Dermatofibrosarcoma Protuberans of the Scalp: A Challenging Tumor With a Proposed Modification to the Slow Mohs Technique

Nelson, Toby G.; Gonda, Peter; Sheppard, Pippa; Keohane, S.G.

doi:10.1097/dss.0000000000002132

Cited by 3 publications

(2 citation statements)

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“…Most DFSPs are localized on the trunk (40-50%), followed by proximal extremities (30-40%), and then the head and neck (10-15%) [43][44][45]. Infrequently documented cases have been reported on the toes [22,[46][47][48][49], scalp [50], breast [51][52][53] and vulva [54].…”

Section: Clinical Characteristicsmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

Hao¹,

Billings

et al. 2020

JCM

105

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Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. It has a high local recurrence rate but low metastatic potential. It is characterized by a uniform spindle cell arrangement, classically with a storiform pattern and CD34 immunoreactivity. The histomorphology and immunophenotype overlap with a broad range of other neoplasms. The standard treatment is complete surgical excision. The surgical procedures include wide local excision (WLE) with tumor free margins, Mohs micrographic surgery (MMS) and amputation. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. DFSP has characteristic t(17; 22) (q22; q13), resulting in a COL1A1- PDGFB fusion transcripts in more than 90% of DFSPs. Molecular detection of the gene rearrangement or fusion transcripts is helpful for the diagnosis of patients with atypical morphology and for screening candidates for targeted therapy with tyrosine kinase inhibitors. The aims of the present review are to update the clinical presentation, tumorigenesis and histopathology of DFSP and its variants for diagnosis and differential diagnosis from other benign and malignant tumors, to compare the advantages and drawbacks of WLE and MMS, to propose the baseline for selecting surgical procedure based on tumor’s location, size, stage and relationship with surrounding soft tissue and bone structures, and to provide a biologic rationale for the systemic therapy. We further propose a modified clinical staging system of DFSP and a surveillance program for the patients after surgical excision.

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Section: Clinical Characteristicsmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

Hao¹,

Billings

et al. 2020

JCM

105

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“…Clinically, DFSP presents as a slow-growing, firm, multilobular nodule or plaque that ranges in colour from flesh-coloured to red and has irregular margins on the trunk (50%), preferentially on the shoulder girdle, upper and lower limbs (30–40%), and head-neck area (10–15%) [ 6 , 7 , 8 ]; rarely, DFSP has been reported to occur on toes [ 9 , 10 , 11 , 12 , 13 ], the scalp [ 14 ], breasts [ 15 , 16 , 17 ], and vulva [ 18 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of Postoperative Radiotherapy in the Management of Dermatofibrosarcoma Protuberans: A Multidisciplinary Systematic Review

Fionda,

Loperfido,

Di Stefani

et al. 2024

JCM

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Background: Dermatofibrosarcoma protuberans (DFSP) is a superficial soft tissue sarcoma, and surgical excision is the first-line treatment. The aim of this systematic review is to provide an update about the current indications and clinical results regarding the use of postoperative radiotherapy in DSFP, considering both adjuvant and salvage setting. Methods: We conducted a systematic literature review using the main scientific database, including Cochrane library, Scopus, and PubMed, for any relevant article about the topic, and we considered all available papers without any time restriction. Results: Twenty-two papers, published between 1989 and 2023, were retrieved and considered eligible for inclusion in this review. Regarding the fractionation schedules, most authors reported using standard fractionation (2 Gy/die) with a wide total dose ranging from 50 to 70 Gy. The local control after postoperative radiotherapy was excellent (75–100%), with a median follow-up time of 69 months. Conclusions: After the primary surgical management of DFSP, postoperative radiotherapy may either be considered as adjuvant treatment (presence of risk factors, i.e., close margins, recurrent tumours, aggressive histological subtypes) or as salvage treatment (positive margins) and should be assessed within the frame of multidisciplinary evaluation.

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