Malignant triton tumor of the kidney in a child: A case report

Abstract

Introduction Malignant triton tumor (MTT) is an extremely rare variant of the malignant peripheral nerve sheath tumors (MPNSTs) with rhabdomyosarcomatous differentiation, which was first described in 1932 by Mason. MTT affects, in most cases, patients under 35 years of age, and it is usually manifested as a mass that may or not be painful. However, the incidence in pediatric patients is atypical. This tumor presents an aggressive course and limited survival rate, and the prognosis is different between individuals with or without a concomitant diagnosis of neurofibromatosis type 1 (NF1). Currently, the recommended treatment is surgical resection, and adjuvant chemotherapy and radiotherapy, but its efficacy is not yet clear. Presentation of the case A 13-year-old female patient was referred to the pediatric oncology service due to the presence of an abdominal mass and weight loss, initially diagnosed with Wilms' tumor. After extensive investigation, surgical resection, and immunohistopathological evaluation, the diagnosis of malignant triton tumor was confirmed. The patient also underwent cycles of chemotherapy after resection, and is currently awaiting immunotherapy. Discussion and conclusion Malignant triton tumor is extremely rare and difficult to diagnose, especially in children or young people, age groups in which the incidence of the disease is even lower. This may be the reason it is rarely suspected, and it was a great challenge for the clinical care team. It is essential to consider and investigate this possibility of differential diagnosis, as patients diagnosed with this malignant tumor have a low survival rate and poor prognosis.

Malignant triton tumor of uterus: A case report and literature review

“…A range of cases presentation in the literature we reviewed describe the imaging manifestations (including CT and MRI) correlated with MTT: giant lump, heterogeneity, irregular margins, intratumoral hemorrhage, necrosis, lobulation, calcifications, heterogenous enhancement on postcontrast CT or MRI, hypointense/isointense T1WI and hyperintense T2WI signal on MRI, and diffusion restriction on DWI and ADC 1,8–32,39,40 . Zain et al 20 and Li et al 29 had reported similar conclusions, Li et al pointed out some characteristics common to MTTs and MPNSTs, including ill‐defined margin, intratumoral lobulation, peripheral edema, calcifications, and destruction of adjacent bone tissues.…”

“…We compiled data on reported cases of MTT in the recent 6 years (2018–2023, Table 1). 1,6–38 There were totally 37 cases with a mean age of 39.4 years (range 8 months old–75 years old), including 24 male and 13 female patients. Eleven cases originated in the abdomen (30%), 10 cases in the head and neck (27%), 8 cases in the thoracic cavity (21%), 6 cases in the limbs (16%), 1 case in the spine (3%), and 1 case in the buttocks (3%).…”

“…1 Whereas another case adopted the same treatment for a patient with rectum MTT, who passed away under palliative therapy and survived only 9 months. 18 Therefore, it is necessary to seek effective methods for MTT treatment.…”

“…A MTT case of gluteal underwent full resection with postoperative chemoradiotherapy and there were no recurrence symptoms for 4 years after treatment 1 . Whereas another case adopted the same treatment for a patient with rectum MTT, who passed away under palliative therapy and survived only 9 months 18 . Therefore, it is necessary to seek effective methods for MTT treatment.…”

Malignant triton tumor in the abdominal wall: A case report

“…A gluteal MTT case underwent full resection with postoperative chemoradiotherapy and there were no recurrence symptoms for 4 years after treatment[ 17 ]. In contrast, another case adopted the same treatment for a patient with rectal MTT, who passed away under palliative therapy and survived only 9 mo[ 4 ]. Therefore, it is necessary to seek effective methods for MTT treatment[ 6 ].…”