Recurrent dermatofibrosarcoma protuberans: challenging a surgeon’s dexterity for the ‘tricky’ margins

Abstract

Soft tissue tumours represent 0.2%–1% of all breast malignancies. [Al Tarakji M, Toro A, and Di Carlo I, et al (2015) Unusual presentation of dermatofibrosarcoma protuberans in a male patient’s breast: a case report and review of the literature World J Surg Oncol 13 158 https://doi.org/10.1186/s12957-015-0562-1]. Out of those, Dermatofibrosarcoma protuberans (DFSP) of the breast is extremely rare, especially in men with only six cases, including this case, reported so far. We report a case of recurrent DFSP in a 35-year-old male after a latency of 8 years in the region of previous surgical scar. It was managed by a wide local excision followed by reconstruction using latissimus dorsi flap. It is important to carefully manage recurrent cases because the post-operative margin status is an important determinant of recurrence, and therefore, requires vigilant resection of the tumour without causing extensive morbidity to the patient.

A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia

“…Ipsilateral axillary lymph nodes were inconspicuous. present with fibrosarcomatous progression that is more aggressive and has higher rates of recurrence and metastasis (11).…”

“…The pathogenesis of DFSP is poorly explained. DFSP was observed to occur in pretraumatic areas, including vaccination sites, burn scars, tattoos, surgical scars, and radiotherapy (11). Almost all molecularly characterized cases have been found to have a COL1A1-PDGFB fusion gene.…”

“…Most DFSPs are typically small and superficial and diagnosis may be suspected based on the tumor's clinical appearance and pathologic examination. When found in the breast region, patients usually undergo only breast ultrasound and mammography without the need for magnetic resonance imaging (MRI) (11). Nevertheless, there is a possibility of the in vivo usage of MR spectroscopy (MRS) that gives additional valuable information of a normal cholin resonance peak which combined with other imaging and pathohistological characteristics can be suggestive of the diagnosis of DFSP (18).…”

“…Nevertheless, there is a possibility of the in vivo usage of MR spectroscopy (MRS) that gives additional valuable information of a normal cholin resonance peak which combined with other imaging and pathohistological characteristics can be suggestive of the diagnosis of DFSP (18). Pathological and immunohistochemical examinations are currently the gold standard for diagnosing DFSP (11).…”

“…No impact on survival was found in patients undergoing radiation therapy (21). Imatinib mesylate, a protein tyrosine kinase inhibitor, is used for the treatment of 8 unresectable, recurrent and/or metastatic DFSP in adult patients because it inhibits the overactivity of PDGF receptor in these tumor cells (11). A response rate of approximately 65% has been achieved among DFSP patients treated with imatinib.…”