Elsevier

Current Problems in Cancer

Volume 39, Issue 4, July–August 2015, Pages 248-257
Current Problems in Cancer

Current approaches to cutaneous sarcomas: Dermatofibrosarcoma protuberans and cutaneous leiomyosarcoma

https://doi.org/10.1016/j.currproblcancer.2015.07.006Get rights and content

Introduction

Dermatofibrosarcoma protuberans (DFSP) and cutaneous leiomyosarcoma (LMS) are 2 rare dermal sarcomas that are often locally aggressive but rarely metastasize.1, 2 LMS accounts for less than 3% and DFSP accounts for less than 1% of all soft tissue sarcomas.2, 3 Both malignancies are slow-growing tumors; however, LMS has a propensity to afflict white individiuals.1

DFSP and LMS often present as firm nodules on the skin with minimal symptoms. Both cutaneous sarcomas, but especially DFSP, tend to grow in an asymmetric pattern with fingerlike projections and without pigmentation. On biopsy, it is highly recommended that a dermatopathologist or sarcoma pathologist evaluate the specimen, because of the rarity of these lesions.

Staging of both DFSP and LMS is according to the American Joint Committee on Cancer (AJCC) guidelines for soft tissue sarcomas (Table 1). In general, these tumors are superficial to the deep fascia and less than 5 cm in greatest dimension; as such, they are often staged as T1aN0M0 lesions, provided there is no evidence of metastatic spread (Table 1). Most lesions are characteristically low grade. However, very large exophytic lesions, DFSP with fibrosarcomatous changes (DFSP-FS), or high-grade LMSs are often stage II or III.

Our current treatment approach to LMS and DFSP is multidisciplinary. As described later, the role of meticulous pathologic review in margin analysis by the dermatopathologist is just as important as the meticulous nature in which the surgeon resects the tumors.4 Although radiotherapy is rarely used as a definitive treatment for DFSP or LMS, it certainly has a role as adjuvant therapy in selected cases1, 5, 6 (Table 2).

Chemotherapy (ie, gemcitabine and docetaxel) and other small-molecule inhibitors (ie, imatinib or sorafenib) may also have some activity in patients with metastatic or unresectable DFSP, but high-level evidence is lacking (Table 2).6, 7

Section snippets

Clinical features

DFSP is a rare tumor that predominantly affects young and middle-aged adults and occurs less frequently in pediatric and elderly patients.8, 9, 10 DFSP represents the second most common primary dermal sarcoma after Kaposi sarcoma.11, 12 DFSP is a tumor that often arises on the trunk and proximal extremities, less frequently on the head and neck area, and rarely affects the genitalia.7, 13, 14, 15 Clinically, DFSP appears as a slow-growing, nonpigmented, subcutaneous, and often multinodular mass

Conclusions

DFSP and LMS are 2 cutaneous sarcomas with similar management strategies. Both require multidisciplinary management, and both carry very good long-term prognosis when aggressively treated and intensely monitored. In caring for these patients, the most important factor is close cooperation between the patient, the dermatopathologist, the dermatologist, and the surgeon.

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