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Clinical Images  |   May 2020
Dermatofibrosarcoma Protuberans
Author Notes
  • From the Edward Via College of Osteopathic Medicine–Carolinas Campus in South Carolina, the Regional Medical Center in Orangeburg, South Carolina, and the University of Texas Health Science Center at San Antonio in Texas. 
  • Financial Disclosures: None reported. 
  • Support: None reported. 
  •  *Address correspondence to Stefano Natali, OMS IV, 5770 SW 53rd Terrace, Miami, FL, 33155–6334. Email: stefano.jose.natali@gmail.com
     
Article Information
Imaging / Obstetrics and Gynecology / Clinical Images
Clinical Images   |   May 2020
Dermatofibrosarcoma Protuberans
The Journal of the American Osteopathic Association, May 2020, Vol. 120, 362. doi:https://doi.org/10.7556/jaoa.2020.056
The Journal of the American Osteopathic Association, May 2020, Vol. 120, 362. doi:https://doi.org/10.7556/jaoa.2020.056
A 26-year-old woman with no prenatal care presented to the labor and delivery department in active labor at 38.4-weeks’ gestation. A large multinodular abdominal mass suspicious for central necrosis and infected tissue was noted along her right lower quadrant. After successful vaginal delivery, computed tomography of the abdomen and pelvis demonstrated an enhancing multilobulated mass involving the skin and soft tissue, measuring 10.0 cm by 5.4 cm (image A, arrows). One week postpartum, the mass was surgically excised via wide local excision. 
The histopathologic examination revealed a spindle-cell neoplasm showing a storiform type arrangement of the spindle cells (image B, circle; hematoxylin-eosin, original magnification × 100), with mild nuclear pleomorphism, invasion of the subcutaneous fat, and positive immunohistochemistry for vimentin and CD34 (not shown). These findings revealed a rare soft tissue sarcoma known as dermatofibrosarcoma protuberans, which has an estimated incidence of 0.8 to 4.5 cases per million per year.1 These tumors comprise roughly 6% of all soft tissue sarcomas.2 They are superficial, slow-growing, and have a low malignant potential, with roughly 85% to 90% being low grade with a relatively good prognosis. 3 Dermatofibrosarcoma protuberans has a high frequency of local regional recurrence, with rates ranging from 26% to 60%.4 
References
Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. 2007;56(6):968-973. doi 10.1016/j.jaad.2006.09.006 [CrossRef] [PubMed]
WHO classification of tumours of soft tissue and bone. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. World Health Organization Classification of Tumours. 4th ed. Vol 5. International Agency for Research on Cancer; 2013.
Angouridakis N, Kafas P, Jerjes W, et al. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck. Head Neck Oncol. 2011;3:5. doi: 10.1186/1758-3284-3-5
Li Y, Wang C, Xiang B, Chen S, Li L, Ji Y. Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer. . 2017;8(7):1319-1323. doi: 10.7150/jca.17988 2 [CrossRef] [PubMed]