Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1

Abstract

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Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: Case report and subject review

“…This occurs predominantly in patients who are 20 -50 years old. 1,13 From a report of five adult cases, the first symptoms were an increase in volume of a previously known subcutaneous neurofibroma, an exacerbation of pain, or the appearance of a motor deficit. 3 It is estimated that between 7.5% and 15% of patients with NF1 develop malignant peripheral nerve sheath tumors, most of which originate in areas containing plexiform neurofibromas.…”

“…The local recurrence rate is estimated to be 40%-65%, with metastasis occurring in 40%-68% of patients. 1,20 The 5-year survival rate for NF1 patients given best medical and surgical therapy is 21%, compared to 42% in sporadic (non-NF1) cases. 5 The association of peripheral neuropathy and NF1 was initially reported by Thomas et al 17 in a series of six patients with neurofibromatosis.…”

Would a Massive Intra-abdominal Malignant Peripheral Nerve Sheath Tumor with Growth into the Inguinal Canal and Scrotum Preclude Surgical Option? A Case Report and Review of Literature

“…One of the macroscopic criteria laid down for diagnosis is demonstration of nerve of origin at surgery [4]. However, this is often not possible in view of the fact that they arise from small and insignificant nerves [9]. Immunohistochemistry and electron microscopic studies are essential as confirmatory diagnostic methods.…”

Neurofibromatosis type 1 presenting with Horner's syndrome

“…The incidence of MPNST arising in NF1 is 4.6 and 0.001% in the general population. 5 Treatment consists of complete and wide surgical resection. Adjuvant irradiation seems to improve local control of the disease.…”