Wednesday, August 5, 2009

Neoplasms Associated with the Neurocutaneous Syndromes

Neurofibromatosis 1
  • Low-grade optic nerve pilocytic glioma
  • Increased risk of multicentric gliomas
  • Neurofibrosarcomas (malignant degeneration of neurofibromas).
  • Leukemia, lymphoma
  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Melanoma
  • Wilms tumor
Neurofibromatosis 2: MISME (Multiple Inherited Schwannomas, Meningioma, and Ependymoma)
  • Schwannomas: CN 8 > CN V > spinal canal
  • Spinal ependymomas
  • Meningiomas
von Hippel-Lindau
  • Hemangioblastomas: Cerebellum > Retina > Brainstem
  • Renal cell carcinoma (more commonly bilateral and smaller than sporadic form)
  • Pheochromocytoma
Sturge-Weber
  • Angiomas: leptomeninges and skin of the face
Tuberous Sclerosis
  • Angiomyolipomas
  • Subependymal nodules may degenerate to subependymal giant-cell astrocytomas (SEGA)
  • Cardiac rhabdomyomas

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