E Kamperis1, N Barbetakis2, C Asteriou2, A Kleontas2, V Christoforidou3. 1. Radiotherapy Department, Theagenio Cancer Hospital, Thessaloniki, Greece. 2. Thoracic Surgery Department, Theagenio Cancer Hospital, Thessaloniki, Greece. 3. Pathology Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
Abstract
BACKGROUND: 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.
BACKGROUND: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.
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