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OVERVIEW

image SUMMARY

  • There are several different types of sarcomatous tumors, including dermatofibrosarcoma protuberans, leiomyosarcoma, liposarcoma, angiosarcoma, epithelioid sarcoma, fibrosarcoma, and undifferentiated pleomorphic sarcoma.

  • Histological analysis of sarcomas is critical in differentiating between the numerous different subtypes.

  • Surgery is the mainstay of treatment for sarcomas.

  • Immunotherapy is a growing field of treatment for these tumors.

image DON’T FORGET

  • Mohs micrographic surgery can play a significant role in the surgical management of sarcomas.

  • The management of sarcomas requires a patient-centered multidisciplinary approach.

image CLINICAL PEARLS

  • Sarcomas can involve blood vessels, fibrous tissues, fat, muscle or nerves, and bone.

  • Clinically sarcomas often present as plaques or nodular tumors.

image PATIENT EDUCATION POINTS

  • Patients and caretakers should be aware that surgery plays an important role in sarcoma management. Chemotherapy, radiation, and immunotherapy can also be effective.

INTRODUCTION

Sarcomas are malignant tumors of the soft tissue, including blood vessels, fibrous tissues, fat, muscle or nerves, and bone. These tumors are relatively rare; the American Cancer Society estimates that in the year 2020 about 13,000 new soft tissue sarcomas will be diagnosed and that about 5000 people will die from these malignancies. Sarcomas, like many other tumors, are staged by the American Joint Committee on Cancer by classifying them in categories I through IV based on their histologic grade and TNM profile. TNM refers to the tumor size, the spread to nearby lymph nodes, and the presence or absence of metastases. Sarcoma grade refers to how closely the tumor cells resemble normal cells based on their differentiation, mitotic count, and tumor necrosis. The most common types of sarcoma are undifferentiated pleomorphic sarcoma (UPS), liposarcoma, and leiomyosarcoma.1

DERMATOFIBROSARCOMA PROTUBERANS

Epidemiology

Dermatofibrosarcoma protuberans (DFSP) is a low-grade, locally aggressive sarcoma that comprises 0.1% of all malignancies and 1% of all soft tissue sarcomas.2,3 Women are more likely to develop DFSP than men, and patients of African descent have a higher incidence than White patients. DFSP most often arises in the third to fifth decades of life and occurs on the trunk in 50% to 60% of cases, on the proximal extremities in 20% to 30%, and in the head and neck region in 10% to 15%.2,4 Ten-year survival is high at 99.1%5; however, metastasis, most commonly to the lung, can occur in 2% to 5% of patients.4 Seven percent to 16% of DFSPs undergo fibrosarcomatous transformation into a higher-grade malignancy.2

Larger tumor size is associated with older age, Black race, and male sex,6 while factors associated with a worsened prognosis include male sex, Black race, and increased tumor size.

Clinical Findings

DFSP typically presents as a violaceous, erythematous, or flesh-colored indurated plaque that can transform over time ...

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