medigraphic.com
Colegio de Medicina Interna de México.

2014, Number 5

<< Back Next >>

Med Int Mex 2014; 30 (5)

Diagnostic-therapeutic approach of dermatofibrosarcoma protuberans in adults

García-Santos EP, Sánchez-García S, Alberca-Páramo A, Martín-Fernández J, Relea-Calatayud F

Language: Spanish
References: 12
Page: 613-617
PDF size: 438.19 Kb.


ABSTRACT

Dermatofibrosarcoma protuberans is a dermal neoplasm of uncertain origin, probably of mesenchymal origin. It grows slowly with ease at locoregional recur, but rarely gives distant metastases (5%). We report the case of a 71-year-old man who consulted for a right inguinal mass of months of evolution that had increased in size gradually. A CT scan showed a nodular lesion in subcutaneous fat tissue without lesions suspicious of distance disemination. It was decided the tumor excision, aiming onlay mass that did not affect the deep planes. The final pathology was consistent with the diagnosis of dermatofibrosarcoma protuberans with nodular sclerotic changes and immunohistochemical study showed positivity for vimentin and CD34. Dermatofibrosarcoma protuberans is a locally malignant tumor of slow growth, and resembles with various pathologies that can develop in the same location, which can lead to making inappropriate treatments, delaying diagnosis and treatment, therefore, the need to raise awareness of this tumor type.


REFERENCES

  1. Cabanillas M, Labandeira J, Toribio J. Casos para el diagnóstico. Tumor en espalda. Dermatofibrosarcoma protuberans. Actas Dermosifiliogr 2005;96:623-625.

  2. Bowne WB, Antonescu CR, Leung DH, Katz SC, et al. Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer 2000;88:2711-2720.

  3. McArthur GA. Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior. Curr Opin Oncol 2006;18:341-346.

  4. Bednar B. Storiform neurofibromas of the skin, pigmented and nonpigmented. Cancer 1957;10:368-376.

  5. Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol 2006;30:436-443.

  6. Kerob D, Pedeutour F, Leboeuf C, Verola O, et al. Value of cytogenetic analysis in the treatment of dermatofibrosarcoma protuberans. J Clin Oncol 2008;26:1757-1759.

  7. Aiba S, Tabata N, Ishii H, Ootani H, Tagami H. Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34. Br J Dermatol 1992;127:79-84.

  8. Lee SJ, Mahoney MC, Shaughnessy E. Dermatofibrosarcoma protuberans of the breast: imaging features and review of the literature. Am J Roentgenol 2009;193:W64.

  9. Gloster HM Jr, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996;35:82-87.

  10. Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol 2012;9:1055-1063.

  11. Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 2010;28:1772-1779.

  12. Hattori H. Nodular sclerotic change in dermatofibrosarcoma protuberans: a potential diagnostic problem. Br J Dermatol 2003;148:357-360.




2020     |     www.medigraphic.com