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Washington University Experience | NEOPLASMS - CRANIAL AND PARASPINAL NERVEs | Malignant Peripheral Nerve Sheath Tumor (MPNST) | 20A0 Case 20 History
Case 20 History ---- This patient was a 14 year old female admitted to SLCH for the third time. Shortly after birth she was noted to have multiple cafe au lait spots and the diagnosis of neurofibromatosis was established. Multiple cutaneous neurofibromas were noted from the age of 5 years. Her first symptoms were for back pain. Over the next 10 months she developed scoliosis with frequent falling and lower extremity weakness. Spine films at this time revealed scoliosis from T10 through L2 to the right with enlargement of the neural foramina to L3 on the left and a left paraspinal mass which depressed the left kidney and displaced the stomach anteriorly. Positive findings on neurologic examination included: bilateral lower extremity weakness, distal greater than proximal and left greater than right. She walked with a diplegic gait. She had a sensory level to pinprick at L3 bilaterally. Knee jerks were absent bilaterally. A combination lumbar and cervical myelogram was performed; from below a complete extradural block at the inferior margin of T11 was noted and from above complete block at the top of T12 was demonstrated. The patient underwent a total laminectomy from T12 through L2 with subtotal removal of epidural neurofibroma at the level of T12. The tumor extended out through the foramen of T12 and appeared to originate from the extradural segment of the Tl2 root. During the same hospitalization, the patient underwent an abdominal laparotomy with subtotal removal of a retroperitoneal neurofibroma on the left side of the kidney. Pathologic diagnosis of the retroperitoneal tumor, because of areas of hemorrhage and necrosis with numerous mitotic figures was neurofibrosarcoma (an old name for MPNST). The patient was then treated with a combination of radiation therapy and chemotherapy. She received 6000 rads total tumor dose partially administered to the retroperitoneal area and partially to the spinal cord. Concomitant with the radiotherapy, chemotherapy with vincristine and cytoxan was instituted. On discharge, the patient had much improved function neurologically. She was fitted with an appropriate brace and followed as an outpatient. Her abdominal mass regrew despite chemotherapy. Further surgical excision was not recommended. X-rays demonstrated the mass to be roughly 19 x 12 cm. with widespread extension into the chest, into the left kidney, into the stomach, also involving the lower 4 ribs on the left and the diaphragm, invading into the spinal canal and eroding lumbar vertebral bodies and finally crossing into the midline. The patient's last admission to SLCH was prompted by increasing shortness of breath and decreasing mental status. Her disease was end stage and she succumbed during the first 24 hours of hospitalization.
