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Washington University Experience | NEOPLASMS - CRANIAL AND PARASPINAL NERVEs | Malignant Peripheral Nerve Sheath Tumor (MPNST) | 5A0 Case 5 History
Case 5 History ---- This was a 33 year old female with neurofibromatosis 1. She was in good health until the age of 6 years when she had problems "keeping up with the other children". She noticed her first subcutaneous nodules at that time. A neoplasm was apparently resected from her spine (no more detail). The patient apparently did well postoperatively and received a diagnosis of neurofibromatosis. During the past 3 years, the patient gradually lost strength in her lower limbs and loss of the ability to walk, required a Foley catheter, lacked sensation below the waist and had intermittent blurred vision in both eyes. ---- Neurologic exam showed countless subcutaneous nodules and café-au-lait spots covering her entire body. Both optic discs were pale. The impression on admission was severe neurofibromatosis with possible optic gliomas. A large abdominal mass was thought to represent a large neurofibroma which had undergone sarcomatous degeneration. A CT scan of the abdomen revealed a large complex mass in the region of the right lower quadrant with invasion of the 1umbar vertebra and right pelvis. There was also obstructive hydronephrosis of the right kidney. Lumbar spine films revealed bony destruction of L4-5. An abdominal aortograrn revealed hydronephrosis of the right kidney and a large right lower quadrant vascular abdominal mass. An inferior vena cavogram revealed bilateral common iliac vein obstruction with extensive thrombus formation. She was transferred to General Surgery for intravenous hyperalimentation and possible surgical therapy. The hyperalimentation procedure went well except for a low grade fever of less than 38.6°C which was felt to be a consequence of her severe sacral decubitus. An extensive tumor was found at the time of surgery, and only limited resection was possible. Postoperatively the patient exhibited a persistent sinus tachycardia. She continued to tolerate oral feeding very poorly. Her condition deteriorated further and she passed away
