Suprasellar haemangioblastoma. Report of two cases and review of the literature (original) (raw)

Contiguous haemangioblastomas of the brain and spine in a patient of Von Hippel-Lindau disease

BMJ case reports, 2013

Von Hippel-Lindau (VHL) disease is an inherited, autosomal-dominant syndrome caused by heterozygous germline mutations in the VHL gene, and predisposing to the development of benign and malignant tumours and cysts in multiple organ systems involving eyes, kidneys, pancreas, liver and central nervous system. The responsible tumour suppressor gene for VHL disease is in chromosome 3p25. We are presenting a case of a patient with both cerebellar as well as spinal haemangioblastoma in addition to polycystic pancreas. We operated on both the spinal and the cerebellar haemangioblastomas and the patient had made a very good recovery. We present this case for its rarity along with the literature review.

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

HAEMANGIOBLASTOMAS : AN OBSERVATIONAL STUDY OF TWO YEARS IN A TERTIARY CARE CENTRE.

International Journal of Advanced Research (IJAR), 2019

Background: 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. 9 cases of hemangioblastomas were examined with haematoxylin & eosin (H&E), reticulin stain and immunohistochemistry where ever needed. Their ages ranged from 12 years to 60 years. All of them were as presented as cystic nodules. The main histological differential diagnosis of Haemaniioblastoma is metastatic clear cell carcinoma. Additionally, because of the cystic mural features, pilocytic astrocytomas of the cerebellum must be separated from haemangioblastomas. Materials and Methods: The data for the present study was collected from the record section of the department of Pathology of our hospital. Histomorphological and immunohistochemical evaluation of all the cases of hemangioblastomas was done. Results: In the present study we had 9 cases with 2 of them being recurrent in nature. Age range was 12 to 60 yrs (median: 32 yrs) with Male to Female ratio of 2: 1 (M-6; F-3). Cerebellum was commonest location followed by brain stem. Conclusion: Hemangioblastomas can occur in throughout the neuroaxis. Cerebellum is the commonest site of occurrence for Haemangioblastoms.

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.

Molecular genetic analysis of a supratentorial haemangioblastoma in a non-Von Hippel Lindau patient

The Medical journal of Malaysia, 2005

We describe a rare tumor site in a 46 year old man who presented with a two week history of headache. Physical examination revealed bilateral papilloedema with no other localizing signs. Computed Tomographic Scan as well as Magnetic Resonance Imaging of the brain revealed a lesion with a dura tail located adjacent to the falx cerebri of the right frontal lobe. This lesion was not invading the inner table of the skull base. A tumor blush was seen on angiogram. There were no abnormalities on CT scan of the abdomen and fundoscopy was normal. Intraoperatively a vascular tumor not attached to the dura was noted and removed totally. Histopathological examination was typical of a hemangioblastoma. Analysis revealed no mutations of the VHL gene in 5 regions, exon 5-8 of the p53 gene, exon 1-2 of the p16 gene and exon 5,6 and 8 of the PTEN gene. This is the first case report of a supratentorial hemangioblastoma in a non-Von Hippel Lindau patient with genetic evidence.

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.

Cerebellar hemangioblastoma in a patient with von hippel-lindau disease : a case report

The Malaysian journal of medical sciences : MJMS, 2000

A 23 year-old Chinese woman presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus as a sequel to a mass effect from cerebellar haemangioblastoma. She underwent removal of the right cerebellar haemangioblastoma and ventriculo-peritoneal shunting. She also had bilateral retinal haemangioblastoma, left renal carcinoma, renal and pancreatic cysts without phaeochromocytoma. A left partial nephrectomy was performed for renal cell carcinoma followed by radiotherapy. She survived the initial episode only to succumb to another cerebellar haemorrhage 18 months later.

Supratentorial hemangioblastoma without von Hippel-Lindau syndrome in an adult

Romanian Neurosurgery, 2019

Hemangioblastomas (HBLs) are highly vascular and cystic benign neoplasms. They form very small part of intracranial tumours and are often localized in the posterior fossa. Although most of them are sporadic, a significant group is accompanied with von Hippel-Lindau (VHL) syndrome. This case report presents a 57-year-old woman treated with total resection using micro-surgical technique and was diagnosed as HBL based on histopathologic findings. Contrast-enhanced cranial MRI of the patient with the complaints of spasms in the right side of body showed a right paracentral mass that caused midline shift. In literature, the previously reported cases of supratentorial HBL unaccompanied with VHL syndrome were searched in PUBMED, compiled and presented. It should be borne in mind that rare HBLs manifesting with various neurological symptoms may occur in the supratentorial region, and may not accompany with VHL syndrome.