Dermatofibrosarcoma Protuberans Treated at a Single Institution: A Surgical Disease With a High Cure Rate

Fiore, Marco; Miceli, Rosalba; Mussi, Chiara; Vullo, Salvatore Lo; Mariani, Luigi; Lozza, Laura; Collini, Paola; Olmi, Patrizia; Casali, Paolo G.; Gronchi, Alessandro

doi:10.1200/jco.2005.02.5122

Cited by 196 publications

(176 citation statements)

References 19 publications

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“…No differences were detected in sex, age, and anatomic location between DFSP and FS-DFSP. As we had already described (2), in this more recent and larger series, focused only on primary cases, we confirm that negative margin resection was always curative for DFSP and prevented LR (not DM) in FS-DFSP as well. The only factor associated to a metastatic risk was the presence of fibrosarcomatous features.…”

Section: Discussionsupporting

confidence: 62%

“…Dermatofibrosarcoma protuberans is a rare cutaneous sarcoma known for its indolent course, its tendency to recur mostly at the site of the primary tumor and its high cure-rate when it can be completely resected (1)(2)(3). The superficial location and the very low metastatic potential of classic dermatofibrosarcoma protuberans (DFSP) may justify a less aggressive surgical approach as compared with what applied to soft tissue sarcomas (STS).…”

Section: Introductionmentioning

confidence: 99%

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Evolution of Dermatofibrosarcoma Protuberans to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial–Mesenchymal Transition–like Process and 22q Loss

Stacchiotti

Astolfi

Gronchi

et al. 2016

Molecular Cancer Research

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Dermatofibrosarcoma protuberans (DFSP) is a rare and indolent cutaneous sarcoma. At times, a fibrosarcomatous transformation marked by a more aggressive clinical behavior may be present. We investigated the natural history and the molecular bases of progression from classic DFSP to the fibrosarcomatous form (FS-DFSP), looking, retrospectively, at the outcome of all patients affected by primary DFSP treated at our institution from 1993 to 2012 and analyzing the molecular profile of 5 DFSPs and 5 FS-DFSPs by an integrated genomics approach (whole transcriptome sequencing, copy number analysis, FISH, qRT-PCR, IHC). The presence of fibrosarcomatous features was identified in 20 (7.6%) patients out of 263 DFSP. All cases were treated with macroscopic complete surgery. A local relapse occurred in 4 of 23 patients who received a microscopic marginal surgery (2 classic DFSP, 2 FS-DFSP), while metastasis affected 2 patients, both FS-DFSP (10% of FS-DFSP), being the first event. DFSP evolution to FS-DFSP was paralleled by a transcriptional reprogramming. The recurrent loss of chromosome 22q appeared to contribute to this phenomenon by promoting the expression of epigenetic regulators, such as EZH2. Loss of the p16/CDKN2A/INK4A locus at 9p was also observed in two FS-DFSP metastatic cases.Implications: FS-DFSP is a rare subgroup among DFSP, with a 10% metastatic risk, that was independent from local recurrence and that was not observed in DFSP, that were all cured by wide surgery. Chromosome 22q deletion might play a role in FS-DFSP, and p16 loss may convey a poor outcome. EZH2 dysregulation was also found and represents a druggable target.

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Section: Discussionsupporting

confidence: 62%

Section: Introductionmentioning

confidence: 99%

Evolution of Dermatofibrosarcoma Protuberans to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial–Mesenchymal Transition–like Process and 22q Loss

Stacchiotti

Astolfi

Gronchi

et al. 2016

Molecular Cancer Research

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“…However, the response from patients remains poor and locoregional recurrence has been observed during follow-up (10)(11)(12). The natural history of DFSP is low-grade malignancy, with a 26-60% local recurrence rate attributed to incomplete excision due to poor circumscription and irregular boundaries (13).…”

Section: Discussionmentioning

confidence: 99%

“…Wide excision remains the cornerstone of the treatment of DFSP (10)(11)(12). However, the response from patients remains poor and locoregional recurrence has been observed during follow-up (10)(11)(12).…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological features of dermatofibrosarcoma protuberans

et al. 2015

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Abstract. Dermatofibrosarcoma protuberans (DFSP) is a superficial cutaneous tumor of low malignant potential characterized by a high rate of local recurrence. The histopathological appearance shows uniform spindle neoplastic cells arranged in a predominantly storiform pattern, typically with positive staining for cluster of differentiation (CD)34 and vimentin on immunohistochemistry. A minority of cases of DFSP have areas of sarcomatous transformation. Wide surgical excision is the cornerstone of treatment for DFSP. The objective of the present study was to determine the clinicopathological features of DFSP. Pathological records were searched for cases of DFSP in the database of the Department of Pathology, Faculty of Medicine Ramathibodi Hospital (Mahidol University, Bangkok, Thailand) between 1994 and 2013. The results showed 68 cases with DFSP. The mean age at diagnosis was 40 years (range, 3-86 years). Among this group of patients, 26 cases (38.2%) experienced local recurrence and 6 (8.8%) exhibited sarcomatous transformation of DFSP. The factors that predict the recurrence of DFSP are an incorrect first pathological diagnosis and an inadequate surgical margin. The factors that predict the sarcomatous transformation of DFSP are a larger tumor size and an incorrect first pathological diagnosis. In patients who have tumors with spindle cells arranged in a storiform pattern, CD34 immunohistochemical staining provides the definitive diagnosis. Exact histopathological categorization is important to select the appropriate treatment and predict the clinical outcome.

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“…The skin lesions were limited to the interdigital spaces of the hand. In 1993, Patrizi et al reported the case of a 7-year-old female who showed symmetrical lesions of the second, third and fourth interdigital spaces of the hands [3]. In 1995, Di Lernia et al reported a similar case of a 28-year-old male who showed symmetrical lesions of all the finger interdigital spaces of the hands [4].…”

Section: Sirmentioning

confidence: 96%