title: Giant dermatofibrosarcoma protuberans - rare form of mesenchymal tissue neoplasm: case presentation.
abstract: Dermatofibrosarcoma protuberans (DFSP), a rare type of mesenchymal neoplasm, is defined by the WHO as a superficial sarcoma with low-grade malignancy that develops in the cutaneous and subcutaneous tissues. The purpose of this paper is to present a case of a giant DFSP, with post-traumatic onset in childhood and a very long evolution.
  
  
  METHODS
  51-year-old Caucasian patient presents for 41 years a presternal neoplastic lesion, with onset at 10-year-old, few months after a strong trauma. The patient addressed for a clinic examination, secondary to a spontaneous hemorrhage of the lesion. The local examination reveals the presence of a red-purple polynodular neoplastic lesion of 180×110×30 mm, of firm consistency, adherent to the subcutaneous tissue, painless, with lateral extension at 8 o'clock as an erythematous infiltrated atrophic plaque appearance. One of these nodular masses presents surface ulceration and areas of necrosis. The CT scan did not detect any infiltration into the pectoral muscle or loco-regional metastasis. Under general anesthesia a wide surgical excision with free macroscopic margins of 3 cm was performed. Histopathological diagnosis was DFSP, with evidence of tumoral spindle cells disposed in storiform pattern, embedding small adipocyte panicles, creating a lace-like or honeycomb appearance. Immunohistochemically, the tumor cells express an intense and diffuse CD34 and they are negative for S-100 and SMA. The Ki-67 is focal positive in almost 2-4%. Clinical and paraclinical monitoring at 18 months follow-up does not detect any local recurrences or metastases, and an excellent quality of life.
authors.affiliation: Department of Surgery, University of Medicine and Pharmacy of Tirgu Mures, Romania; muresanadi@yahoo.com.
authors.forename: Ovidiu Simion
authors.initials: OS
authors.lastname: Cotoi
authors.forename: Adrian Vasile
authors.initials: AV
authors.lastname: Mureşan
authors.forename: Mariana Cornelia
authors.initials: MC
authors.lastname: Tilinca
authors.forename: Andreea Luciana
authors.initials: AL
authors.lastname: Chiotoroiu
authors.forename: Mihai Alexandru
authors.initials: MA
authors.lastname: Badea
authors.forename: Mihai Dorin
authors.initials: MD
authors.lastname: Vartolomei
authors.forename: Mircea
authors.initials: M
authors.lastname: Suciu
authors.forename: Silviu Horia
authors.initials: SH
authors.lastname: Morariu
mesh_terms: D001706:Biopsy; D018223:Dermatofibrosarcoma / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008648:Mesoderm / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology* / Q000601:surgery