American Journal of Clinical Dermatology

, Volume 6, Issue 1, pp 61–64 | Cite as

Dermatofibrosarcoma Protuberans at the Site of a Central Venous Line

Case Report
Case Report

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous tumor that usually does not metastasize. Clinically, it may present as a reddish macule to protuberant nodule affecting the trunk and proximal extremities of young and middle-aged adults. Histologically, it is characterized by a monomorphous storiform proliferation of spindle cells, involving the dermis and hypodermis, with a honeycomb pattern of infiltration of the subcutaneous fat. In case of difficulty in diagnosis, immunohistochemical markers (especially CD34) are highly sensitive for DFSP. It is important to remember that the rarity of DFSP, its resemblance to hypertrophied scars and keloids, and its appearance at trauma sites sometimes make the diagnosis of DFSP difficult and delayed.

In the literature, DSFP was not described in a child until 1957. Here we report an instance of DFSP at an unusual site in a 9-year-old child, which was the location of a previous central venous line insertion in the left supraclavicular area. A complete excision of the tumor with a wide surgical margin of 3cm of visibly uninvolved tissue was performed, followed by a deltopectoral flap and skin split graft. Postoperatively, the patient’s general condition was stable and he was discharged after 5 days to be followed up 3 months later in our clinic.

Keywords

Factor XIIIa Fibrous Histiocytoma Hypertrophied Scar Dermatofibrosarcoma Protuberans Skin Split Graft 

Notes

Acknowledgment

No sources of funding were used to assist in the preparation of this case report. The authors have no conflicts of interest that are directly relevant to the content of this case report.

References

  1. 1.
    Fletcher CD, Evans BJ, MacArtney JC, et al. Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature. Histopathology. 1985; 9 (9): 921–38PubMedCrossRefGoogle Scholar
  2. 2.
    Weiss SW. Histological typing of soft tissue tumours. 2nd ed. WHO international histological classification of tumours. Berlin: Springer, 1994Google Scholar
  3. 3.
    Koh CK, Ko CB, Bury HP, et al. Dermatofibrosarcoma protuberans. Int J Dermatol. 1995; 34: 256–60PubMedCrossRefGoogle Scholar
  4. 4.
    Taylor RW, Helwig EB. Dermatofibrosarcoma protuberans: a study of 115 cases. Cancer. 1962; 15: 717–25PubMedCrossRefGoogle Scholar
  5. 5.
    Gloster HM. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996; 36: 355–74CrossRefGoogle Scholar
  6. 6.
    Chaung TY, Su WPD, Muller SA. Incidence of cutaneous T-cell lymphoma and other rare skin cancers in a defined population. J Am Acad Dermatol. 1990; 23: 254–6CrossRefGoogle Scholar
  7. 7.
    Enzinger FM, Weiss SW. Soft tissue tumors. 3rd ed. St Louis (MO): Mosby, 1995: 325–37Google Scholar
  8. 8.
    Pack GT, Tabah EJ. Dermatofibrosarcoma protuberans: a report of thirty-nine cases. AMA Arch Surg. 1951; 62: 391–411PubMedCrossRefGoogle Scholar
  9. 9.
    Hadju SI. Pathology of soft tissue tumors. 1st ed.Philadelphia (PA): Lea & Febiger, 1979: 82Google Scholar
  10. 10.
    Fletcher CD, Theaker JM, Flanagan A, et al. Pigmented dermatofibrosarcoma protuberans (Bednar tumour): melanocytic colonization or neuroectodermal differentiation? A clinicopathological and immunohistochemical study. Histopathology. 1988; 13: 631–43PubMedCrossRefGoogle Scholar
  11. 11.
    Prieto VG, Reed JA, Shea CR. CD34 immunoreactivity distinguishes between scar tissue and residual tumor in re-excisional specimens of dermatofibrosarcoma protuberans. J Cutan Pathol. 1994; 21: 324–9PubMedCrossRefGoogle Scholar
  12. 12.
    Mark RJ, Bailet JW, Tran LM, et al. Dermatofibrosarcoma protuberans of the head and neck: a report of 16 cases. Arch Otolaryngol Head Neck Surg. 1993; 119: 891–6PubMedCrossRefGoogle Scholar
  13. 13.
    Gutierrez G, Ospina JE, de Baez NE, et al. Dermatofibrosarcoma protuberans. Int J Dermatol. 1984; 23: 396–401PubMedCrossRefGoogle Scholar
  14. 14.
    Escalona-Zapata J, Alvarez Fernandez E, Llorca Escuin F. The fibroblastic nature of dermatofibrosarcoma protuberans: a tissue culture and ultrastructural study. Virchows Arch A Pathol Anat Histol. 1981; 391: 165–75PubMedCrossRefGoogle Scholar
  15. 15.
    Hashimoto K, Brownstein MH Jakobiec FA. Dermatofibrosarcoma protuberans: a tumor with perineural and endoneural features. Arch Dermatol. 1974; 11: 874–5CrossRefGoogle Scholar
  16. 16.
    Diaz-Cascajo C. Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant. J Cutan Pathol. 1997; 24: 197–8PubMedCrossRefGoogle Scholar
  17. 17.
    Laurier R, Wolff HH, Jones RE. An immunohistochemical study of dermatofibrosarcoma protuberans supports its fibroblastic character and contradicts neuroectodermal or histiocytic components. Am J Dermatopathol. 1990; 12: 25–30CrossRefGoogle Scholar
  18. 18.
    Pedeutour F, Simon MP, Minoletti F. Translocation, t(17;22)(q22;q13), in dermatofibrosarcoma protuberans: a new tumor-associated chromosome rearrangement. Cytogenet Cell Genet. 1996; 72: 171–4PubMedCrossRefGoogle Scholar
  19. 19.
    Pedeutour F, Lacour JP, Perrin C. Another case of t(17;22)(q22;q13) in an infantile dermatofibrosarcoma protuberans. Cancer Genet Cytogenet. 1996; 89: 175–6PubMedCrossRefGoogle Scholar
  20. 20.
    Craver RD, Correa H, Kao Y, et al. Dermatofibrosarcoma protuberans with 46,XY,t(X;7) abnormality in a child. Cancer Genet Cytogenet. 1995; 80: 75–7PubMedCrossRefGoogle Scholar
  21. 21.
    Mandahl N, Heim S, Willen H, et al. Supernumerary ring chromosome as the sole cytogenetic abnormality in a dermatofibrosarcoma protuberans. Cancer Genet Cytogenet. 1990; 49: 273–5PubMedCrossRefGoogle Scholar
  22. 22.
    Green JJ, Heymann RH. Dermatofibrosarcoma protuberans occurring in smallpox vaccination scar. J Am Acad Dermatol. 2003; 48: S54–5PubMedCrossRefGoogle Scholar
  23. 23.
    Keshen TH, Cedema PS, Savell Jr VH, et al. Clinical and pathological features of pediatric dermatofibrosarcoma protuberans. Ann Plast Surg. 1995; 35: 633–7PubMedCrossRefGoogle Scholar
  24. 24.
    McKee PH, Fletcher CDM. Dermatofibrosarcoma protuberans presenting in infancy and childhood. J Cutan Pathol. 1991; 18: 241–6PubMedCrossRefGoogle Scholar
  25. 25.
    McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases: five with metastasis. Ann Surg. 1967; 166: 803–16PubMedCrossRefGoogle Scholar
  26. 26.
    Burkhardt BR, Soule EH, Winkelmann RK, et al. Dermatofibrosarcoma protuberans: study of fifty-six cases. Am J Surg. 1966; 111: 638–44PubMedCrossRefGoogle Scholar
  27. 27.
    Bouyssou-Gauthier ML, Labrousse F, Longis B, et al. Dermatofibrosarcoma protuberans in childhood. Pediatr Dermatol. 1997; 14: 463–5PubMedCrossRefGoogle Scholar
  28. 28.
    Garg SK, Subbuswamy SG, Alabi JO, et al. Dermatofibrosarcoma protuberans in Northern Nigeria: a clinico-pathological review of 17 cases. Clin Oncol. 1978; 4: 113–22PubMedGoogle Scholar
  29. 29.
    Altman DA, Nickoloff BJ, Fivenson DP. Differential expression of factor XIIIa and CD34 in cutaneous mesenchymal tumors. J Cutan Pathol. 1993; 16: 154–8CrossRefGoogle Scholar
  30. 30.
    McGregor JK. Role of surgery in the management of dermatofibrosarcoma protuberans. Ann Surg. 1961; 154: 255–8PubMedCrossRefGoogle Scholar
  31. 31.
    Teirrer-Lacombe MJ, Gilloub L, Maire G, et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data. Am J Surg Pathol. 2003; 27 (1): 27–39CrossRefGoogle Scholar
  32. 32.
    Checketts SR, Hamilton TK, Baughman RD. Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature. J Am Acad Dermatol. 2000; 42: 907–13PubMedCrossRefGoogle Scholar
  33. 33.
    Schmoeckel C, Albini A, Krieg T, et al. The fibroblastic nature of dermatofibrosarcoma protuberans: morphological investigations in vivo and in vitro. Arch Dermatol Res. 1985; 278: 138–47PubMedCrossRefGoogle Scholar
  34. 34.
    Dupree WB, Langloss JM, Weiss SW. Pigmented dermatofibrosarcoma protuberans (Bednar tumor): a pathologic, ultrastructural and immunohistochemical study. Am J Surg Pathol. 1985; 9: 630–9PubMedCrossRefGoogle Scholar
  35. 35.
    Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol. 1993; 15: 429–34PubMedCrossRefGoogle Scholar
  36. 36.
    Weiss SW, Nickoloff BJ. CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions. Am J Surg Pathol. 1993; 17: 1039–45PubMedCrossRefGoogle Scholar
  37. 37.
    Kutzner H. Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin fixed, paraffin-embedded tissue. J Am Acad Dermatol. 1993; 28: 613–7PubMedCrossRefGoogle Scholar
  38. 38.
    Shmookler BM, Enzinger FM. Giant cell fibroblastoma: a peculiar childhood tumor [abstract]. Lab Invest. 1982; 46: 76AGoogle Scholar
  39. 39.
    Khan H, Fekete E, From L. Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa. Hum Pathol. 2001; 32: 50–6CrossRefGoogle Scholar
  40. 40.
    Kransdorf MJ, Meis Kindblom JM. Dermatofibrosarcoma protuberans: radiologic appearance. AJR Am J Roentgenol. 1994; 163: 391–4PubMedGoogle Scholar
  41. 41.
    Sato N, Kimura K, Tomita Y. Recurrent dermatofibrosarcoma protuberans with myxoid and fibrosarcomatous changes paralleled by loss of CD34 expression. J Dermatol. 1995; 22: 665–72PubMedGoogle Scholar
  42. 42.
    Bashara ME, Jules KT, Potter GK. Dermatofibrosarcoma protuberans: 4 years after local trauma. J Foot Surg. 1992; 31: 160–5PubMedGoogle Scholar
  43. 43.
    Hoffmann E. Uber das Krollentreibende Fibrosarkom der Haut (Dermatofibrosarkoma protuberans). Dermal Ztschr. 1925; 43: 1–28CrossRefGoogle Scholar
  44. 44.
    Kahn TA, Liranzo MO, Vidimos AT, et al. Pathological case of the month: congenital dermatofibrosarcoma protuberans. Arch Pediatr Adolesc Med. 1996; 150: 549–50PubMedCrossRefGoogle Scholar
  45. 45.
    Mbonde MP, Amir H, Kitinya JN. Dermatofibrosarcoma protuberans: a clinicopathological study in an African population. East Aft Med J. 1996; 73: 410–3Google Scholar
  46. 46.
    Rowsell AR, Poole MD, Godfrey AM. Dermatofibrosarcoma protuberans: the problems of surgical management. Br J Plast Surg. 1986; 39: 262–4PubMedCrossRefGoogle Scholar
  47. 47.
    Smola MG, Soyer HP, Scharnagl E. Surgical treatment of dermatofibrosarcoma protuberans: a retrospective study of 20 cases with review of literature. Fur J Surg Oncol. 1991; 17: 447–53Google Scholar

Copyright information

© Adis Data Information BV 2005

Authors and Affiliations

  1. 1.Dermatology DepartmentKing Faisal University, College of MedicineAlkhobarSaudi Arabia

Personalised recommendations