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Case Reports in Oncological Medicine
Volume 2015, Article ID 405351, 6 pages
http://dx.doi.org/10.1155/2015/405351
Case Report

Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1

1Trinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USA
2Department of Internal Medicine, Trinitas Regional Medical Center, Elizabeth, NJ 67202, USA
3Touro College of Osteopathic Medicine, 230 W 125th Street No. 1, New York, NY 10027, USA
4Department of Hematology and Oncology, Trinitas Comprehensive Cancer Center, Elizabeth, NJ 07202, USA

Received 12 March 2015; Revised 16 May 2015; Accepted 18 May 2015

Academic Editor: Yoshihito Yokoyama

Copyright © 2015 Basnet Alina et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above.