Dermatofibrosarcoma Protuberans: Insights into a Rare Soft Tissue Tumor

Barwani, Aliya Sarhan Al; Taif, Sawsan; Mazrouai, Reem Ahmed Al; Muzahmi, Khamis Salim Al; Alrawi, Asif

doi:10.4103/2156-7514.181492

Cited by 27 publications

(25 citation statements)

References 22 publications

(36 reference statements)

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“…The median age of presentation is 38.5 years with roughly equal distribution between males and females [ 4 ]. The trunk and extremities are considered the most common sites for DFSP; nevertheless, it has been described in various parts of the body including the neck, head, breast and even vulva [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…The lesions show little nuclear pleomorphism and have low to moderate mitotic activity. They occasionally show myxoid or densely collagenous areas or areas of haemorrhage [ 3 ]. Three-dimensional reconstruction of DFSP has shown tumours with highly irregular shapes and frequent finger-like extensions.…”

Section: Discussionmentioning

confidence: 99%

“…It grows slowly and presents usually as a nodular superficial lesion on the trunk or the extremities. Although these tumours are locally aggressive, with a high rate of recurrence following surgery, the prognosis is considered excellent when it is effectively treated [ 3 ]. In this case report, we discuss a case of recurrent DFSP of the breast after a latency of 8 years and its surgical management in detail.…”

Section: Introductionmentioning

confidence: 99%

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

Diwakar¹,

Wadhwani²,

Paruthi³

2018

ecancer

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Diwakar¹,

Wadhwani²,

Paruthi³

2018

ecancer

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“…Often, patients have the tumor steady or slow-growing for a period of time, which varies from months to years. It brings them to the clinic only after increasing growth rate, suffering from pain, or complications like ulceration or hemorrhage of the lesion [12]. US study of a DFSP depicts a subcutaneous oval or round mass adjacent to the skin.…”

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans; Case Report of a Rare Tumor of the Breast

Hoseinpour

Olfatbakhsh

Keshavarz-Fathi

et al. 2016

mcij

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Introduction: Dermatofibrosarcoma protuberans (DFSP) is an intermediate and slowgrowing sarcoma, developing most commonly in the trunk and extremities and rarely in the breast. It may be recurrent, but metastasis is not common. Excisional surgery with enough resection margins decreases the recurrence rate much more. Case Presentation: An 18-year-old woman presented with posttraumatic bruising of the right axilla near her breast which had been converted to a nodular mass. A well-defined firm mass, fixed to the dermis, palpable with mild tenderness, was present. Ultrasound (US) revealed a cyst with thickened wall, internal echo septa and high vascularity. The findings were suggestive of a complex cyst or infected epidermal inclusion cyst (EIC). Pathological investigation revealed uniform spindle cells in a storiform arrangement. In immunohistochemistry (IHC), tumoral cells showed reactivity for CD34 and the diagnosis of DFSP was made. Conclusion: Various breast lesions including cysts as well as benign and malignant masses may show similar appearance in imaging studies, which makes clear the crucial role of the pathological and immunohistochemical investigations to confirm a definite diagnosis.

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“…However, other subtypes are also known: atrophic, fibrosarcomatous, granular, myxoid, pigmented (Bednar tumor), and sclerosing. 3 …”

Section: Introductionmentioning

confidence: 99%

An unusual variant of atrophic dermatofibrosarcoma protuberans

Aragão

Cardoso

Leite

et al. 2018

An. Bras. Dermatol.

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Dermatofibrosarcoma protuberans is an uncommon neoplasm that is most often seen in young adults. The most common clinical presentation is the protruding form; however, other subtypes are known, such as the atrophic. In 2012 there were only 33 reports of this variant in the literature. Many cases of Dermatofibrosarcoma protuberans in children are only discovered in adulthood because they were not diagnosed early. Due the high morbidity, we raise the need for attention from the dermatologist to recognize uncommon neoplasms in the clinical practice. We report a case of a 15-year-old patient diagnosed with atrophic Dermatofibrosarcoma protuberans on the back.

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Dermatofibrosarcoma Protuberans: Insights into a Rare Soft Tissue Tumor

Cited by 27 publications

References 22 publications

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

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

Dermatofibrosarcoma Protuberans; Case Report of a Rare Tumor of the Breast

An unusual variant of atrophic dermatofibrosarcoma protuberans

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