ABSTRACT
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a low rate of metastatic disease. Previous series have shown a superiority of Mohs micrographic surgery (MMS) compared with wide local excision (WLE). Likewise, there is paucity of data examining the long-term follow-up of patients. OBJECTIVE: The purpose of the current study was to examine the outcome of surgical treatment of primary DFSP of the trunk and extremities. METHODS: We reviewed 236 patients (115 females, 121 males, mean age 41 ± 15 years) undergoing MMS (n = 81, 34%) or WLE (n = 155, 66%) to treat a primary DFSP. Mean tumor size and follow-up was 4 ± 2 cm and 7 years, respectively. Final margins were negative in 230 (97%) patients. RESULTS: There was no difference (p > 0.05) in patient age, sex, tumor size, negative margin excision, or history of a previous inadvertent excision between patients who underwent WLE and those undergoing MMS. There were two cases of local recurrence and two cases of metastasis, with no difference in the 5-year local recurrence-free survival (98% vs. 99%, p = 0.69) or metastatic-free survival (98% vs. 100%, p= 0.27) between WLE and MMS. CONCLUSION: There was no difference in oncologic outcome comparing MMS with WLE for DFSP outside the head and neck. The goal of treatment for DFSP is to achieve a negative margin, regardless of surgical treatment modalities. A 'less is more' approach to follow-up can likely be taken for patients with completely resected DFSP in easy-to-examine anatomical areas. In these patients, no formal follow-up should be required.
Subject(s)
Dermatofibrosarcoma , Skin Neoplasms , Male , Female , Humans , Adult , Middle Aged , Dermatofibrosarcoma/surgery , Dermatofibrosarcoma/pathology , Retrospective Studies , Skin Neoplasms/surgery , Skin Neoplasms/pathology , Neoplasm Recurrence, Local/surgery , Neoplasm Recurrence, Local/pathology , Margins of Excision , Treatment Outcome , Multicenter Studies as TopicABSTRACT
BACKGROUND/AIM: Dermat of ibrosarcoma protuberans (DFSP) is a soft-tissue sarcoma with a high risk of local recurrence, though typically never metastasizes. DFSP can transform into high-grade fibrosarcoma (DFSP-FS), which has a risk of metastasis. Currently, treatment for DFSP includes Moh's micrographic surgery (MMS); however, this is not recommended for DFSP-FS. Often, the transformation to DFSP-FS is not recognized until the final histological diagnosis. At that point, wide local excision (WLE) of a previous MMS site can be morbid. As such, we analyzed patient risk factors to allow identification of DFSP-FS transformation at presentation. PATIENTS AND METHODS: We reviewed 368 (174 female, 194 male) patients with a mean age of 42 years from two sarcoma centers. A total of 319 (87%) patients had a history of DFSP and 49 (13%) had DFSP-FS. RESULTS: When comparing patients with a DFSP to those with a DFSP-FS, patients with a DFSP-FS were more likely (p<0.05) to be older, female and with larger tumors. A painful mass and rapidly enlarging mass were associated with DFSP-FS. CONCLUSION: Patients who presented with DFSP-FS were found to typically have a larger, painful, and growing mass. Patients with these features should be referred for WLE over MMS at presentation.