Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1
Joint Authors
Alina, Basnet
Sebastian, Jofre A.
Gerardo, Capo
Source
Case Reports in Oncological Medicine
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-05-31
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Alina, Basnet& Sebastian, Jofre A.& Gerardo, Capo. 2015. Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1. Case Reports in Oncological Medicine،Vol. 2015, no. 2015, pp.1-6.
https://search.emarefa.net/detail/BIM-1059274
Modern Language Association (MLA)
Alina, Basnet…[et al.]. Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1. Case Reports in Oncological Medicine No. 2015 (2015), pp.1-6.
https://search.emarefa.net/detail/BIM-1059274
American Medical Association (AMA)
Alina, Basnet& Sebastian, Jofre A.& Gerardo, Capo. Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1. Case Reports in Oncological Medicine. 2015. Vol. 2015, no. 2015, pp.1-6.
https://search.emarefa.net/detail/BIM-1059274
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1059274