The case to be described is unusual in presenting most of the changes of Lindau's disease without any cerebellar tumour. In addition there is evidence of a hereditary tendency to the disease, as four relations of the patient suffered from progressive visual defect, and one other had an intracranial tumour. The patient herself had an hkmangioblastoma in one retina and two in the other, an hemang...

We describe five patients diagnosed with von Hippel-Lindau disease who complained of abdominal distension, pain and discomfort for a long time. All patients underwent ultrasonography, CT scan and MRI, which showed huge pancreas filled with multiple cysts. Additionally, extrapancreatic findings such as cerebellar hemangioblastoma (3 patients), retinal hemangioblastoma (2), renal cell carcinoma (...

Von Hippel-Lindau (VHL) syndrome is a rare, autosomal dominantly inherited multicancer syndrome. Disease is predisposed by hemangioblastoma, renal cell carcinoma, pheochromocytoma, pancreatic islet cell and cystic tumors or epididymis tumors under the mutation or deletion at the tumor suppressor gene. Here we report a 42-year-old woman who had been operated for cerebellar hemangioblastoma and r...

BACKGROUND Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited, multisystemic tumor syndrome caused by mutations in the VHL gene. To date, more than 1,000 germline and somatic mutations of the VHL gene have been reported. We present a novel mutation in the VHL tumor suppressor gene that presented with gestational diabetes mellitus. METHODS A 30-year-old woman presented with g...

Sashank Prasad, MD Nicholas J. Volpe, MD Madhura A. Tamhankar, MD SECTION 1 A 36-year-old man with Von Hippel-Lindau syndrome presented with binocular vertical diplopia following suboccipital craniotomy for resection of a cerebellar hemangioblastoma. His diplopia was worse in left gaze. He was effectively treated with a 6-diopter base-down prism in the right eye. With-out spontaneous improvemen...

Infrequently, intracranial neoplasms metastasize to extracranial sites. In 1963, Glasauer and Yuan reviewed the 88 reported cases of metastatic intracranial tumours of which approximately two-fifths were meningiomas. This report concerns an angioblastic meningioma with a large hepatic metastasis. Cushing's original classification of angioblastic meningiomas and the differential diagnosis betwee...

INTRODUCTION Cystic meningiomas are rare variants of meningiomas; they can pose a radiological diagnostic dilemma. CASE PRESENTATION We present a rare case of a 30-year-old Chinese woman with a histopathological diagnosis of infratentorial cystic meningioma (World Health Organization Grade 1) in which the features in imaging modalities were suggestive of a hemangioblastoma. Intraoperatively, ...

A 29-year-old male patient with von Hippel-Lindau disease who had an operation for cerebellar hemangioblastoma six years ago is presented. The patient had a four-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by MR imaging, was almost completely cystic in appearance. Pathological examination after...

A case of Lindau's disease is presented where renal carcinoma was detected 2.5 years after surgical removal of a cerebellar haemangioblastoma. This patient had no retinal lesions. The importance of screening for renal carcinoma in patients showing one or more manifestations of the disease and screening relatives is emphasized.

We present a magnetic resonance imaging of a 38 old woman, admitted with clinical symptoms of expansive cerebellar lesion. No mural nodule and no enhancement after gadolinium injection were noted. She was operated upon and an anatomico-pathological diagnosis of a simple cyst was made. Cysts of the posterior fossa are common in the literature, but the simple cyst is rare. Considered in the diffe...