Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors - 13/08/11
, Carlos Monteagudo, MD d, Onofre Sanmartín, MD a, José Antonio López-Guerrero b, Carlos Serra-Guillén, MD a, Andrés Poveda, MD c, Esperanza Jorda, MD e, Antonio Fernandez-Serra b, Antonio Pellín, MD d, Carlos Guillén, MD a, Antonio Llombart-Bosch, MD d
Reprint requests: Beatriz Llombart, MD, Department of Dermatology, Instituto Valenciano de Oncología, C/Profesor Beltrán Báguena, 4. 46009 Valencia, Spain.Abstract |
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.
Il testo completo di questo articolo è disponibile in PDF.Key words : collagen type I alpha 1–platelet-derived growth factor beta, dermatofibrosarcoma protuberans, fibrosarcomatous, Ki-67, Mohs micrographic surgery, p53, wide local excision
Abbreviations used : COL1A1, DFSP, DFSP-FS, MMS, PCR, PDGFB, WLE
Mappa
| Supported by Fomación en Investigación en Salud grant P1040822 and grant GV06/274 from the Consellería de Educación y Ciencias (Generalitat Valenciana). |
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| Conflicts of interest: None declared. |
Vol 65 - N° 3
P. 564-575 - settembre 2011 Ritorno al numero
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