A malignant Triton tumor (MTT) is a rare and aggressive soft tissue sarcoma associated with poor prognosis and with no established treatment protocol.

A 38-year-old man presented with severe right thigh pain and limited hip motion. Since childhood, the patient had a mass on their thigh, accompanied by café-au-lait spots. Upon admission, a biopsy confirmed MTT that had metastasized to the lungs. Preoperative radiation therapy was administered to reduce the tumor size; however, the tumor did not shrink. Extensive resection was not feasible because of the tumor size and location, prompting a decision to perform volume reduction surgery aimed at alleviating the patient's pain and improving mobility. Although the surgery provided temporary relief from the symptoms, the patient died two weeks later.

MTT requires a multidisciplinary approach that includes surgery, chemotherapy, and radiation therapy; however, in advanced cases such as this one, palliative measures may be more appropriate. This case underscores the challenges in managing MTT and highlights the potential role of volume reduction surgery in improving the quality of life of patients with significant symptoms. Despite the poor prognosis, symptom palliation during the two weeks leading up to the patient's death was significant, illustrating the importance of addressing pain and mobility issues while considering overall treatment strategies in such complex cases. These findings emphasize the need for further research on effective management options for MTT to improve patient outcomes.

Malignant triton tumors (MTTs) comprise a subgroup of malignant peripheral nerve sheath tumors (MPNSTs) that exhibits rhabdomyosarcomatous differentiation and follow an aggressive course. MTTs are primarily located along peripheral nerves. Cases of MTTs in the abdominal wall have not been reported. MTT has a poorer prognosis than classic MPNSTs, and accurate diagnosis necessitates a keen understanding of the clinical history and knowledge of its differential diagnosis intricacies. Treatment for MTTs mirrors that for MPNSTs and is predominantly surgical.

A 49-year-old woman presented with a subcutaneous mass in her lower abdominal wall and a pre-existing surgical scar that had grown slowly over 3-4 months before the consultation. She had previously undergone radical hysterectomy and concurrent chemo-radiotherapy for cervical cancer approximately 5 years prior to the consultation. Abdominal computed tomography (CT) showed a 1.3 cm midline mass in the lower abdomen with infiltration into the rectus abdominis muscle. There was no sign of metastasis (T1N0M0). An incisional biopsy identified sporadic MTT of the lower abdomen. A comprehensive surgical excision with a 3 cm margin inclusive of the peritoneum was executed. Subsequently, the general surgeon utilized an approach akin to the open peritoneal onlay mesh technique. The patient underwent additional treatment with an excision shaped as a mini-abdominoplasty for the skin defect. No complications arose, and annual follow-up CTs did not show signs of recurrence or metastasis.

An abdominal MTT was efficaciously treated with extensive excision and abdominal wall reconstruction, eliminating the need for postoperative radiotherapy.

Malignant triton tumor (MTT) is a highly aggressive malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. There are few reports that MTT occurred in urogenital system. In the present study, we report the first MTT occurring in the uterus. A 57-year-old woman came to the emergency department due to persistent vaginal bleeding for 2 months. The gynecological palpation found that a club-shaped excrescence existed in the vagina about 7 cm × 3 cm × 3 cm. The mass located in the lower segment of the uterus and the cervix was confirmed by gynecological vaginal ultrasound and magnetic resonance imaging, which was preliminarily diagnosed as cervical carcinoma. After neoplasm punch biopsy, the pathological diagnosis was malignant triton tumor. The patient finally lost follow-up. This is the first report about MTT in the uterus and suggests that pathological biopsy combined with imaging examination is necessary for the diagnosis of rarely MTT.