Malignant peripheral nerve sheath tumors of the buttock and lower extremity: a study of 43 cases

Article Abstract:

After reviewing 35 years of patient records, the authors identified 43 cases of malignant tumors of the peripheral nerve sheath occurring in the buttock or leg. The most common peripheral nerve sheath tumors are the neurofibroma, the schwannoma, and malignant peripheral nerve sheath tumors (PNST). The authors point out that many of the terms applied to similar tumors in the medical literature, such as malignant schwannoma, malignant neurilemmoma, and neurogenic sarcoma, are imprecise and used diversely and ambiguously by various investigators. Malignant PNST are extremely uncommon, with an incidence of 0.0001 percent in the general population. However, the condition is much more common among patients with von Recklinghausen's neurofibromatosis, occurring at a rate of 2 to 5 percent in this population. Among the 43 patients in the present study, 23 had neurofibromatosis. Overall, 44 percent of the cancers could be associated with an existing neurofibroma; this was true in 65 percent of the patients with von Recklinghausen's neurofibromatosis. The prognosis of malignant PNST is not good; the mortality rate in the present study was observed to be 63 percent. While some previous studies have found a poorer prognosis for malignant PNST patients with von Recklinghausen's neurofibromatosis, this observation could not be confirmed in the present study; the mortality rates were roughly comparable, at 65 percent for von Recklinghausen's patients and 60 percent for the others. In the present study, 88 percent of the patients with malignant PNST had a palpable mass. The authors suggest that any neurologic exam for symptoms in the leg should include palpation of the area of the affected nerve for signs of a tumor mass. (Consumer Summary produced by Reliance Medical Information, Inc.)

Complications and side effects, Neurofibromatosis

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Benign schwannoma of the obturator nerve: a case report

Article Abstract:

A case is reported of a 66-year-old women with schwannoma of the obturator nerve. Schwannoma is a benign tumor originating in Schwann cells, which surround most nerves. She was hospitalized for pelvic pain and ultrasound and CT scans showed a mass in her abdomen. Laparoscopic surgery revealed a tumor surrounding her obturator nerve, which innervates muscles in the hip, thigh and knee.

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