A 20-year-old man is examined by a new family physician who discovers numerous pigmented patches and pedunculated skin tumors on his chest. Biopsy of a tumor discloses a benign neoplasm derived from Schwann cells. Neither the patient's father nor mother shows signs of this disease. The patient is at increased risk of developing which of the following malignant neoplasms?
One of the major complications of neurofibromatosis type 1 (NF1), occurring in 3% to 5% of patients, is the appearance of a neurofibrosarcoma in a neurofibroma. NF1 is also associated with an increased incidence of other neurogenic tumors, including meningioma, optic glioma, and pheochromocytoma. The other tumors listed are not associated with NF1.Diagnosis: Neurofibromatosis, type 1
Ganglioneuroma
Glioblastoma multiforme
Neurofibrosarcoma
Serous cystadenocarcinoma
Genetics