Primary cutaneous rhabdomyosarcoma: a clinicopathologic review of 11 cases
Trent B. Marburger
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
These authors contributed equally to this work and are considered as co-first authors.
Search for more papers by this authorJerad M. Gardner
Departments of Pathology and Dermatology, Emory University Hospital, Atlanta, GA, USA
These authors contributed equally to this work and are considered as co-first authors.
Search for more papers by this authorVictor G. Prieto
Departments of Pathology and Dermatology, MD Anderson Cancer Center, Houston, TX, USA
Search for more papers by this authorCorresponding Author
Steven D. Billings
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
Dr. Steven D. Billings, MD
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
Tel: +216-444-2826
Fax: +216-445-6967
e-mail: [email protected]
Search for more papers by this authorTrent B. Marburger
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
These authors contributed equally to this work and are considered as co-first authors.
Search for more papers by this authorJerad M. Gardner
Departments of Pathology and Dermatology, Emory University Hospital, Atlanta, GA, USA
These authors contributed equally to this work and are considered as co-first authors.
Search for more papers by this authorVictor G. Prieto
Departments of Pathology and Dermatology, MD Anderson Cancer Center, Houston, TX, USA
Search for more papers by this authorCorresponding Author
Steven D. Billings
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
Dr. Steven D. Billings, MD
Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA
Tel: +216-444-2826
Fax: +216-445-6967
e-mail: [email protected]
Search for more papers by this authorAbstract
Background
Rhabdomyosarcoma is a malignant mesenchymal tumor with skeletal muscle differentiation. Primary cutaneous rhabdomyosarcoma is rare. We report a series of 11 cases of primary cutaneous rhabdomyosarcoma.
Methods
Cases diagnosed as rhabdomyosarcoma arising in the dermis/subcutis with no identified primary tumor elsewhere were retrospectively reviewed. Follow-up was obtained.
Results
The tumors occurred in five children and six adults. The adult subset consisted of pleomorphic, epithelioid and not otherwise specified (NOS) subtypes while the pediatric subset showed alveolar and embryonal subtypes. All cases showed immunohistochemical staining consistent with the diagnosis of rhabdomyosarcoma. Three adult cases showed immunoreactivity for cytokeratins (one pleomorphic, one epithelioid and one NOS.
Conclusions
Primary cutaneous rhabdomyosarcoma shows a bimodal age distribution and male predominance, correlating with rhabdomyosarcoma in deep soft tissue. Follow-up, available on all patients, showed aggressive behavior in both children and adults. Primary cutaneous rhabdomyosarcoma should be considered in the differential diagnosis of tumors with abundant eosinophilic cytoplasm and those with “small round blue cell” morphology. Desmin, myogenin and MYOD1 are a trio of markers with high sensitivity and specificity for primary cutaneous rhabdomyosarcoma. Cytokeratin immunoreactivity in primary cutaneous rhabdomyosarcoma represents a potential diagnostic pitfall in the differential diagnosis with sarcomatoid carcinoma.
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