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Malignant triton tumor of the chest wall invading the lung. A case report and literature review.

AbstractBACKGROUND:
Malignant triton tumor (MTT) is an histological deviation of malignant peripheral nerve sheath tumor with additional rhabdomyosarcomatous elements. It is very rare, profoundly aggressive, with a tendency to recur locally and metastasize early. If manifests itself more often in individuals with neurofibromatosis type I (NF-1) disease but also sporadically or post radiotherapy. Description of case: A 57-year-old male was admitted with a history of malignant triton tumor of the chest wall. Despite prior aggressive locoregional treatment including wide excision and adjuvant consolidating radiotherapy, the tumor recurred. The patient underwent a new operation and systemic chemotherapy, but expired a few months later due to disease progression.
CONCLUSION:
MTT is exceedingly malignant requiring multimodality treatment. The cornerstone of management is radical surgical resection with clear margins. Nevertheless, the overall prognosis remains dismal.
AuthorsE Kamperis, N Barbetakis, C Asteriou, A Kleontas, V Christoforidou
JournalHippokratia (Hippokratia) Vol. 17 Issue 3 Pg. 277-80 (Jul 2013) ISSN: 1108-4189 [Print] Greece
PMID24470743 (Publication Type: Case Reports)

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