION: Intracranial capillary hemangioma (ICH) is a rare tumor with a slightly higher incidence in women. Surgical management of ICH during pregnancy requires a joint decision, for it has been a contentious issue owing to the inadequate number of cases.

PRESENTATION OF CASE: A 23-year-old female with an ICH in the posterior fossa underwent subtotal tumor resection (STR). One year later, she was referred to the neurosurgery department in the 8th week of pregnancy due to a progressive headache, vomit, and a bulging occipital mass. Subsequent to an abortion, preoperative angiography and Bleomycin injection were performed, and the tumor was totally resected thereafter. Over a one-year follow-up, her symptoms faded, and she experienced no recurrence.

DISCUSSION: ICH, a highly vascular entity, poses a substantial intraoperative bleeding risk. During the first surgery, intraoperative bleeding deterred the surgeon from a gross total resection (GTR), leading to a recurrence due to the growth of residual tissue during pregnancy. Having aborted the fetus, the patient underwent angioembolization to lessen the risk of intraoperative bleeding before reoperation, rendering it more possible for the surgeon to achieve GTR. Accordingly, quality of resection and a multidisciplinary approach is necessary to ensure optimal treatment.

CONCLUSION: There is a growing consensus that not only is STR a contributory factor in ICH recurrence, but female hormones and increased cardiac output in pregnancy might play a pivotal role in the progression of the tumor. Thus, paying further attention to pregnant or peripartum patients with suspected ICH should assume greater significance.

PMID:36582875 | PMC:PMC9793240 | DOI:10.1016/j.amsu.2022.104913

A Prospective Audit Comparing Optos Widefield Imaging to Fundus Examination for Von Hippel-Lindau Retinal Screening

Fetched: December 31st, 2022, 5:02am GMT by Naeem Iqbal

Cureus. 2022 Dec 22;14(12):e32814. doi: 10.7759/cureus.32814. eCollection 2022 Dec.

ABSTRACT

Background Von Hippel-Lindau (VHL) disease is an autosomal dominant multisystem disorder caused by germline mutations at chromosome 3p25-26 in the VHLtumour suppressor gene. Retinal manifestations include capillary haemangiomas that develop in up to 80% of gene carriers. Lifelong retinal surveillance involves yearly assessment usually by fundoscopy and often as part of a VHL multidisciplinary clinic. Optos ultra-widefield retinal imaging is now becoming more widely used in virtual retinal screening clinics. We aimed to assess discrepancies in the pickup rate of angioma and angiomatous-associated disease between slit-lamp fundoscopy and Optos ultra-widefield imaging. Methodology A total of 49 patients had both Optos ultra-widefield retinal imaging and slit-lamp fundoscopy over 16 months in VHL retinal surveillance clinics at the John Radcliffe Hospital, Oxford, UK. Optos images were analysed for image quality and presence of angioma(s) by a Consultant Ophthalmologist who was masked to the fundoscopy findings. The pickup rate was compared between slit-lamp fundoscopy and Optos imaging. Results In total, data on 94 eyes were collected. Of the total Optos retinal images, 12.8% were positive for angiomas compared to 11.7% from the slit-lamp examination. There was a discrepancy of 1.1% (one value) where the Optos image analysis suggested a possible angioma, which was not identified on slit-lamp examination. Optos imaging identified all angiomas in this cohort. Conclusions Optos imaging was non-inferior to slit-lamp examination in this sample of 94 eyes. In the current COVID-19 climate, reducing clinician-patient interaction is important. This research supports providing retinal imaging as an acceptable alternative to the yearly slit-lamp fundus examination.

PMID:36582420 | PMC:PMC9794529 | DOI:10.7759/cureus.32814

Infant in extremis: respiratory failure secondary to lower airway infantile hemangioma

Fetched: December 30th, 2022, 5:02am GMT by Matthew S MacDougall

BMC Pediatr. 2022 Dec 30;22(1):744. doi: 10.1186/s12887-022-03821-1.

ABSTRACT

BACKGROUND: Infantile hemangiomas (IHs) are vascular tumors that commonly affect infants and usually regress spontaneously or can be easily treated as an outpatient with topical beta-blockers. However, IHs that present in the airway may cause life-threatening symptoms due to airway obstruction or risk of bleeding. Here we present the first documented case of an infant with rapid deterioration and acute respiratory failure secondary to a lower airway hemangioma.

CASE PRESENTATION: This 3-month-old male initially presented in respiratory distress with symptoms consistent with a viral respiratory infection, however showed no clinical improvement with standard therapies. An urgent CT scan revealed a mass occluding the right mainstem bronchus. Upon transfer to a tertiary care facility, he developed acute respiratory failure requiring emergent intubation and single lung ventilation. The availability of multiple subspecialists allowed for stabilization of a critically ill child, expedited diagnosis, and ultimately initiation of life-saving treatment with beta blockers. After 17 total hospital days, he was extubated successfully and discharged home in good condition.

CONCLUSIONS: While IH is a rare cause of infantile respiratory distress, we present multiple pearls for the general pediatrician for management of IHs of the airway.

PMID:36581920 | PMC:PMC9801545 | DOI:10.1186/s12887-022-03821-1

Cryosurgery of multiple haemangiomas of oral cavity

Fetched: December 30th, 2022, 5:02am GMT by Pavithran Ashokkumar

BMJ Case Rep. 2022 Dec 29;15(12):e253654. doi: 10.1136/bcr-2022-253654.

ABSTRACT

Haemangiomas of the oral cavity are common benign vascular tumours of infancy. Several treatment modalities are described for haemangiomas, including sclerotherapy, embolisation, laser surgery and cryotherapy. Cryotherapy is the application of varying extremes of low temperatures to destroy abnormal tissue. Since cryosurgery is effective, simple and easy to perform, it is used in the treatment of lesions in both medicine and dentistry. Cryosurgery provides many advantages such as easy operation, absence of intraoperative bleeding and low infection rate. In this case, the cryosurgical treatment of a young patient who suffered from multiple haemangiomas of the oral cavity that was refractory to medical treatment is presented.

PMID:36581363 | PMC:PMC9806050 | DOI:10.1136/bcr-2022-253654

Masseteric giant capillary haemangioma: a bulky mass treated with scarless and minimally invasive surgery

Fetched: December 30th, 2022, 5:02am GMT by Flavia Cascino

BMJ Case Rep. 2022 Dec 29;15(12):e252579. doi: 10.1136/bcr-2022-252579.

ABSTRACT

In this article, the authors report their management with minimally invasive surgery and embolisation of a bulky intramuscular capillary haemangioma. Masseteric capillary haemangioma is a rare tumour in adults: in cases of large-sized tumours we suggest a multidisciplinary approach in choosing the best treatment and minimally invasive, scarless surgery.

PMID:36581357 | PMC:PMC9806058 | DOI:10.1136/bcr-2022-252579

Infantile Kaposiform hemangioendothelioma in a female patient complicated with severe obstructed jaundice: a case report

Fetched: December 30th, 2022, 5:02am GMT by Eiichiro Watanabe

Surg Case Rep. 2022 Dec 29;8(1):225. doi: 10.1186/s40792-022-01581-9.

ABSTRACT

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular neoplasm that occurs mainly in the pediatric population. KHE usually originates just underneath the skin and affects deeper tissues through infiltrative growth; however, visceral tissue involvement is quite rare.

CASE PRESENTATION: An 8-month-old girl with jaundice and acholic stool was referred to our hospital for further evaluation of a hepatoduodenal ligament tumor. A blood examination revealed high bilirubin and liver enzyme levels, but no signs of coagulopathy. The first attempt at a diagnostic surgical procedure did not provide sufficient diagnostic information. However, the histopathological diagnosis of the cystic duct excised in the second surgery indicated KHE. Therefore, in our case, KHE was considered a cause of obstructive jaundice. Sirolimus (rapamycin) was initiated, and the patient was discharged 7 months after admission.

CONCLUSIONS: In cases of atypical hypervascular lesions in the abdominal cavity, especially in the pediatric population, it is important to consider the possibility of KHE, and surgical intervention with proper strategies is required for diagnosis, followed sequentially by promising treatments.

PMID:36580178 | PMC:PMC9800675 | DOI:10.1186/s40792-022-01581-9

Targetoid Hemosiderotic Hemangioma (Hobnail Hemangioma)

Fetched: December 30th, 2022, 5:02am GMT by Lluís Corbella-Bagot

J Cutan Med Surg. 2022 Dec 29:12034754221147292. doi: 10.1177/12034754221147292. Online ahead of print.

NO ABSTRACT

PMID:36579733 | DOI:10.1177/12034754221147292

Atypical aggressive vertebral hemangioma of the sacrum with postoperative recurrence: A case report

Fetched: December 30th, 2022, 5:02am GMT by Guan-Xi Wang

World J Clin Cases. 2022 Dec 6;10(34):12648-12653. doi: 10.12998/wjcc.v10.i34.12648.

ABSTRACT

BACKGROUND: Aggressive vertebral hemangioma (VH) is an uncommon lesion in the adult population. The vast majority of aggressive VHs have typical radiographic features. However, preoperative diagnosis of atypical aggressive VH may be difficult. Aggressive VHs are likely to recur even with en bloc resection.

CASE SUMMARY: A 52-year-old woman presented with a 3-mo history of numbness and pain in her right lower extremity. Physical examination showed sacral tenderness and limited mobility, and the muscle strength was grade 4 in the right digital flexor. Computed tomography revealed osteolytic bone destruction from S1 to S2. Magnetic resonance imaging (MRI) showed that the mass was compressing the dural sac; it was heterogeneously hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, and gadolinium contrast enhancement showed that the tumor was heterogeneously enhanced and invading the vertebral endplate of S1. The patient developed progressive back pain and numbness in the bilateral extremities 6 mo postoperatively, and MRI examination showed recurrence of the mass. The mass was larger in size than before the operation, and it was extending into the spinal canal.

CONCLUSION: The radiographic findings of atypical aggressive VH include osteolytic vertebral bone destruction, extension of the mass into the spinal canal, and heterogeneous signal intensity on T1-, T2-, and enhanced T1-weighted MRI. These characteristics make preoperative diagnosis difficult, and biopsy is necessary to verify the lesion. Surgical decompression and gross total resection are recommended for treatment of aggressive VH. However, recurrence is inevitable in some cases.

PMID:36579090 | PMC:PMC9791517 | DOI:10.12998/wjcc.v10.i34.12648

Lobular Capillary Haemangioma Occurring Over Port-Wine Stain in a Patient of Sturge-Weber Syndrome: A Case Report

Fetched: December 30th, 2022, 5:02am GMT by A Ramesh

Indian J Dermatol. 2022 Jul-Aug;67(4):481. doi: 10.4103/ijd.ijd_78_21.

NO ABSTRACT

PMID:36578767 | PMC:PMC9792022 | DOI:10.4103/ijd.ijd_78_21

Permalink for 'A Nodular GLUT1 Positive Verrucous Haemangioma Arising Over an Acral Pseudolymphomatous Angiokeratoma of Children (APACHE): A Diagnosis of Rarity'

A Nodular GLUT1 Positive Verrucous Haemangioma Arising Over an Acral Pseudolymphomatous Angiokeratoma of Children (APACHE): A Diagnosis of Rarity

Fetched: December 30th, 2022, 5:02am GMT by Ojas Dipak Kulkarni

Indian J Dermatol. 2022 Jul-Aug;67(4):450-451. doi: 10.4103/ijd.ijd_1127_20.

NO ABSTRACT

PMID:36578716 | PMC:PMC9792041 | DOI:10.4103/ijd.ijd_1127_20

Permalink for 'The Application of Color Doppler Ultrasound in the Evaluation of the Efficacy of 595-nm Pulsed Dye Laser Combined with 755-nm Long-Pulse Alexandrite Laser in the Treatment of Hybrid Infantile Hemangiomas'

The Application of Color Doppler Ultrasound in the Evaluation of the Efficacy of 595-nm Pulsed Dye Laser Combined with 755-nm Long-Pulse Alexandrite Laser in the Treatment of Hybrid Infantile Hemangiomas

Fetched: December 30th, 2022, 5:02am GMT by Yuwen Lu

Clin Cosmet Investig Dermatol. 2022 Dec 22;15:2831-2839. doi: 10.2147/CCID.S393962. eCollection 2022.

ABSTRACT

PURPOSE: We used color Doppler ultrasound to conduct an objective evaluation of the 595-nm Pulsed Dye Laser (PDL) combined with 755-nm long-pulse alexandrite sequential laser treatment for hybrid IH.

PATIENTS AND METHODS: A total of 116 cases of hybrid IH were selected for this study. The interval between laser treatments was around 4 weeks, and 6 laser treatments or complete removal of the tumor was the end point. All children underwent color Doppler ultrasonography at the 0th, 1st, 6th months of treatment. Children were grouped by gender, age (<6 months, ≥6 months), thickness (<8 mm, ≥8 mm), and location (face and neck, trunk, and extremities). Calculate the volume of IHs according to color Doppler ultrasound. The volume ratio before and after treatment was defined as the A-value. Treatment outcomes were defined as effective when the A-value <75%.

RESULTS: In total, 74 cases (63.79%) had effective outcomes. Overall, the samples showed a statistically significant difference in the reduction of IH volume after 6 months of laser treatment (P < 0.001). The treatment of <6 months group had better efficacy than the ≥6 months group (P < 0.001), the treatment of thickness <8 mm group had better efficacy than the thickness ≥8 mm group (P < 0.001) and there was no significant difference in efficacy between the three different location groups (P > 0.05). Greater reduction in blood flow in the group with the effective outcome than in the group with the ineffective outcome (P < 0.001).

CONCLUSION: Color Doppler ultrasound can be applied to the diagnosis of hybrid IH and to the evaluation of treatment time and outcomes, and it can help clinicians recognize hybrid IH with greater accuracy. The earlier intervention for hybrid IH we perform, the better outcomes will be.

PMID:36578671 | PMC:PMC9792221 | DOI:10.2147/CCID.S393962

Hamartomas of the Retina and Optic Disc

Fetched: December 30th, 2022, 5:02am GMT by Ibadulla Mirzayev

Turk J Ophthalmol. 2022 Dec 28;52(6):421-431. doi: 10.4274/tjo.galenos.2022.25979.

ABSTRACT

Hamartomas are local malformation of cells that demonstrate abnormal proliferation in the area where they are normally present. Retinal and optic disc hamartomas include astrocytic hamartoma, congenital hypertrophy of the retinal pigment epithelium (CHRPE), simple congenital hamartoma of the retinal pigment epithelium (CSHRPE), combined hamartoma of the retina and retinal pigment epithelium (CHRRPE), retinal hemangioblastoma (retinal capillary hemangioma), and retinal cavernous hemangioma. Retinal and optic disc hamartomas can be observed sporadically as well as with systemic associations. Astrocytic hamartoma usually appears as a flat, transparent yellowish lesion. CHRPE is a round, pigmented, and flat lesion. CSHRPE usually presents as a dark black macular tumor. CHRRPE consists of vascular, glial, and pigment epithelial components, which can demonstrate peripapillary, macular, and peripheral localization. Retinal hemangioblastoma is a vascular tumor, red-pink in color with tortuous and dilated afferent and efferent vessels, typically located in the peripheral retina or optic disc. Retinal cavernous hemangioma is characterized by the formation of thin-walled saccular angiomatous structures in the retina or optic nerve head resembling concord grapes. Ultrasonography, fundus autofluorescence, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography methods are used in the diagnosis of retinal and optic disc hamartomas. Some retinal and optic disc hamartomas do not require treatment. However, complications including vitreous hemorrhage, macular exudation, retinal detachment, macular hole, epiretinal membrane, and choroidal neovascularization require treatment.

PMID:36578224 | PMC:PMC9811234 | DOI:10.4274/tjo.galenos.2022.25979

Sinonasal Lobular Capillary Hemangioma After Continuous Self-tests for COVID-19: A Case Report and Review of the Literature

Fetched: December 30th, 2022, 5:02am GMT by Athanasios Luca Fountarlis

Medeni Med J. 2022 Dec 28;37(4):339-345. doi: 10.4274/MMJ.galenos.2022.00533.

ABSTRACT

Sinonasal lobular capillary hemangiomas (LCH) are rare benign vascular lesions commonly arising from the nasal septum. Nasopharyngeal, nasal mid-turbinate, and anterior nasal swabbing is the preferred method of screening for coronavirus disease-2019 (COVID-19). Herein, we present a case of a sinonasal LCH in a child after continuous self-tests for COVID-19, with an anterior nasal swab. The child presented with a well-defined red mass in the anterior part of the nasal septum, which was removed endoscopically. Histopathology revealed a LCH. This is the first report of a complication other than epistaxis with the use of an anterior nasal swab. Our literature review identified 32 studies reporting complications of COVID-19 screening. Cerebrospinal fluid leaks and foreign body retention are the most common ones. A proper specimen collection technique and a quick patient history with an emphasis on risk factors are the best practices to prevent complications from COVID-19 screening.

PMID:36578162 | PMC:PMC9808853 | DOI:10.4274/MMJ.galenos.2022.00533

Early propranolol treatment of infantile hemangiomas improves outcome

Fetched: December 29th, 2022, 5:02am GMT by Ana Giachetti

An Bras Dermatol. 2022 Dec 26:S0365-0596(22)00316-6. doi: 10.1016/j.abd.2022.04.008. Online ahead of print.

ABSTRACT

BACKGROUND: Infantile hemangiomas (IH) are the most common soft tissue tumors of childhood. Although most of these tumors are not worrisome, some IH may be life or function-threatening, can lead to permanent disfigurement, or have associated structural congenital anomalies, requiring early recognition and referral to specialists for treatment consideration. Since 2008, oral propranolol has been widely considered to be the first-line treatment for IH.

OBJECTIVES: To evaluate aesthetic and functional outcome in propranolol-treated infantile hemangiomas according to the age of treatment onset.

METHODS: Retrospective, observational study of infantile hemangioma patients under 4 years of age at the time of diagnosis, treated with oral propranolol. Evaluated parameters included: pre and post-treatment morphologic/aesthetic aspects of the hemangioma, total resolution rate, degree of functional compromise of affected areas and its evolution. Two independent pediatric dermatologists evaluated all cases reviewing clinical data from medical records and comparing clinical photographs taken at initiation and at the end of treatment of each patient. Data were analyzed with STATA 13.0 program.

RESULTS: The cohort included 138 patients, with a female predominance. The median age at therapy onset was 3 months. The morphological/aesthetic improvement rate was 99% (95% CI 96‒99), the total resolution rate was 48% (95% CI 44‒60) and the functional improvement rate reached 100%. When comparing total resolution outcome versus age when treatment started, the improvement was larger in younger patients (3.5 vs. 4.9 months, p = 0.01). When comparing the total resolution rate in those younger or older than 3 months at treatment initiation, the percentage of total resolution in the younger group was 57% vs. 40% in the older one (p = 0.05).

STUDY LIMITATIONS: Retrospective design; patients photographs were the sole indicators used to measure regression rates. Visual assessment is subjective.

CONCLUSION: The present results strongly suggest that early (before 3 months of age) initiation of treatment of infantile hemangiomas with propranolol results in significantly higher aesthetic and functional improvement rates and a higher percentage of total resolution.

PMID:36577593 | DOI:10.1016/j.abd.2022.04.008

Improving liver lesions classification on CT/MRI images based on Hounsfield Units attenuation and deep learning

Fetched: December 28th, 2022, 5:01am GMT by Anh-Cang Phan

Gene Expr Patterns. 2022 Nov 28;47:119289. doi: 10.1016/j.gep.2022.119289. Online ahead of print.

ABSTRACT

The early sign detection of liver lesions plays an extremely important role in preventing, diagnosing, and treating liver diseases. In fact, radiologists mainly consider Hounsfield Units to locate liver lesions. However, most studies focus on the analysis of unenhanced computed tomography images without considering an attenuation difference between Hounsfield Units before and after contrast injection. Therefore, the purpose of this work is to develop an improved method for the automatic detection and classification of common liver lesions based on deep learning techniques and the variations of the Hounsfield Units density on computed tomography scans. We design and implement a multi-phase classification model developed on the Faster Region-based Convolutional Neural Networks (Faster R-CNN), Region-based Fully Convolutional Networks (R-FCN), and Single Shot Detector Networks (SSD) with the transfer learning approach. The model considers the variations of the Hounsfield Unit density on computed tomography scans in four phases before and after contrast injection (plain, arterial, venous, and delay). The experiments are conducted on three common types of liver lesions including liver cysts, hemangiomas, and hepatocellular carcinoma. Experimental results show that the proposed method accurately locates and classifies common liver lesions. The liver lesions detection with Hounsfield Units gives high accuracy of 100%. Meanwhile, the lesion classification achieves an accuracy of 95.1%. The promising results show the applicability of the proposed method for automatic liver lesions detection and classification. The proposed method improves the accuracy of liver lesions detection and classification compared with some preceding methods. It is useful for practical systems to assist doctors in the diagnosis of liver lesions. In our further research, an improvement can be made with big data analysis to build real-time processing systems and we expand this study to detect lesions from all parts of the human body, not just the liver.

PMID:36574537 | DOI:10.1016/j.gep.2022.119289

Permalink for 'Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma: Extremely Wide Morphologic Spectrum and Immunophenotype'

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma: Extremely Wide Morphologic Spectrum and Immunophenotype

Fetched: December 28th, 2022, 5:01am GMT by Yan Li

Am J Surg Pathol. 2022 Dec 28. doi: 10.1097/PAS.0000000000002011. Online ahead of print.

ABSTRACT

Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell (FDC) sarcoma (EBV+ IFDCS) is a rare entity, and its histopathological characteristics have not been fully described. Here, we investigated the wide morphologic spectrum and immunophenotype of this tumor with the aim to help avoid misdiagnosis. Thirteen cases of EBV+ IFDCS were retrospectively analyzed, combined with a review of 70 cases reported in the literature. The median age of patients was 49 (range, 29 to 67 y). Six patients were male and 7 were female. Most cases (92.3%, 12/13) occurred in the liver or spleen, and only 1 case affected an extra-hepatosplenic site (lung, 7.7%, 1/13). Tumors were assessed for a variety of histologic features and assigned to the following morphologic groups: classic type (53.8%, 7/13), lymphoma-like subtype (38.5%, 5/13), and hemangioma-like subtype (7.7%, 1/13). The classic type had distinct EBV-positive neoplastic cells with a fascicular or storiform growth pattern, variable lymphoplasmacytic infiltrates, and blood vessels. The lymphoma-like subtype had extremely prominent lymphoplasmacytic infiltrates (resembling marginal zone lymphoma with plasmacytoid differentiation) with singly dispersed distinct EBV-positive neoplastic cells, highlighted by in situ hybridization for EBV-encoded small RNA. The hemangioma-like subtype had extremely prominent blood vessels with hyaline and/or fibrinoid degeneration, singly dispersed distinct EBV-positive neoplastic cells, and limited lymphoplasmacytic infiltrates. Immunohistochemically, the neoplastic cells showed variable staining for FDC markers (CD21, CD35, CD23, and SSTR2) and the fibroblastic marker SMA, with the staining ranging from very focal to extensive. The number of EBV-positive neoplastic cells ranged from 80 to 400/HPF. All cases showed variable expression of PD-ligand 1 (PD-L1) (CPS: 5-90). IgG4-positive cells ranged from rare up to 100/HPF. Interestingly, 2 cases satisfied the criteria proposed in a previous study, mimicking IgG4-related disease. EBV+ IFDCS is an entity with an extremely wide morphologic spectrum and immunophenotype. Awareness of the spectrum of morphologic presentations of this rare tumor, specifically the lymphoma-like subtype and hemangioma-like subtype, is important for accurate diagnosis.

PMID:36574358 | DOI:10.1097/PAS.0000000000002011

KIAA1429 promotes infantile hemangiomas regression by facilitating the stemness of hemangioma endothelial cells

Fetched: December 27th, 2022, 5:02am GMT by Luying Wang

Cancer Sci. 2022 Dec 26. doi: 10.1111/cas.15708. Online ahead of print.

ABSTRACT

Infantile hemangiomas are common vascular tumors with a specific natural history. The proliferation and regression mechanism of infantile hemangiomas may be related to the multilineage differentiation ability of hemangioma stem cells, but the specific mechanism is not well elucidated. KIAA1429 is an N⁶ -methyladenosine methylation-related protein that can also exert its role in a methylation-independent manner. This study aims to explore the function of KIAA1429 in infantile hemangiomas. qRT-PCR, western blotting, and immunostaining were performed to verify the expression of KIAA1429. The endothelial and fibroblast-like phenotypes of hemangioma endothelial cells were detected after KIAA1429 knockdown and overexpression. The stemness properties of hemangioma endothelial cells and the underlying mechanism of KIAA1429 in hemangiomas were also investigated. Nude mouse models of infantile hemangiomas were conducted to ascertain the effects of KIAA1429 in vivo. The results showed that KIAA1429 was highly expressed in infantile hemangiomas, particularly in involuting hemangiomas. In vitro experiments confirmed that KIAA1429 inhibited the endothelial phenotype, enhanced the differentiation ability, and promoted the fibroblast-like phenotype of hemangioma endothelial cells by inducing endothelial cell transition to facultative stem cells. However, the effect of KIAA1429 on the potential target was shown to be independent of N⁶ -methyladenosine methylation modification. Mouse models further revealed that KIAA1429 could inhibit the proliferation and promote the regression of hemangiomas. In conclusion, this study found that KIAA1429 played an important role in the regression of infantile hemangiomas by enhancing the stemness of hemangioma endothelial cells and could be a potential treatment target for infantile hemangiomas.

PMID:36572002 | DOI:10.1111/cas.15708

Isolated cardiac valve involvement in smoldering adult T-cell leukemia/lymphoma

Fetched: December 26th, 2022, 5:01am GMT by Cristian Aguilar

Cardiovasc Pathol. 2022 Dec 23;64:107513. doi: 10.1016/j.carpath.2022.107513. Online ahead of print.

ABSTRACT

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive mature T-cell neoplasm caused by infection with the Human T-cell Lymphotropic Virus Type 1 (HTLV-1). Cardiac involvement in patients with ATLL is infrequent, and when it happens it is usually seen in aggressive ATLL subtypes. However, ATLL presenting as isolated cardiac valve involvement is extremely rare. To date, only three histologically proven cases of ATLL with isolated cardiac valve involvement have been reported. Herein, we describe a 61-year-old Peruvian man who presented heart failure symptoms secondary to progressive cardiac valve infiltration. The patient underwent mitral valve replacement with a mechanical prosthesis. Histopathological evaluation of the resected valve revealed leaflet thickening with a nodular appearance due to fibrous tissue containing atypical T-lymphocytes with Foxp3 expression, infiltrating all layers of the resected valve. Interestingly, tumor cells were distributed around an incidental venous malformation (i.e., cavernous hemangioma). Postoperative evaluation demonstrated positive serology for HTLV-1, and a diagnosis of ATLL was established. Postoperative positron emission tomography/computed tomography did not show lesions outside the heart and cell blood counts were within normal range with low level of circulating CD4+ CD25+ lymphoma cell counts (7%); therefore, patient's disease was considered as smoldering ATLL and a "watch and wait" strategy was pursued. Currently, the patient is alive with no progression of disease after 18 months from diagnosis. Isolated cardiac valve involvement by ATLL should be considered in the differential diagnosis of HTLV-1 carriers with progressive heart failure, even when systemic lymphoma involvement is absent or not apparent.

PMID:36567046 | DOI:10.1016/j.carpath.2022.107513

Intralesional steroid in the era of propranolol for infantile hemangioma-Do we need it?

Fetched: December 26th, 2022, 5:01am GMT by Anand Pandey

J Plast Reconstr Aesthet Surg. 2022 Nov 24;77:117-122. doi: 10.1016/j.bjps.2022.11.038. Online ahead of print.

ABSTRACT

INTRODUCTION: Despite its effectivity, there are reports of poor response to propranolol in the treatment of infantile hemangioma (IH). The literature is limited to the type of IH that fails to respond to propranolol. This study was conducted to analyze which types of hemangiomas respond poorly to propranolol and the effects of intralesional triamcinolone (IL TMC) in them.

MATERIAL AND METHODS: In this prospective cohort study, IH was classified as superficial, deep, and mixed. The clinical details were recorded. Propranolol was started in the patients at a dose of 1 mg/kg/day and increased to 2-3 mg/kg/day. The response to the treatment was evaluated as excellent, good, poor, and no response. IL TMC was given in the non-responding group at a dose of 1-2 mg/kg at one-month interval for a total of six doses after stopping propranolol.

RESULTS: Ninety-six patients (median age, 7 months; M/F = 2:1) were treated. Superficial hemangioma was present in 40 (41.7%), deep in 10 (10.4%), and mixed in 46 (47.9%) patients. The response was statistically better if initiated within four months of age. It was not influenced by the sex, number, site, or size. The response was statistically better in superficial hemangioma. IL TMC was administered in the 16 patients. The response was good or excellent in 10 patients.

CONCLUSION: Propranolol will be used as a first-line drug for IH. All superficial IHs are likely to respond. There will be a possibility of non-responding mixed or deep IH. Use of IL TMC seems reasonable for IH not responding to propranolol.

PMID:36566639 | DOI:10.1016/j.bjps.2022.11.038

Radiological and pathological characteristics of synovial hemangioma of the knee

Fetched: December 24th, 2022, 5:02am GMT by Tatsuru Sonobe

Exp Ther Med. 2022 Nov 23;25(1):23. doi: 10.3892/etm.2022.11722. eCollection 2023 Jan.

ABSTRACT

Synovial hemangioma, a rare benign tumor that occurs most frequently in the knee in children and young adults, has four histological subtypes: Venous, arteriovenous, cavernous and capillary hemangiomas. Since the clinical presentation and radiological findings of synovial hemangioma are non-specific, there is frequently a long period between the onset and the diagnosis. The cases of nine patients, pathologically diagnosed with synovial hemangioma and surgically treated, were retrospectively analyzed. All nine patients had persistent knee pain. In addition, three patients also had a swollen knee with intra-articular hemorrhage. Plain radiography revealed intra-articular phleboliths in two patients. In seven patients, T1-weighted magnetic resonance imaging showed low signal intensity with small signal voids. On T2-weighted imaging, all patients showed high signal intensity containing small signal voids. All patients underwent surgical excision; there was no postoperative recurrence after the final operation, and the knee pain had disappeared at the final follow-up. From the pathological findings, the diagnoses were venous hemangioma, cavernous hemangioma and capillary hemangioma (three patients each).

PMID:36561614 | PMC:PMC9748666 | DOI:10.3892/etm.2022.11722

Giant breast cavernous hemangioma

Fetched: December 24th, 2022, 5:02am GMT by Ho Xuan Tuan

Radiol Case Rep. 2022 Dec 12;18(2):697-700. doi: 10.1016/j.radcr.2022.11.050. eCollection 2023 Feb.

ABSTRACT

Although breast vascular tumors are exceedingly rare, the benign group's most common tumor, the hemangioma, is frequently found in lumpectomy or mastectomy tissues during histological analyses. The 3 types of hemangiomas are capillary, cavernous, and venous. The most typical kind of hemangioma is cavernous. Cavernous hemangiomas are benign blood vessel tumors that arise from established blood vessels. The best imaging technique for examining the makeup of breast vessels is magnetic resonance imaging. In this article, we purposed to describe an exceptionally uncommon case of giant breast cavernous hemangioma.

PMID:36561544 | PMC:PMC9763672 | DOI:10.1016/j.radcr.2022.11.050

Retiform Hemangioendothelioma: A Rare Tumor in the Medial Canthus: Case Report and Review of Literature

Fetched: December 24th, 2022, 5:02am GMT by Shalaka Khade

J Cutan Aesthet Surg. 2022 Jul-Sep;15(3):319-322. doi: 10.4103/JCAS.JCAS_186_21.

ABSTRACT

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with intermediate malignant potential mostly occurring in extremities. It is important to differentiate this neoplasm from malignant conditions as RH has a good prognosis. As it has a tendency to recur locally, it may be misdiagnosed as a malignant tumor. Herein, we report a rare case of RH occurring in the medial canthus.

PMID:36561409 | PMC:PMC9764955 | DOI:10.4103/JCAS.JCAS_186_21

Infantile hemangioma in a 17th century painting

Fetched: December 24th, 2022, 5:02am GMT by Nicolas Kluger

J Eur Acad Dermatol Venereol. 2022 Dec 22. doi: 10.1111/jdv.18833. Online ahead of print.

NO ABSTRACT

PMID:36560847 | DOI:10.1111/jdv.18833

"Face(s)" of a PHACE(S) Syndrome Patient before and after Therapy: Particular Case Report and Review of Literature

Fetched: December 24th, 2022, 5:02am GMT by Maria-Corina Stănciulescu

Children (Basel). 2022 Dec 15;9(12):1970. doi: 10.3390/children9121970.

ABSTRACT

A rare, uncommon disorder called PHACE(S) (P-posterior fossa anomalies, H-hemangioma, A-arterial anomalies, C-cardiac anomalies, E-eye anomalies, and S-sternal cleft) of unknown etiology was rarely reported. Children are susceptible to developing PHACE(S) syndrome from the moment they are born. It may be challenging for a physician to appropriately diagnose and treat children with PHACE due to the multifaceted nature of the disease and the extensive range of consequences that may be associated with it. A one-month-old newborn girl was admitted to hospital with extensive, multiple facial infantile hemangiomas, ulceration of the lower lip hemangioma-like lesion, cardiovascular, sternal, and neurological concomitant malformations. Five days following the initial application of the medication, systemic treatment with propranolol and topical treatment with silver sulfadiazine produced their first noticeable benefits. The lip ulceration was mostly healed and facial hemangioma started to regress. The regression continued under therapy and this effect persists for 6 months since Propranolol therapy ended. No cardiovascular or neurological clinical events have been registered during follow-up. The present case has three peculiarities: (1) high number of facial hemangiomas; (2) presence of subependymal cyst not yet reported in the literature associated with PHACE syndrome; and (3) lack of cardiovascular events during therapy knowing that these events frequently appear in PHACE syndrome patients.

PMID:36553413 | PMC:PMC9776585 | DOI:10.3390/children9121970

Permalink for 'Analysis of Therapeutic Decisions for Infantile Hemangiomas: A Prospective Study Comparing the Hemangioma Severity Scale with the Infantile Hemangioma Referral Score'

Analysis of Therapeutic Decisions for Infantile Hemangiomas: A Prospective Study Comparing the Hemangioma Severity Scale with the Infantile Hemangioma Referral Score

Fetched: December 24th, 2022, 5:02am GMT by Tong Qiu

Children (Basel). 2022 Nov 28;9(12):1851. doi: 10.3390/children9121851.

ABSTRACT

BACKGROUND: In view of the high incidence of infantile hemangioma (IH) in infants and young children, a comprehensive and reasonable evaluation scale for referral is urgently needed. This study compared the influence of the Hemangioma Severity Scale (HSS) and the Infantile Hemangioma Referral Score (IHReS) on treatment decisions for infantile hemangioma patients.

OBJECTIVE: We aimed to establish a reliable and effective evaluation method for referral.

METHODS: This was a prospective study to determine whether treatment was needed for IH patients after evaluation with the HSS and IHReS.

RESULTS: A total of 266 consecutive referred IH patients were evaluated for the risk of IH, and the treatment rate was 80.8%. The area under the curve (AUC) of the subject receiver operating characteristic curve (ROC) of treatment decision making after referral by the HSS was 0.703 (95% CI: 0.634-0.772), and after referral by the IHReS was 0.892 (95% CI: 0.824-0.960).

LIMITATIONS: This was a single-center study.

CONCLUSIONS: For decisions regarding the treatment of IH patients, the IHReS has a higher efficiency and sensitivity than the HSS. However, the specificity of the IHReS is lower than that of the HSS.

PMID:36553295 | PMC:PMC9777170 | DOI:10.3390/children9121851

Comprehensive Analyses of Coagulation Parameters in Patients with Vascular Anomalies

Fetched: December 24th, 2022, 5:02am GMT by Friedrich G Kapp

Biomolecules. 2022 Dec 8;12(12):1840. doi: 10.3390/biom12121840.

ABSTRACT

BACKGROUND: Vascular anomalies comprise a diverse group of rare diseases with altered blood flow and are often associated with coagulation disorders. The most common example is a localized intravascular coagulopathy in venous malformations leading to elevated D-dimers. In severe cases, this may progress to a disseminated intravascular coagulopathy with subsequent consumption of fibrinogen and thrombocytes predisposing to serious bleeding. A separate coagulopathy is the Kasabach-Merritt phenomenon in kaposiform hemangioendothelioma characterized by platelet trapping leading to thrombocytopenia and eventually consumptive coagulopathy. Our previous work showed impaired von Willebrand factor and platelet aggregometry due to abnormal blood flow, i.e., in ventricular assist devices or extracorporeal membrane oxygenation. With altered blood flow also present in vascular anomalies, we hypothesized that, in particular, the von Willebrand factor parameters and the platelet function may be similarly impacted.

METHODS: We prospectively recruited 73 patients with different vascular anomaly entities and analyzed their coagulation parameters.

RESULTS: Acquired von Willebrand syndrome was observed in both of our patients with Kasabach-Merritt phenomenon. In six out of nine patients with complex lymphatic anomalies, both the vWF antigen and activity were upregulated. Platelet aggregometry was impaired in both patients with Kasabach-Merritt phenomenon and in seven out of eight patients with an arteriovenous malformation.

CONCLUSIONS: The analysis of coagulation parameters in our patients with vascular anomalies advanced our understanding of the underlying pathophysiologies of the observed coagulopathies. This may lead to new treatment options for the, in part, life-threatening bleeding risks in these patients in the future.

PMID:36551267 | PMC:PMC9775116 | DOI:10.3390/biom12121840

Permalink for 'Ultrasound and contrast-enhanced ultrasound imaging in hepatic epithelioid hemangioendothelioma: a retrospective study of 13 patients'

Ultrasound and contrast-enhanced ultrasound imaging in hepatic epithelioid hemangioendothelioma: a retrospective study of 13 patients

Fetched: December 23rd, 2022, 5:01am GMT by Ling Ren

Med Ultrason. 2022 Dec 21;24(4):414-420. doi: 10.11152/mu-3683.

ABSTRACT

AIM: The purpose of this study was to analyze the features of conventional ultrasound (US) and contrast-enhanced ultrasound (CEUS) in hepatic epithelioid hemangioendothelioma (HEHE).

MATERIAL AND METHODS: We retrospectively analyzed the US images (grayscale, color Doppler and CEUS) of the patients with histopathologically confirmed HEHE in our hospital from March 2015 to August 2021 who had underwent a US investigation.

RESULTS: A total of 13 patients were reported during the study period (seven men, aged from 23 to 62 years, with an average age of 40 years). The unifocal, multifocal, and diffuse lesions were 2, 9, and 2, respectively. Five patients (5/13) had liver involvement of both lobes, and eight (8/13) patients had only right lobe involvement. The maximum diameter of the lesions ranged from 1.9 to 7.0 cm. The grayscale US of HEHE mainly showed multiple hypoechoic lesions (n=9) near the capsule of the right lobe of the liver, with well-defined margins (n=7), accompanied by a hypoechoic halo (n=3) or capsule retraction (n=4) and calcification (n=8). Color Doppler US can detect blood flow in the lesion (n=8). CEUS was performed in five patients (5/13). The enhancement pattern of CEUS varied in the arterial phase, mainly including rim-like hyper-enhancement (n=2) and inhomogeneous hypo-enhancement (n=2), but was approximately the same in the portal venous phase and the late venous phase, both showing varying degrees of regression.

CONCLUSIONS: The grayscale US and CEUS seem to provide some reference value for diagnosing HEHE.

PMID:36546435 | DOI:10.11152/mu-3683

Laparoscopic resection of a rare diaphragmatic haemangioma: a case report

Fetched: December 23rd, 2022, 5:01am GMT by Xiqi Chen

J Int Med Res. 2022 Dec;50(12):3000605221140688. doi: 10.1177/03000605221140688.

ABSTRACT

This case report describes the laparoscopic resection of a rare diaphragmatic haemangioma. A 45-year-old male patient was diagnosed incidentally with a left subphrenic mass by computed tomography. Laparoscopic left subphrenic mass excision was performed under general anaesthesia. A phrenic haemangioma was confirmed by postoperative pathology. Tumours originating in the diaphragm are rare, with only approximately 200 cases reported in the past century. The diaphragmatic tumour was determined to be primary because intraoperative imaging showed that the tumour was relatively isolated and had no obvious relationship with the surrounding tissues and organs.

PMID:36545868 | PMC:PMC9793033 | DOI:10.1177/03000605221140688

Epithelioid Hemangioendothelioma Presenting as a Right Paratracheal Mass: A Case Report

Fetched: December 23rd, 2022, 5:01am GMT by Pa Hong

J Korean Soc Radiol. 2022 Nov;83(6):1373-1379. doi: 10.3348/jksr.2022.0057. Epub 2022 Sep 5.

ABSTRACT

Epithelioid hemangioendothelioma (EHE) is a low-grade malignant vascular neoplasm that can occur anywhere in the body. EHE has a low annual incidence (0.38/10⁶) and prevalence (< 1/10⁶), and primary mediastinal EHE is exceedingly rare. We report a case of EHE in a 53-year-old female which manifested as an incidentally discovered mass in the right paratracheal region. In this report, authors describe the pathological and radiological findings of primary mediastinal EHE invading the superior vena cava in the right paratracheal area.

PMID:36545423 | PMC:PMC9748454 | DOI:10.3348/jksr.2022.0057

Multidisciplinary management of a neonate with kaposiform hemangioendothelioma with extensive cranial fossa destruction

Fetched: December 23rd, 2022, 5:01am GMT by Amelia Aynaz Khoei

SAGE Open Med Case Rep. 2022 Dec 17;10:2050313X221142685. doi: 10.1177/2050313X221142685. eCollection 2022.

ABSTRACT

Kaposiform hemangioendothelioma is a rare, benign, locally destructive vascular tumor. Kasabach-Merritt phenomenon, a consumptive coagulopathy, is a life-threatening complication associated with kaposiform hemangioendothelioma. We describe a case of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in a neonate born with a large facial mass with deep extension toward the cranium and airway. The mass was not identified prenatally. The patient was a 37-week gestation age female neonate born via spontaneous vaginal delivery and noted to have a large left-sided facial mass that was not noted on the most recent prenatal ultrasound at 22 weeks gestation age. At birth, the patient was in respiratory distress and required continuous positive airway pressure support. Imaging revealed a large highly vascularized soft tissue mass adjacent to the airway with intracranial extension and bony destruction. Fine needle aspiration confirmed kaposiform hemangioendothelioma. On day of life 6, the patient was noted to have thrombocytopenia, elevated d-dimer, anemia, and hypofibrinogenemia, consistent with Kasabach-Merritt phenomenon, which resolved at day of life 12. Given the location and extent of the mass, medical therapy with single agent oral sirolimus was chosen over surgery. At 13-month follow-up, the infant is well on sirolimus therapy, and the mass has decreased in size, both clinically and on imaging. This case highlights the importance of prompt diagnosis and management of kaposiform hemangioendothelioma with extensive craniofacial and bony involvement with Kasabach-Merritt phenomenon with single oral therapy of sirolimus. Fibrinogen concentrate may be considered in the Kasabach-Merritt phenomenon refractory to cryoprecipitate.

PMID:36545011 | PMC:PMC9761797 | DOI:10.1177/2050313X221142685

Clinical factors associated with late seizure remission after failed epilepsy surgery

Fetched: December 22nd, 2022, 5:01am GMT by Lesley C Kaye

Epilepsy Behav. 2023 Jan;138:109055. doi: 10.1016/j.yebeh.2022.109055. Epub 2022 Dec 19.

ABSTRACT

BACKGROUND: Some patients who initially fail epilepsy surgery later become seizure-free, but it is not clear how the clinical characteristics of the patients or post-operative modifications of anti-seizure medication (ASM) regimens contribute to late seizure remission.

METHODS: We performed a retrospective chart review of patients undergoing epilepsy surgery at the University of Washington Regional Epilepsy Center between 2007 and 2017, including patients receiving neocortical resection, temporal lobectomy, and hippocampal laser interstitial therapy (LITT) ablation. We assessed seizure freedom, ASM changes, seizure frequency at the first and last follow-up, and type of lesion. Two-tailed Fisher's exact test and Mann-Whitney U test were used for statistical analyses.

RESULTS: Two hundred and fifteen patients undergoing epilepsy surgery between 2007 and 2017 had both first and last follow-ups. Ninety-eight (46%) were not seizure-free at the first follow-up (mean 1.1 years post-operative). By the last follow-up (mean 4.7 years post-operative), 20% of those not initially seizure-free had become so. Those who were seizure-free at the last visit had lower median seizures per month in the first post-operative year (0.21 versus 0.95 per month in those not seizure-free, p < 0.001). There was also a significantly higher proportion of patients with cavernomas who were seizure-free at the last visit (25% vs. 1% of those not seizure-free at the last visit; p = 0.001), but no other differences in clinical characteristics. Of the 98 patients who had seizures at the first follow-up, 63% underwent post-operative modification of their ASM regimens. The rate of late seizure freedom was similar for patients with or without ASM changes: 21% were seizure-free at the last visit with ASM changes and 19% without ASM changes. There were no significant differences in which ASMs were changed between those who became seizure-free and those who did not, but patients who were subjected to further medical management were less likely to have had mesial temporal sclerosis (MTS) than those who were not. A number of patients not initially seizure-free who underwent ASM changes achieved seizure freedom as long as 10 years post-surgery.

CONCLUSION: A substantial proportion of patients who initially fail epilepsy surgery will have late seizure remission. Those with cavernous hemangiomas were more likely to achieve late remission from seizures as were those with lower rates of seizures in the first year after surgery. The chances of achieving remission were similar in those with or without modification of their ASM regimens, but those with pre-operative MTS were more likely to achieve late seizure freedom without medication changes. At the individual level, patients may still achieve seizure freedom with ASM changes as long as ten years after the initial surgery.

PMID:36543042 | DOI:10.1016/j.yebeh.2022.109055

Infantile Haemangioma - Elaboration and Piloting a Specific Quality of Life Questionnaire

Fetched: December 22nd, 2022, 5:01am GMT by Anca-Maria Raicu

Maedica (Bucur). 2022 Sep;17(3):576-582. doi: 10.26574/maedica.2022.17.3.576.

ABSTRACT

Backround:Infantile haemangiomas are the most common benign tumours of the child with clinical manifestations in the first two years of life, which is an additional cause of parents' concerns. Objective:This study describes the first stage in elaborating a specific instrument to evaluate the quality of life of both patients with infantile haemangioma under two years of age and their parents, adapted to the reality of the Romanian context. Methods:Items were generated from a literature review - from both the current generic pediatricians' instruments and specific tools in dermatology for assessing quality of life and the existing consensus among experts - as well as from a qualitative analysis of parents' concerns. The instrument was piloted on a group of patients' relatives. Results:We have developed a 28-item specific infantile haemangioma quality of life questionnaire with four sub-scales to assess physical health, the social function of the child, parents' emotional health and the social function of parents. Demographic data and clinical features (meanings of symptoms and outcomes) that have an impact on the quality of life were obtained. Conclusion:It is important to be able to measure and compare the quality of life of both patients with infantile haemangioma and their parents for adapting the treatment to the specific needs of patients and their family. The effectiveness of new therapeutic options which are especially useful for infants with haemangiomas can be checked by using the questionnaire as a measure of patient-reported outcome. The questionnaire developed by us was well accepted by the patients' parents.

PMID:36540594 | PMC:PMC9720659 | DOI:10.26574/maedica.2022.17.3.576

Racemose Hemangioma of the Bronchial Artery with Pulmonary Artery Fistula

Fetched: December 21st, 2022, 5:01am GMT by Tsuyoshi Yachi

Kyobu Geka. 2022 Dec;75(13):1083-1087.

ABSTRACT

A 55-year-old woman was suspected of having hilar lymph node enlargement on a routine examination of the chest computed tomography( CT) scan at our hospital. On further examination, thoracic contrast CT and bronchial arteriography showed prominent dilation and meandering of the right bronchial artery with an aneurysm which formed a fistula to the pulmonary artery A7 at its distal end. Diagnosed as racemose hemangioma of bronchial artery with pulmonary artery fistula, we performed a surgical resection by open thoracotomy on the second day following bronchial artery embolization( BAE). No recurrence was observed in the five years of follow-up period after surgery. Therefore, surgical resection for asymptomatic racemose hemangioma of bronchial artery with bronchial artery-pulmonary artery fistula can be considered an effective method for the long-term prevention of bleeding.

PMID:36539223

Update December 2022

Fetched: December 21st, 2022, 5:01am GMT by Francine Blei

Lymphat Res Biol. 2022 Dec;20(6):671-694. doi: 10.1089/lrb.2022.29133.fb.

NO ABSTRACT

PMID:36537708 | DOI:10.1089/lrb.2022.29133.fb

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