Cytogenetic study of malignant triton tumor: a case report.
ABSTRACT Malignant triton tumor (MTT) is a highly malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. Few cytogenetic studies of MTT have been reported using conventional cytogenetic analysis. Here, we report a comprehensive cytogenetic study of a case of MTT using G-banding, Spectral Karyotyping(), and fluorescence in situ hybridization (FISH) for specific regions. A complex hyperdiploid karyotype with multiple unbalanced translocations was observed: 48 approximately 55,XY,der(7)add(7)(p?)dup(7),der(7) t(7;20)(p22;?)ins(20;19),der(7)ins(8;7)(?;p22q36)t(3;8)t(8;20),-8,-8,r(8)dup(8), +der(8)r(8;22),-9,der(11)t(11;20)(p15;?)ins(20;19),der(12)t(8;12)(q21;p13),der(13) t(3;13)(q25;p11),-17,-19,der(19)t(17;19)(q11.2;q13.1),-20,-22,+4 approximately 7r[cp24]/46,XY. The 1995 International System for Human Cytogenetic Nomenclature was followed where possible. Note that breakpoints were frequently omitted where only SKY information was known for a small part of an involved chromosome. Our analysis revealed some breakpoints in common with those previously reported in MTT, MPNST, and rhabdomyosarcoma, namely 7p22, 7q36, 11p15, 12p13, 13p11.2, 17q11.2, and 19q13.1. FISH showed high increase of copy number for MYC and loss of a single copy for TP53.
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ABSTRACT: Malignant 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. We 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. Malignant 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.World Journal of Surgical Oncology 05/2012; 10:96. · 1.12 Impact Factor