Postradiation malignant triton tumor. A case report and review of the literature.

OBJECTIVE

To report the third case of postradiation malignant triton tumor and to review the literature on malignant triton tumor with regard to features of possible prognostic significance.

DATA SOURCES: Published articles were retrieved through MEDLINE, and additional articles were obtained through searches of the bibliographies.

STUDY SELECTION: Cases were selected on the basis of histologic, immunohistochemical, and ultrastructural studies of malignancies showing both neurogenic and myogenic differentiation. Only those cases with survival data were selected for analysis.

DATA EXTRACTION: The relationship between survival time and the possible prognostic variables of sex, presence or absence of von Recklinghausen's disease, age, and tumor location were evaluated by Cox regression analysis.

RESULTS

Kaplan-Meier analysis showed a 5-year specific survival of 26%. Tumor location showed a statistically significant association with survival time (P = .01).

CONCLUSIONS

This study suggests that malignant triton tumors occurring in the upper extremities, lower extremities, and head and neck have a better prognosis than tumors located in the retroperitoneum, buttock, or trunk. It is not clear if this variation is due to a difference in tumor grade, tumor stage, or resectability, or if it is a consequence of therapy.