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


CONCLUSIONS
MTT is exceedingly malignant requiring multimodality treatment. The cornerstone of management is radical surgical resection with clear margins. Nevertheless, the overall prognosis remains dismal.
Radiotherapy Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
E
E
Kamperis
Thoracic Surgery Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
N
N
Barbetakis
Thoracic Surgery Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
C
C
Asteriou
Thoracic Surgery Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
A
A
Kleontas
Pathology Department, Theagenio Cancer Hospital, Thessaloniki, Greece.
V
V
Christoforidou
Masaoka operation; malignant peripheral nerve sheath tumor; neurofibromatosis disease; triton tumor