HAEMANGIOBLASTOMAS : AN OBSERVATIONAL STUDY OF TWO YEARS IN A TERTIARY CARE CENTRE. (original) (raw)
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Hemangioblastomas of the central nervous system
Journal of …, 1989
w" The findings of a 10-year study (1976 to 1986) conducted in southwest Germany on hemangioblastomas (HBL's) of the central nervous system (CNS) are presented. During that period, 47 HBL's were diagnosed and surgically removed in 44 patients, with a good postoperative survival rate and prognosis. The majority (83%) of these tumors were located in the cerebellum. By thorough clinical examination of the patients and careful evaluation of their family background, it was found that 23% of the HBL patients were afflicted with von Hippel-Lindau syndrome. In addition to the CNS tumors, 14 neoplastic or similar lesions were detected in other tissues. These included angiomatosis of the retinae, pheochromocytomas, pancreatic cysts, renal cysts, and renal carcinoma. The diagnosis of von Hippel-Lindau syndrome was thus established in seven families. The authors suggest the need for a screening program for patients with HBL of the CNS which is designed to confirm or exclude ocular or visceral lesions associated with von Hippel-Lindau syndrome. KEY WORDS 9 central nervous system neoplasm 9 hemangioblastoma 9 Lindan tumor 9 von Hippel-Lindau syndrome 9 familial disease
The histopathological features of cerebellar hemangioblastoma: Two case reports
Turkish Journal of Cancer
Summary Capillary hemangioblastomas are benign, highly vascular tumors of controversial origin limited almost exclusively to the central nervous system. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in the posterior fossa. Most commonly they develop in the cerebellum of male adults in the third through fifth decades of life. Although the majority of the cases arise sporadically, some hemangioblastomas are associated with the autosomal dominantly inherited disease, von Hippel-Lindau syndrome. In this article, 2 cases of hemangioblastoma, diagnosed in our department of pathology are presented and the histopathological and the clinical features are discussed. Keywords: Capillary hemangioblastoma, von Hippel-Lindau syndrome, posterior fossa
Serebellar Hemangioblastoma: Dört Olgu Sunumu ve Literatür Incelemesi
Cukurova Medical Journal, 2015
Hemangioblastoma (HB) is a benign, slow-growing, highly vascular tumour of not well defined histological origin. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in the posterior fossa. Hemangioblastomas can occur sporadically but in about 20% to 30% cases, it is associated with von Hippel-Lindau (VHL) disease. Four cases of cerebellar haemangioblastoma, not associated with von Hippel-Lindau disease (sporadic haemangioblastomas), were presented and reviewed the relevant literature.Four hemangioblastomas of the central nervous system were examined with haematoxylin & eosin (H&E), reticulin stain and with a panel of antibodies including CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, cytoceratin, epithelial membrane antigen (EMA), CD10. Of the 4 patients in this study 1 was male and 3 were female. Their ages ranged from 46 years to 60 years with a mean age of 54.75 years. All of them were as cystic nodules about 2-3 cm in diameter. In the histopathological examination, the tumors sections showed large and vacuolated stromal cells and numerous arborizing capillary-size blood vessels. Some tumors showed atypical nuclei. Vimentin was strongly positive both stromal cells and blood veessels in all tumors. In 4 cases of HB, some stromal cells were positive for NSE and CD99. Three tumors were positive for S-100 and CD56, two tumors were focally positive for glial fibrillary acidic protein (GFAP). CD34 immunostaining highlighted the arborizing and complex vascular network, whereas the tumor stromal cells were negative. The stromal cells were negative for epithelial markers such as cytokeratin, EMA and CD10. Ki-67 index was less than 1% of the tumor cells. Hemangioblastoma, a rare, benign tumors of uncertain histogenesis, is characterized histologically by the presence of vacuolated, lipid containing cells and a well developed, fine capillary network. The main histological differential diagnosis of HB is metastatic clear cell carcinoma. Additionally, because of the cystic mural features, pilocytic astrocytomas of the cerebellum must be separated from haemangioblastomas.
Cerebral and cerebellar haemangioblastoma occurring in the same patient
The Italian Journal of Neurological Sciences, 1983
A middle-aged man who had undergone two previous operations on a cerebellar haemangioblastoma was found at CT follow-up thirteen years later to have a double supra-and infra-tentorial haemangioblastoma. The tumors were confirmed by vertebral angiography and removed by two-stage surgery. The importance of careful CT follow-up to detect the recurrence of multifocal haemangioblastoma is clear.
World Neurosurgery, 2018
BACKGROUND: Posterior cranial fossa (PCF) hemangioblastomas are benign, highly vascularized, and well-differentiated tumors with well-described histopathologic features. Although relatively rare, this tumor is the most prevalent primary tumor of the cerebellum in adults.-OBJECTIVE: Because the demographics of patients with such a tumor (as well as the clinical, morphologic, pathologic, surgical features, and outcomes) are not fully understood, we systematized characteristic patient and tumor features.-METHODS: We undertook a systematic review of the English-language literature in PubMed for PCF hemangioblastomas in adults published in the past 31 years. We analyzed geographic distribution and year of publication of articles; demographic data of patients; presenting symptoms and clinical signs; tumor location and morphology; histopathologic features, extent of tumor resection, perioperative blood loss, and postoperative complications; length of hospital stay; and outcomes.-RESULTS: We reviewed 207 articles describing 1759 infratentorial hemangioblastomas in a cohort of 1515 adult patients. We found female predominance in patients with Von Hippel-Lindau disease (VHLD) compared with male predominance in the general patient group. Symptoms of intracranial hypertension were more common in the VHLD group compared with the general group of patients. The cerebellar location was more common in the VHLD group and solid (parenchymatous) tumor was the most common type. Most patients underwent total resection but rate of resection did not differ between the general and VHLD groups. Most patients had a favorable outcome.-CONCLUSIONS: The literature of adult PCF hemangioblastomas is limited and general surgical experience with such tumors is scarce because of their rarity. Rates of postoperative complications and mortality remain higher than expected. However, prognosis and surgical outcomes are generally favorable. Nevertheless, surgery of adult PCF hemangioblastomas is a demanding and challenging task.
Management Difficulties of Cerebeller Haemangioblastoma: A Case Report and Literature Review
Journal of Medicine, 2010
Introduction Cushing and Bailey used the term 'Haemangioblastomas' to describe tumors arising from the endothelial cells of the central nervous system. 1 Haemangioblastomas are solid or cystic benign vascular tumors that may arise anywhere in the body including central nervous system. 2 Though histologically benign, their development is often unfavorable due to high frequency of recurrence and multicentricity especially when occurring in a familial setup i.e., Von Hippel Lindau (VHL) syndrome. 3 They account for 1.5-2.5% of all intracranial and 7-12% of posterior fossa tumors. Inspite of some advancement of endovascular and radiosurgical therapy in the treatment of these tumours, they are rarely effective in isolated form; Microsurgical removal CNS haemangioblastomas is the treatment of choice, though it is often complicated and difficult, because of tumor hypervascularity and location. In addition, though surgical mortality ,morbidity rates have been reduced, but still there may be mortality and morbidity, even with the advantages of modern microsurgical techniques. 4,5,6,7 Here, we report on a male patient with recurrent haemangioblastoma in the cerebellum with Von Hippel Lindau disease who underwent partial microsurgical excision
Cerebellar haemangioblastoma: a rare entity
BMJ case reports, 2011
A 45-year-old female presented with headache, nausea, vomiting and ataxia of 4-month duration. CT head showed a posterior fossa tumour which was diagnosed as a case of haemangioblastoma on microscopic examination.
2019
Objectives Hemangioblastoma refers to a benign vascular neoplasm that comprises stromal and capillary cells. Based on the classification of nervous system tumors proposed by WHO, hemangioblastomas are classified as Grade I meningeal tumors of uncertain origin. These tumors are found almost exclusively in the central nervous system (CNS) and account for 0.9% to 2.1% of all primary CNS tumors. Materials & Methods In this descriptive retrospective study, the archives of pathology reports were reviewed in the Department of Pathology of Shohada-e-Tajrish Hospital, Tehran, Iran and patients with definite diagnosis of hemangioblastoma made through histopathological examinations during 2004-2014 were identified. Age, gender and the location of tumor were extracted from the medical records and entered into SPSS statistical software v.22 for analysis. Results Thirty patients including 16 males (53.3%) and 14 females (46.7%) were identified. The mean age of the patients was calculated to be 41...
Journal of Kidney Cancer and Vhl, 2014
Central nervous system (CNS) hemangioblastoma is the most common manifestation of von Hippel-Lindau (VHL) disease. It is found in 70-80% of VHL patients. Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS hemangioblastomas are typically located in the posterior fossa and the spinal cord. VHL patients usually develop a CNS hemangioblastoma at an early age. Therefore, they require a special routine for diagnosis, treatment and follow-up. The surgical management of symptomatic tumors depend on many factors such as symptom, location, multiplicity, and progression of the tumor. The management of asymptomatic tumors in VHL patients are controversial since CNS hemangioblastomas grow with intermittent quiescent and rapid-growth phases. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Radiotherapy should be reserved for inoperable tumors. Because of complexities of VHL, a better understanding of the pathological and clinical features of hemangioblastoma in VHL is essential for its proper management. Copyright: The Authors.