Primary epididymis malignant triton tumor: case report and review of the literature

Abstract

Background Malignant triton tumor (MTT) is a rare and histological complexity characterized by a mixture of peripheral nerve sheath tumors and with rhabdomyoblastic differentiation. It follows a particularly malignant course. Case presentationIn the present study, we report the first MTT of epididymis. The patient is a 22-year-old male presented with swelling in the left scrotum over a 2-month period. He did not have the history or symptoms of neurofibromatosis type 1. A mass measured about 3 cm × 4 cm was found in the left epididymis by ultrasound and CT scan. It was diagnosed as epididymis tumor and underwent exploration; intraoperative frozen section was diagnosed malignant tumor and treated with radical orchidoepididymectomy. The pathological report was malignant triton tumor. Despite taken high-dose radiation therapy and followed by chemotherapy for four cycles, he was died of progressive disease with multiple metastases 26 months after surgery. The clinic pathologic characteristics and optimal treatment strategy are reviewed.

Computed Tomography Imaging Features of Malignant “Triton” Tumor for Its Clinical Diagnosis: Report of Two Cases

“…Generally, a de nitive diagnosis for an MTT is based upon observations from histological staining and true positives for S-100 protein, Myo-D1 and Desmin in post-surgical IHC assays. [2][3][8][9] Clinical histories of most patients with MTT cases typically range between several months to more than ten years in all age groups. [15][16] In the two cases exempli ed in this present work, the patient ages were 37 and 41 years, respectively.…”

“…[4][5] At present, treatment scenarios for MTTs include radical surgical resection, adjuvant chemotherapy and radiation, with the latter consideration as the standard protocol for MPNST or soft tissue sarcoma. [6][7] In respect of tumor location, MTTs primarily arise in head, neck and trunk regions, but also infrequently observed in lung, mediastinum, abdomen and prostate tissues, [8][9] which invariably culminates with the untimely demise of the infected individual.…”

“…11 Subsequent immunohistochemical (IHC) analyses for the skeletal muscle-speci c markers myogenin or Myo-D1 are then used as con rmation of malignancy. 9 Unfortunately, assays for the presence of rhabdomyoblasts with MTTs are prone to error being reliant upon objective separation of MTTs with sample that contain signi cant numbers of Schwann cell that negatively stain for desmin and myogenin and/or Myo-D1. 2 Such investigations are further impeded as IHC assessments are performed post-surgery, although pathological evaluation still provides a reliable tool for neurogenic tumor classi cation.…”

Granulocytic/myeloid sarcoma with trisomy 21 presented as an epididymal tumor: A case report and review of the literature

“…Cystadenomas, papillary tumors, and adenomatoid tumors are the most common; however, preoperative diagnosis is difficult. 14 In immunohistochemistry, some key markers, such as MPO, c-kit, and CD68PG-M1, have been used in the diagnosis of MS. 2 In this case, we were able to diagnose MS based on the presence of other markers for immature myeloid cells despite the few or no markers of CD68 and MPO in the epididymal specimen. Although most markers for the recurrent tumor had similar characteristics to the primary tumor, markers of MPO and CD68 were positive in the recurrent tumor specimens.…”

Malignant Cutaneous Peripheral Nerve Sheath Tumour with Rhabdomyosarcomatous Differentiation (Triton Tumour) in a Domestic Cat

“…Of the 59 PNST reported in cats, (Schulman et al, 2009), in none was divergent growth identified, while 16/59 were viewed as malignant. In man, malignant triton tumour has a poor prognosis and histopathological examination is not able to predict outcome, therefore cases are followed for as long as possible after treatment (Gao et al, 2015). Malignant triton tumours have a poorer prognosis than MPNSTs, which has been linked to a high frequency of grade III tumours in this variant (Brooks et al, 1985).…”