Dermatofibrosarcoma Protuberans

Abstract

MR imaging is useful in identifying the extent and location of dermatofibrosarcoma protuberans. Although most cases of this tumor are superficial and well defined, we have shown three cases in which the tumor was in a deep location and one case in which the tumor was ill defined in appearance. Knowledge of the variable MR imaging appearances of these tumors may aid in the diagnosis of difficult or atypical cases.

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

“…On MR imaging, DFSP shows iso-or low-signal intensity on T1-weighted images and intermediate or high-signal intensity greater than that of the subcutaneous fat on T2-weighted or fast spin-echo T2-weighted images (4,6,7). DFSP shows high-signal intensity, similar to that of water or blood vessels on fat-suppressed images (2,7). Enhancement is generally homogeneous, but it can vary due to intratumoral necrosis or hemorrhage (2, 7).…”

“…DFSP can be found anywhere on the body, but is often found on the chest and shoulders (1). DFSP arises in the dermis and invades deeper tissues such as fascia, muscle and bone (1,2). Due to its tendency for local invasion and asymmetric spread, DFSP in the perianal area can extend to the anal sphincter and show similar imaging findings to those of a malignant tumor arising from the anorectal fistula.…”

A Fibrosarcomatous ("High-Grade") Variant of Dermatofibrosarcoma Protuberans (DFSP

“…Dermatofibrosarcoma protuberance most commonly involves the trunk, accounting for about half of all cases. The extremities, followed by the head and neck, are the next most common sites, but tumors may occur on any part of the body [3]. DFSP was first described in 1924 by Darier and Ferrand as "progressive and recurring dermatofibroma".…”

“…DFSP is a locally aggressive tumor with a high recurrence rate, but it rarely metastasize [5]. Patients often ignore these tumors because of their slow growth so they are usually left untreated for several months [3]. There is usually little nuclear pleomorphism and only low to moderate mitotic activity.…”

Middle-aged female with palpable swelling over the abdominal wall

“…MRI is recommended to evaluate local extension and preoperative planning of larger, atypical and recurrent tumors [1,4]. CT can be used for evaluation if bony involvement is suspected, while USG can be used for detection of smaller lesions and for guided biopsies [4].…”