Background: Dermatofibrosarcoma protuberans (DFSP) is an unusual soft-tissue tumor with a propensity for subclinical extension and local recurrence. Surgical excision, even with tissue-sparing techniques, may cause significant deformity or disability because of the infiltrative nature of DFSP. In this study, we evaluate retrospective data obtained from 4 patients with locally advanced or recurrent DFSP who received neoadjuvant imatinib mesylate therapy before undergoing Mohs micrographic surgery.Observations: Patients treated with neoadjuvant imatinib therapy had an average tumor size reduction of 36.9%. This clinical response was paralleled by histopathologic changes, including decreased cellularity in 100% of the total area as well as significant hyalinization. Imatinib therapy for DFSP before Mohs micrographic surgery was associated with 100% local control at a maximum follow-up of 4 years.Conclusions: Neoadjuvant imatinib therapy is a welltolerated, novel approach to DFSP that reduces tumor burden and facilitates resection. Larger prospective studies are needed to confirm and expand on these results.
Malignant melanoma may exhibit morphologic characteristics of nonmelanocytic cell or tissue components, a phenomenon termed divergent differentiation. Melanoma with rhabdomyosarcomatous differentiation is rare, with 6 definite cases in adults reported in the literature. The authors describe a 75-year-old man with a cutaneous lesion of the right ear initially diagnosed as malignant melanoma. Three months later, biopsy of a right cervical lymph node showed changes suggestive of rhabdomyosarcoma. Reexamination of the initial skin biopsy with muscle markers confirmed a diagnosis of malignant melanoma with rhabdomyosarcomatous differentiation. This case serves to highlight the diagnostic challenges associated with this rare subtype of melanoma.
We describe two children with vascular neoplasms that resembled Kaposi's sarcoma in places. Both presented with intraabdominal masses and severe thrombocytopenia. At autopsy the tumors extensively infiltrated the peritoneum and retroperitoneum and surrounded or invaded numerous organs including the kidneys, pancreas, adrenal glands, gastrointestinal tract, mesentery, and lymph nodes in both cases, and spleen or bone marrow in one case each. The neoplasms were histologically identical and displayed two patterns: dilated vascular spaces (angiomatous areas) lined by flat endothelial-like cells and areas of spindle cells forming slitlike vascular spaces similar to those described in Kaposi's sarcoma. Tumor cells in both cases expressed markers for endothelial cells. The clinical and histologic character of these neoplasms differentiates them from Kaposi's sarcoma, hemangioendothelioma, and from conventional juvenile hemangioma.
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