Treatment of malignant triton tumor in zygomatic region.
ABSTRACT Malignant triton tumor (MTT) is extremely rare and supposed to be highly aggressive because of high propensity for local recurrence and metastasis. To date, only about 170 cases were reported in various body locations including trunks, maxillary sinus, neck, extremities, retroperitoneal, and so on. We present a case of MTT in the zygoma with good outcome. A 27-year-old male patient with progressive swelling and pain in the right zygoma was proved to have an MTT by biopsy. Radical resection accompanying postoperative radiotherapy was adopted, and then the soft and hard tissue defects were repaired by prosthesis. The patient recovered well and was satisfied with the facial contour. At 45-month follow-up, there was no recurrence or metastasis that occurred. According to literature review, one third of MTTs appeared in the head and neck regions and seem to have a better prognosis. Radical resection is the most important remedy, and adjuvant radiotherapy could be helpful. With early finding and effective treatments, satisfactory outcome could be achieved.
- SourceAvailable from: PubMed Central[Show abstract] [Hide abstract]
ABSTRACT: Malignant peripheral nerve sheath tumors (MPNST) constitute a rare variety of soft tissue sarcomas thought to originate from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT), a very rare, highly aggressive soft tissue tumor, is a subgroup of MPNST and is comprised of malignant Schwann cells coexisting with malignant rhabdomyoblasts. We herein report the case of a 24-year-old man who presented a subcutaneous mass in his right thigh. The mass was removed surgically in its entirety and radiation therapy was applied locally to prevent tumor regrowth. Nonetheless, the patient died 10 months after surgery from metastases to the lung and brain. He presented neither cafe-au-lait spots nor cutaneous neurofibromas. The histopathology showed a transition from a neurofibroma to an MTT, making this the second report of an MTT arising from a neurofibroma without neurofibromatosis type 1, an autosomal dominant disorder with which 50-70% of tumors reported in previous studies were associated. A histopathological examination using immunostaining with desmin confirmed this diagnosis. MTT has a poorer prognosis than MPNST and should therefore be regarded as a distinct clinical entity.Case Reports in Dermatology 09/2013; 5(3):373-8.