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Malignant triton tumor of the left thoracic cavity: a case report

Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumors with rhabdomyoblastic differentiation. Although the condition may manifest sporadically, it typically affects adult patients with neurofibromatosis type 1. In this article, an extremely rare case of MTT with c...

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Detalles Bibliográficos
Autores principales: Ishikawa, Masashi, Chou, Hiroyuki, Imamura, Naoto, Shimazu, Yumeta, Ono, Kazuo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6735740/
https://www.ncbi.nlm.nih.gov/pubmed/31528328
http://dx.doi.org/10.1093/jscr/rjz246
Descripción
Sumario:Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumors with rhabdomyoblastic differentiation. Although the condition may manifest sporadically, it typically affects adult patients with neurofibromatosis type 1. In this article, an extremely rare case of MTT with chest wall origin, which expanded into the left thoracic cavity, is reported. A 64-year-old male was admitted to the institution with sudden shortness of breath. Radiological examination revealed a large mass with massive pleural effusion occupying the patient’s left hemithorax. A percutaneous needle biopsy was performed and the patient underwent subtotal tumor resection with left pleuropneumonectomy. Immunohistochemical study of postsurgical pathologic specimens confirmed the diagnosis of MTT. Despite extensive surgical removal, tumor recurrence was reported soon after resection, leading to patient’s death 20 days after surgery due to acute respiratory failure. Investigation of rare MTT cases is necessary for understanding this condition.