All is true about von Hippel Lindau syndrome except:
**Core Concept**
Von Hippel-Lindau (VHL) syndrome is a rare genetic disorder characterized by the development of multiple benign and malignant tumors in various organs, including the central nervous system. The syndrome is caused by mutations in the VHL gene, which encodes a tumor suppressor protein that regulates cellular growth and differentiation.
**Why the Correct Answer is Right**
Tumors of Schwann cells, also known as schwannomas, are not typically associated with VHL syndrome. In fact, VHL syndrome is characterized by the development of hemangioblastomas, which are benign tumors that arise from blood vessels in the brain, spine, and retina. These hemangioblastomas are a hallmark feature of the syndrome and can occur in the craniospinal axis, including the cerebellum, spinal cord, and peripheral nerves.
**Why Each Wrong Option is Incorrect**
**Option A:** Hemangioblastomas seen in craniospinal axis is true, as these tumors are a common feature of VHL syndrome and can occur in the craniospinal axis.
**Option B:** Multiple tumors common is also true, as VHL syndrome is characterized by the development of multiple benign and malignant tumors in various organs.
**Option D:** Supratentorial lesions are uncommon is not entirely accurate, as while hemangioblastomas are more common in the posterior fossa, supratentorial lesions can also occur in VHL syndrome.
**Clinical Pearl / High-Yield Fact**
It's essential to remember that VHL syndrome is a multisystem disorder that can affect various organs, including the kidneys, pancreas, and adrenal glands, in addition to the central nervous system.
**β Correct Answer: C. Tumors of Schwann cells are common**