<p>Malignant Triton Tumor in a Child: Case Report and Literature Review</p>

Abstract

Malignant triton tumor (MTT) is a rare and devastating malignant peripheral nerve-sheath tumor, which shows rapid growth and poor clinical outcomes. Here, we reported a 2-year-old girl who was diagnosed as MTT, an overview of the literature was conducted to discuss the clinical features and optimal treatment strategies of MTT. Methods: We conducted an analysis of 42 patients from the PubMed, Medline, Embase and Web of Science databases for relevant articles published between 1938 and 2018. Results: A 2-year-old girl died of tumor recurrence. Forty-two eligible cases of MTT in children (birth to 18 years; mean age, 8.3 years), the highest frequency of occurrence was in 12-16 years; and the male-to-female ratio was 1.7:1. Only 33 provided complete treatment details: 11 patients received treatment by surgery alone; 2 received both surgery and chemotherapy; 4 received both surgery and radiation therapy; 14 received surgery, chemotherapy, and radiation therapy; 1 case received chemotherapy and radiation therapy; and only 1 received supportive care. In the 33 cases, the average OS and 5-year OS probability were 23.9 months (range, 0.3-156 months) and 27.5 ± 4.3%. There were significant differences between radiation therapy and patient survival (p<0.05), postoperative chemotherapy/radiation therapy and patient prognosis (p<0.05). Conclusion: The clinical and histopathological features and therapeutic options for MTT are discussed in the light of published data. Further studies are needed to improve survival in children with this rare malignant tumor.

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%).…”

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

“…Malignant triton tumor is an extremely rare variant of the MPNSTs with rhabdomyosarcomatous differentiation, typically found on the head, neck, extremities, and trunk, and there are case reports that have shown this tumor growing in places such as the nasal cavity, paranasal sinus, lumbar spine, parapharyngeal space, rectum, mediastinum, palate, tibial nerve, esophagus, cerebellopontine angle, and acoustic nerve. It rarely affects the kidney, brain, buttock, retroperitoneum, thoracic spine, pelvis, genitourinary tract, and thigh [1] , [2] , [3] , [5] , [7] , [14] , [19] .…”

“…MTT is manifested as a mass that may or may not be painful. Regarding the estimated 5-year survival rate, studies have shown a variation between 5 and 20% for MTT [2] , [12] , [13] and 34–60% for MPNST [12] , [13] , and the morbidity rates of MTT are equal for both sexes [14] . However, the forms associated with NF1 are more commonly found in men and children, while sporadic forms have a higher incidence in women and older individuals [11] .…”

Intracranial Malignant Triton Tumor in Children: A Case Report

“…It commonly arises from a peripheral nerve in the neck, extremities or trunk, while it is rarely found in the cranial temporal and parietal fossa, viscera, mediastinum and retroperitoneum (1). Recently, Dias et al (cited in Zhao et al) (2) have reported an uncommon case of cerebral MTT in Brazil. In addition, most MTT cases are reported in adults.…”