Abstract
Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that occur in adulthood and are located mainly on the trunk and lower limbs, with a high association with neurofibromatosis type 1 (NF1). Case report A 34-months-old female infant without NF1 with a palpable abdominal tumor is described. The tumor corresponded to a retroperitoneal MPNST. The diagnostic approach and management are presented, highlighting the complications and sequelae during the evolution of the patient. Conclusions Despite their low incidence, MPNSTs are important because of their aggressiveness, and should be considered upon the detection of a tumor located at paravertebral level or limbs, especially in patients with NF1. The cornerstone of the treatment lies in a complete surgical resection due to the high rate of recurrence and limited therapeutic response to radiotherapy and chemotherapy. This case presents clinical manifestations and complications that can be expected with these tumors and their harmful behavior. The absence of NF1 does not exclude the diagnosis.
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Schedule a callMore From: Boletín Médico Del Hospital Infantil de México (English Edition)
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