title: Dermatofibrosarcoma protuberans: a clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors.
abstract: BACKGROUND
  Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS).
  
  
  OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain; Department of Pathology, Hospital Clínico Universitario, Valencia, Spain. Electronic address: beatriz.llombart@uv.es.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Llombart
authors.affiliation: Department of Pathology, Hospital Clínico Universitario, Valencia, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Monteagudo
authors.affiliation: Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Onofre
authors.initials: O
authors.lastname: Sanmartín
authors.affiliation: Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: José Antonio
authors.initials: JA
authors.lastname: López-Guerrero
authors.affiliation: Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Serra-Guillén
authors.affiliation: Department of Medical Oncology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Andrés
authors.initials: A
authors.lastname: Poveda
authors.affiliation: Department of Dermatology, Hospital Clínico Universitario, Valencia, Spain.
authors.forename: Esperanza
authors.initials: E
authors.lastname: Jorda
authors.affiliation: Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Fernandez-Serra
authors.affiliation: Department of Pathology, Hospital Clínico Universitario, Valencia, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Pellín
authors.affiliation: Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Guillén
authors.affiliation: Department of Pathology, Hospital Clínico Universitario, Valencia, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Llombart-Bosch
mesh_terms: D000293:Adolescent; D000328:Adult; D018952:Antigens, CD34 / Q000032:analysis; D000090602:Collagen Type I, alpha 1 Chain; D018223:Dermatofibrosarcoma / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000032:analysis; D008297:Male; D008875:Middle Aged; D015580:Mohs Surgery; D015514:Oncogene Proteins, Fusion / Q000032:analysis*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D016159:Tumor Suppressor Protein p53 / Q000032:analysis; D055815:Young Adult
chemical_list: D018952:Antigens, CD34; C000716127:COL1A1 protein, human; C120892:COLIA1-PDGFB fusion protein, human; D000090602:Collagen Type I, alpha 1 Chain; D019394:Ki-67 Antigen; D015514:Oncogene Proteins, Fusion; D016159:Tumor Suppressor Protein p53