Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors

详细信息    查看全文

• 作者：Beatriz Llombart MD^a ; ^d ; ^{beatriz.llombart@uv.es"" rel=""nofollow} ; Carlos Monteagudo MD^d ; Onofre Sanmartí ; n MD^a ; José ; Antonio Ló ; pez-Guerrero^b ; Carlos Serra-Guillé ; n MD^a ; André ; s Poveda MD^c ; Esperanza Jorda MD^e ; Antonio Fernandez-Serra^b ; Antonio Pellí ; n MD^d ; Carlos Guillé ; n MD^a ; Antonio Llombart-Bosch MD^d
• 关键词：collagen type I alpha 1– ; platelet-derived growth factor beta ; dermatofibrosarcoma protuberans ; fibrosarcomatous ; Ki-67 ; Mohs micrographic surgery ; p53 ; wide local excision
• 刊名：Journal of the American Academy of Dermatology
• 出版年：2011
• 出版时间：September 2011
• 年：2011
• 卷：65
• 期：3
• 页码：564-575
• 全文大小：2404 K

文摘

Background

Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS).

Objectives

We sought to compare the clinicopathological, immunohistochemical, genetic, and therapeutic features between DFSP and DFSP-FS.

Methods

The clinicopathological features were reviewed in 63 DFSP and 12 DFSP-FS. Immunohistochemistry and multiplex reverse transcriptase-polymerase chain reaction were carried out using formalin-fixed, paraffin-embedded tissue, using specific primers for collagen type I alpha 1 (COL1A1) and platelet-derived growth factor beta (PDGFB).

Results

DFSP-FS was associated with tumor history longer than 5 years (P = .009), tumor size greater than 4 cm (P = .001), more stages of modified Mohs micrographic surgery (P = .005), expansive subcutaneous infiltration (P = .005), muscular invasion (P = .0001), absence of CD34 staining (P = .018), p53 positivity (P = .006), and increased proliferative activity (P = .004) compared with DFSP. The COL1A1-PDGFB fusion transcript was found in 100 % DFSP-FS and 72 % DFSP. No association was found between the different COL1A1-PDGFB fusion transcripts and the different histologic subtypes. Wide local excision (2 cm) was performed in 47 % of cases and modified Mohs micrographic surgery in 53 % . After a mean follow-up of 73 months (range 21-235), 6 patients had local recurrence (5 DFSP, 1 DFSP-FS) and one died of disease (DFSP-FS). The only factor related to local recurrence was the type of surgery (17 % wide local excision vs 0 % modified Mohs micrographic surgery) (P = .006).

Limitations

Our study is retrospective. Prospective studies are necessary to confirm our results.

Conclusions

DFSP-FS reflects tumor progression in DFSP, with larger size, particular invasive patterns, p53 expression, and increased proliferative activity. However, as in low-grade DFSP, appropriate surgery permits a tumor-free excision. COL1A1-PDGFB is a useful tool for diagnosis of DFSP and particularly for DFSP-FS.