Abstract
BackgroundMalignant triton tumors are relatively rare and highly aggressive tumors in which malignant schwannoma cells coexist with rhabdomyoblasts. Their occurrence in the retroperitoneum is uncommon and has been rarely reported.Case presentationWe present the case of a patient with a retroperitoneal malignant triton tumor. A 32-year-old male was referred with epigastric pain and an abdominal mass. Computed tomography revealed a huge soft tissue retroperitoneal mass that involved adjacent organs and vessels. Complete resection was undertaken. Pathological examination confirmed the presence of a malignant triton tumor. The patient died two and a half months after surgery, as a result of local recurrence.ConclusionMalignant triton tumors are uncommon sarcomas that are associated with a high incidence of developing local recurrence and distant metastases. Immunohistochemical staining showing nerve sheath differentiation with rhabdomyoblastic cells confirms the diagnosis. Complete excision of the tumor is the most effective treatment strategy for these retroperitoneal tumors.
Highlights
Malignant triton tumors are relatively rare and highly aggressive tumors in which malignant schwannoma cells coexist with rhabdomyoblasts
Malignant triton tumors are uncommon sarcomas that are associated with a high incidence of developing local recurrence and distant metastases
Daimaru et al [13] later suggested broadening the definition to include the following: one, tumors in patients without neurofibromatosis type 1 (NF-1) that are microscopically compatible with a malignant schwannoma and contain focal rhabdomyoblasts; and two, tumors consisting predominantly of rhabdomyoblastic differentiation with focal Schwann cell elements occurring within a nerve or in patients with NF-1
Summary
Malignant triton tumors are uncommon sarcomas that are associated with a high incidence of local recurrence and distant metastases. Immunohistochemical staining showing nerve sheath differentiation with rhabdomyoblastic cells confirms the diagnosis of MTT. Complete excision of the tumor is the most effective therapeutic strategy for retroperitoneal MTTs. Consent Written informed consent was obtained from the patient for publication of this case report and the accompanying images. Copies of the written consent form are available for review upon request. Competing interests The authors declare that they have no competing interests
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