Sclerosing Rhabdomyosarcoma

Jian Wang, Xiaoyu Tu, Weiqi Sheng
2008 American Journal of Clinical Pathology  
A b s t r a c t We report 5 cases of sclerosing rhabdomyosarcoma. The patients included 4 adults and 1 adolescent. In the 5 cases, 3 tumors occurred in the head and neck region and 2 in an extremity. Histologically, all 5 tumors were characterized by the presence of abundant extracellular hyaline matrix, mimicking osteoid or chondroid tissue. They were composed mostly of primitive small round cells that displayed diverse growth patterns. In 2 cases, focal areas suggestive of spindle cell
more » ... spindle cell rhabdomyosarcoma were present. Typical features of embryonal or alveolar rhabdomyosarcoma were not noted; however, rare strap rhabdomyoblasts were identified in 1 case. Immunohistochemically, all 5 cases showed diffuse immunoreactivity for MyoD1, with varied expression of myogenin, desmin, muscle-specific antigen, and α-smooth muscle actin. All patients underwent surgery, combined with adjuvant radiation therapy or chemotherapy. Of 4 cases with follow-up, recurrence was found in 2. Sclerosing rhabdomyosarcoma represents a special variant of rhabdomyosarcoma and is possibly related to embryonal rhabdomyosarcoma.
doi:10.1309/ep3frry6gmp555qc pmid:18285263 fatcat:5r3k7fizkrfdfawj2x7xqnkjvy