Sclerosing dermatofibrosarcoma protuberans (DFSP): An unusual variant with focus on the histopathologic differential diagnosis

Department of Pathology, Consorcio General Hospital, Universitario de Valencia, Spain.
International Journal of Dermatology (Impact Factor: 1.31). 02/2006; 45(1):59-62. DOI: 10.1111/j.1365-4632.2004.02340.x
Source: PubMed


A 59-year-old man presented with a 10-cm x 8-cm tumoral plaque with a superficial nodule in the interscapular region of the back (Fig. 1). The lesion had been growing for 25 years. As a cystic lesion was suspected, the superficial nodule was biopsied. The histopathologic diagnosis was low-grade sarcoma with sclerosis. Two months after the initial biopsy, the lesion was completely excised, reaching the muscular fascia, with a 2-cm margin and with a free graft. Formalin-fixed paraffin-embedded samples were submitted to histologic and immunohistochemical study (4-microm paraffin sections); frozen tissue was submitted to electron microscopy. For histopathology, sections were stained with hematoxylin and eosin. Immunohistochemistry was performed following standard avidin-biotin immunoperoxidase procedures with primary antibodies for vimentin, CD34, smooth muscle-specific actin, bcl-2, S-100, desmin, myoglobin, factor VIII, p53 (all from DAKO, Copenhagen, Denmark), HHF-35 (Enzo Diagnostics, Farmingdale NY), cytokeratin (AE1/AE3) (Biogenex, San Ramon, CA), and factor XIIIa (Calbiochem Novabiochem Corporation, La Jolla, CA). At low magnification, the histologic study of the initial tumoral nodule revealed a poorly circumscribed mesenchymal proliferation, with fibroblastic-like neoplastic cells arranged in a fascicular and storiform pattern, admixed with extensive areas of sclerosis. At higher magnification, tumoral cells were spindle-shaped with hyperchromatic nuclei and scant cytoplasm. In some areas, sclerosis was so evident that a keloid-like pattern was seen (Fig. 2a). The surgical specimen showed a fibroblastic neoplastic proliferation infiltrating the dermis and hypodermis. In the dermis, cells were arranged in a storiform pattern, whereas in the hypodermis there was a honeycomb or lace-like pattern (Fig. 2b). There were also cellular areas alternating with sclerotic areas, with transitional zones in between, in both the dermis and hypodermis. The immunohistochemical study of the initial tumoral nodule and the surgical specimen showed that tumoral cells expressed vimentin, CD34 (Fig. 3), bcl-2, HHF-35, and smooth muscle actin. Neoplastic cells failed to show positivity with desmin, myoglobin, factor XIIIa, factor VIII, S-100, cytokeratin (AE1/AE3), and p53. An ultrastructural study revealed spindle cells having an irregular contour with a well-developed granular reticulum endoplasmic (REG) system in their cytoplasm, as well as some Golgi complexes and mitochondria. Also visible was the presence of many actin filaments and some myosin condensations (Fig. 4), characteristics of a fibroblastic cell with myofibroblastic differentiation. The final histopathologic diagnosis of the surgical specimen was sclerosing dermatofibrosarcoma protuberans. Two years after surgery, the patient is alive and well.

Download full-text


Available from: Vicente Sabater Marco, Oct 01, 2014
1 Follower
38 Reads
  • Source
    • "The differential and exclusive diagnosis of diseases that are similar to keloids and HTSs is important because various types of malignant tumors resemble these scars (16)–(19). For example, malignant dermatofibrosarcoma protuberans (DFSP) tumors have been mistaken for keloids or HTSs (16,17). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Scar formation is a consequence of the wound healing process that occurs when body tissues are damaged by a physical injury. Hypertrophic scars and keloids are pathological scars resulting from abnormal responses to trauma and can be itchy and painful, causing serious functional and cosmetic disability. The current review will focus on the definition of hypertrophic scars, distinguishing them from keloids and on the various methods for treating hypertrophic scarring that have been described in the literature, including treatments with clearly proven efficiency and therapies with doubtful benefits. Numerous methods have been described for the treatment of abnormal scars, but to date, the optimal treatment method has not been established. This review will explore the differences between different types of nonsurgical management of hypertrophic scars, focusing on the indications, uses, mechanisms of action, associations and efficacies of the following therapies: silicone, pressure garments, onion extract, intralesional corticoid injections and bleomycin.
    Clinics (São Paulo, Brazil) 08/2014; 69(8):565-573. DOI:10.6061/clinics/2014(08)11 · 1.19 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm of intermediate malignancy that is initially localized to the skin from where it can invade deep structures (fat, fascia, muscle and bone). It is the most frequent fibrohistiocytic tumor, comprising approximately 1.8 % of all soft tissue sarcomas and 0.1 % of all cancers. It has an estimated incidence of 0.8-5 cases per one million persons per year. Treatment of localized disease consists in complete surgical excision of the lesion by conventional surgery with wide margins (>3 cm) or by micrographic Mohs surgery. Although the cases of metastatic DFSP do not reach 5 % of the total, almost all of them appear after previous local relapses. The prognosis for metastatic cases is very poor with a survival of less than 2 years following detection of metastatic disease. Patients with locally advanced DFSP are not candidates for an initial radical surgical therapy therefore neoadyuvant treatment is required prior to surgery in order to reduce tumor burden. In this regard, chemotherapy and radiotherapy have not been highly efficacious so it is necessary to consider new alternatives. The demonstration of the oncogenic power of the translocation COL1A1-PDGFB in DFSP has allowed the successful introduction of drug therapy with antagonists of the PDGFB receptor for metastatic or locally advanced cases.
    Actas Dermo-Sifiliográficas 03/2007; 98(2):77-87. DOI:10.1016/S0001-7310(07)70019-4
  • [Show abstract] [Hide abstract]
    ABSTRACT: Keloid scarring is a clinical diagnosis, usually preceded by a history of localized trauma. Significant variation exists as to whether excised specimens are sent for routine histologic analysis. We aimed to review the histology of all clinically diagnosed keloids at our unit. All keloids diagnosed clinically and excised were identified between April 1995 and April 2006. The subsequent histology results were analyzed. Five hundred sixty-eight specimens were sent for pathologic investigation over an 11-year period. Four hundred fifty-eight (81%) were reported as "keloid," 60 (11%) as "acne keloidalis," 35 (6%) as "hypertrophic scar," and 14 (2%) as "normal scar." There were no reported malignancies or dysplasias. These histology results suggest that, given a good clinical suspicion of keloid, it may be unnecessary to send specimens at excision for routine histology.
    Annals of Plastic Surgery 03/2008; 60(2):186-7. DOI:10.1097/SAP.0b013e318056d6cc · 1.49 Impact Factor
Show more