J Korean Soc Radiol.  2014 Jun;70(6):435-438. 10.3348/jksr.2014.70.6.435.

CT and MR Imaging Findings of Perianal Dermatofibrosarcoma Protuberans Mimicking Mucinous Adenocarcinoma Arising from Fistula in Ano: A Case Report

• Hwang Y
• Lee SL
• Chang ED
• Ahn CH
• Ku YM

• KMID: 1941772
• DOI: http://doi.org/10.3348/jksr.2014.70.6.435

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm which typically originates in the dermis and spreads into the subcutaneous layer and muscle. We report an extremely rare case of a perianal DFSP mimicking mucinous adenocarcinoma arising from anorectal fistula in a 59-year-old woman. In case of a perianal mass, which is connected by a stalk to the anus and shows heterogeneous enhancement without related regional lymphadenopathy, DFSP should be included in the differential diagnosis.

MeSH Terms

Adenocarcinoma, Mucinous*
Anal Canal
Dermatofibrosarcoma*
Dermis
Diagnosis, Differential
Female
Fistula*
Humans
Lymphatic Diseases
Magnetic Resonance Imaging*
Middle Aged
Soft Tissue Neoplasms

Figure

• 

  Fig. 1 A 59-year-old woman with perianal dermatofibrosarcoma protuberans (DFSP). A. Axial enhanced CT image shows a heterogenously enhancing mass (long arrow) in the medial aspect of the subcutaneous layer of the left gluteal region and with a communicating stalk (short arrows) between the mass and the anus, measuring 5.8 cm in size. B, C. The mass (arrows) shows bright signal intensity on fat-suppressed, T2-weighted imaging (B) and iso-signal intensity to muscle on T1-weighted imaging (C). D. Contrast-enhanced, fat-suppressed, T1-weighted MR images reveal heterogenous enhancement of the mass (long arrow) and the connecting stalk (short arrows). E. Photomicrograph shows interwoven fascicles of cells forming a storiform pattern (H&E, × 100). F. Immunohistochemical staining is strongly positive for CD34. These findings have been considered characteristic for the diagnosis of DFSP (CD34 DAB, × 400).

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