Pheochromocytoma is a rare catecholamine-secreting tumour typically arising within the adrenal medulla. It may occur sporadically or be associated as part of a tumour syndrome including Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia (MEN) 2 and Neurofibromatosis Type 1. VHL is associated with multi-organ involvement of benign and malignant tumours characterized by the presence of retinal...

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 (...

Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or...

Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratent...

CONTEXT Von Hippel-Lindau disease is a genetic disorder characterized by neoplasms with multiple organ involvement, the pancreas being involved in about half of the cases. Conservative treatment is indicated because the disease is usually asymptomatic with long-term follow-up. CASE REPORT We herein present the case of a 64-year-old man with von Hippel-Lindau disease who presented with obstruc...

OBJECTIVES To describe the features, natural history, and management of an unusual manifestation of ocular von Hippel-Lindau disease in the form of fine vascular proliferation. METHODS Case series of 14 patients with definite or presumed von Hippel-Lindau disease. RESULTS Retinal vascular proliferation consisting of fine superficial vessels was found in 16 eyes of 14 patients with von Hippe...

Renal cell carcinoma is one of the most common malignant neoplasms genitourinary system. Along with smoking and hereditary syndromes associated mutations in Von Hippel-Lindau (VHL) gene, obesity main risk factors for development renal carcinoma. Emerging data indicate a causal relationship between In large study within framework Metabolic Syndrome Cancer project, clinical on blood pressure, bod...