Dermatofibrosarcoma Protuberans
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Stefano Natali
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
1. CriscioneVD, WeinstockMA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002.J Am Acad Dermatol.2007;56(6):968-973. doi10.1016/j.jaad.2006.09.006Search in Google Scholar PubMed
2. WHO classification of tumours of soft tissue and bone. In: FletcherCDM, BridgeJA, HogendoornPCW, MertensF, eds. World Health Organization Classification of Tumours. 4th ed. Vol 5. International Agency for Research on Cancer; 2013.Search in Google Scholar
3. AngouridakisN, KafasP, JerjesW, et al.. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck. Head Neck Oncol. 2011;3:5. doi:10.1186/1758-3284-3-5Search in Google Scholar PubMed PubMed Central
4. LiY, WangC, XiangB, ChenS, LiL, JiY. Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer.2017;8(7):1319-1323. doi:10.7150/jca.17988 2Search in Google Scholar PubMed PubMed Central
© 2020 American Osteopathic Association
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Articles in the same Issue
- LETTERS TO THE EDITOR
- Transitions in Undergraduate Medical Education
- ORIGINAL CONTRIBUTION
- Osteopathic Manipulative Medicine Practice Patterns of Third-Year and Fourth-Year Osteopathic Medical Students: An Educational Research Project
- Does the Halo Effect for Level 1 Trauma Centers Apply to High-Acuity Nonsurgical Admissions?
- Weekly Osteopathic Manipulative Treatment to Improve Measures of Sympathetic Tone in Women With Polycystic Ovary Syndrome: A Randomized, Controlled Pilot Study
- Regular Mindful Yoga Practice as a Method to Improve Androgen Levels in Women With Polycystic Ovary Syndrome: A Randomized, Controlled Trial
- BRIEF REPORT
- Effect of Abdominal Lymphatic Pump Treatment on Disease Activity in a Rat Model of Inflammatory Bowel Disease
- CLINICAL REVIEW
- Antithrombotic Therapy for Patients With Atrial Fibrillation and Acute Coronary Syndrome or Percutaneous Coronary Intervention
- REVIEW
- Rhinitis: The Osteopathic Modular Approach
- CASE REPORT
- Psychogenic Adipsia Presenting as Recurrent Functional Vomiting and Hypernatremia
- CLINICAL IMAGES
- Dermatofibrosarcoma Protuberans