Von Hippel Lindau is associated with all except,
Hemangioendotheliomas Von hippel lindau syndrome :? It is an autosomal dominant inherited neurocutaneous dysplasia in which the affected patients develop tumour within the :? - Cerebellar hernispheres12, - The retitle, - BrainstemQ and - Spinal corddeg Patients may also have cysts involving the pancreas, liver and kidneys and have a propensity to develop renal cell carcinoma of the kidney. The gene for VHL is a tumour suppressor gene and is located on chromosome 3 and encodes protein (pVHL) Systemic manifestation of VHL are as follows Retina Hemangioblastomaeand retinal angiomatosise Central nervous system Hemangioblastomadeg in cerebellum, cerebrum, medulla oblongata and spinal cord. Labyrinth Endolymphatic sac neoplasms Lung Cyst Hea Rhabdomyomae Kidney Hemangioblastomae, renal cell adenomas, renal cell Cue. Bladder Hemangioblastoma Epidydymisitestis Cysts of the epidydymis, clear cell papillary cystadenoma of the epidydymis. Broad ligament Papillate cystadenoma Adrenal gland Pheochromocytoma0 Pancreas Hemangioblastoma, cystse, cystadenoma, islet cell adenoma and islet cell carcinoma. Liver Cysts, adenoma, angioma Spleen Angioma and cysts Skin Naevue and cafe aulait spots Bone Cyst and hemangioma Polycythemia0 Approximately 10% of hemangioblastoma are associated with polyeythemie the tumour has been shown to be a source of erythropoietin.
Endo lymphatic sac tumors
Hemangioendotheliomas
Pheochromocytoma
Islet cell tumors