RESUMEN
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) occur sporadically, in patients with neurofibromatosis 1, and in patients with prior radiation therapy. The incidence of unrelated prior malignancies and family history of malignancy in patients with MPNSTs has not been previously described. METHODS: A retrospective search for cases of MPNSTs at our institution for the years 1994-2019 was performed. The electronic medical record was reviewed for documentation of personal and family history of malignancies. RESULTS: The study included 331 patients. Of patients, 301 had documentation of their personal history of prior unrelated malignancies; 70 (23.3%) of these patients had a personal history of an unrelated previous malignancy. Of patients, 285 had information in the chart regarding family history of cancer; 210 (73.7%) of these patients had a family history of malignancy. Of patients, 145 had sporadic MPNSTs, 118 had neurofibromatosis 1-associated MPNSTs, 31 had radiation-induced MPNSTs, and 37 were missing this information. Among the sporadic cases, 29 had a personal history of an unrelated prior malignancy, and 10 developed an unrelated malignancy following diagnosis of MPNST. A family history of malignancy was present in 109 patients. There was a trend toward longer time to recurrence, time to metastasis, and overall survival in patients with sporadic MPNSTs and negative personal and family histories compared with patients with positive personal or family histories or both. CONCLUSIONS: Patients with sporadic MPNSTs had a high incidence of personal and family history of malignancy. The genetics associated with sporadic MPNSTs include RAS and p53 mutations, which are found in multiple oncologic processes and tumor-forming syndromes. This suggests an underlying genetic predisposition to formation of malignancies, including MPNSTs.