25 yo male with 5 cm slowly enlarging back mass.
02/16/2011
25 yo male with 5 cm slowly enlarging back mass.
Immunostains:
Desmin - focal "dot-like" positivity
Myogenin - negative
MyoD1 - nuclear positive
Case courtesy of Dr. Mark Edgar, Bone and Soft Tissue Pathology, Emory University, Atlanta, Georgia, USA.
Condition:
5 cm slowly enlarging back mass.
Diagnosis:
Sclerosing Rhabdomyosarcoma
DDx:
Differential includes:
*Sclerosing Rhabdomyosarcoma
*Sclerosing Osteosarcoma
*Extraskeletal Myxoid Chondrosarcoma
*Mesenchymal Chondrosarcoma
*Sclerosing Epithelioid Fibrosarcoma
*Angiosarcoma
Immunostains are most helpful in arriving at the correct diagnosis. Note that desmin is typically weak and focal, often with dot-like staining, in these tumors, and that myogenin may be weak or negative. myoD1 shows diffuse nuclear positivity. Although in general, myogenin is a better stain than myoD1 (due to high background cytoplasmic staining in myoD1), in this tumor, myoD1 is important.
Discussion:
These tumors were only recently described, and, thus, few cases have been reported. They are composed of primitive round cells arranged in cords, nests, and microalveoli in a sclerotic background. Sclerosing rhabdomyosarcoma does not appear to be related to alveolar rhabdomyosarcoma. Although some have suggested a possible relationship to embryonal rhabdomyosarcoma, that has not yet been confirmed.
References:
Goldblum and Weiss. Soft Tissue Tumors. 5th Ed.

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