Introduction:

Malignant triton tumor is a rare neoplasm in which rhabdomyoblasts are present in a malignant tumor of the peripheral nerve sheath. This condition is characterized by its aggressiveness and bad prognosis. Location in the head and neck is infrequent. Immunohistochemical testing plays an important role in its diagnosis.

Objective:

Describe an unusually large malignant triton tumor.

Case presentation:

A case is presented of a female 16-year-old patient referred to the maxillofacial surgery service of the National Institute of Pediatrics in Mexico City with a diagnosis of neuroectodermal tumor of one year's evolution in the facial and cervical region. In clinical terms, the tumor was exophytic, multilobed, with extensive areas of necrosis, a surface in several shades of color and an approximate size of 18 x 10 x 12 cm. Biopsy and immunohistochemical testing confirmed the diagnosis of malignant triton tumor. The patient underwent surgery in which the lesion was totally excised with a safety margin. Evolution was clumsy, with a fatal outcome at six months of treatment.

Conclusions:

Triton tumor is an aggressive neoplasm whose early detection makes it possible for surgeons to provide an appropriate treatment.

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