School of Medicine and Health Sciences Poster Presentations

Title

The Rarest of Rare: Unlikely Presentation of Dermatofibrosarcoma Protuberans

Document Type

Poster

Keywords

Dermatofibrosarcoma Protuberans; Soft Tissue Sarcoma; Recurrence

Publication Date

Spring 2017

Abstract

A 31-year-old female presented to the emergency department following witnessed syncope. At the time of admission, she reported that she had a “small mass” on the left thigh that was previously excised in 2006 only to return two years later. Over the last three to four weeks, she noted rapid expansion, with associated discomfort, bleeding, and even functional impairment due to its size. On exam, the patient was febrile to 101F, sinus tachycardia to the 120s, and hypotensive 90/55 mmHg. Initial labs were noteworthy for leukocytosis of 15k as well as low hemoglobin and hematocrit of 7.2 g/dL and 24 respectively. The physical exam highlighted a 13cm x 16cm x 7cm, necrotic, ulcerating mass on the left hip. MRI imaging revealed a 13 cm x 13 cm heterogeneously enhancing mass along the left hip and thigh, indicating rapid progression from the 7.5 cm x 6 cm mass seen on a 2015 MRI. Management for acute blood loss included crystalloid and colloid products and initiation of broad spectrum antibiotic for a superimposed soft tissue infection. Histopathology highlighted a storiform pattern of monotonous, slender highly atypical, spindle shaped cells with focal regions of fibrosarcomatous change that demonstrated a fascicular, herringbone pattern of growth, consistent with Dermatofibrosarcoma protuberans. The patient underwent wide resection and flap reconstruction without adjunctive radio or chemotherapy per her preference.

Dermatofibrosarcoma protuberans is a rare soft tissue tumor that arises from the dermis and accounts for approximately 2 percent of all soft tissue sarcomas and less than 0.1 percent of all malignancies. The infiltrative soft tissue tumor is locally aggressive and has an increased probability to recur or metastasize if not resected with disease free margins. While rare to be evaluated and diagnosed in the outpatient setting, it is even more uncommon to have syncope as the presenting complaint for a soft tissue tumor. At the time of presentation, the size, associated functional impairment and resulting hemodynamic instability required immediate medical intervention and subsequent surgical treatment, highlighting this case as an extreme example of this tumor’s aggressive nature.

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Poster to be presented at GW Annual Research Days 2017.

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The Rarest of Rare: Unlikely Presentation of Dermatofibrosarcoma Protuberans

A 31-year-old female presented to the emergency department following witnessed syncope. At the time of admission, she reported that she had a “small mass” on the left thigh that was previously excised in 2006 only to return two years later. Over the last three to four weeks, she noted rapid expansion, with associated discomfort, bleeding, and even functional impairment due to its size. On exam, the patient was febrile to 101F, sinus tachycardia to the 120s, and hypotensive 90/55 mmHg. Initial labs were noteworthy for leukocytosis of 15k as well as low hemoglobin and hematocrit of 7.2 g/dL and 24 respectively. The physical exam highlighted a 13cm x 16cm x 7cm, necrotic, ulcerating mass on the left hip. MRI imaging revealed a 13 cm x 13 cm heterogeneously enhancing mass along the left hip and thigh, indicating rapid progression from the 7.5 cm x 6 cm mass seen on a 2015 MRI. Management for acute blood loss included crystalloid and colloid products and initiation of broad spectrum antibiotic for a superimposed soft tissue infection. Histopathology highlighted a storiform pattern of monotonous, slender highly atypical, spindle shaped cells with focal regions of fibrosarcomatous change that demonstrated a fascicular, herringbone pattern of growth, consistent with Dermatofibrosarcoma protuberans. The patient underwent wide resection and flap reconstruction without adjunctive radio or chemotherapy per her preference.

Dermatofibrosarcoma protuberans is a rare soft tissue tumor that arises from the dermis and accounts for approximately 2 percent of all soft tissue sarcomas and less than 0.1 percent of all malignancies. The infiltrative soft tissue tumor is locally aggressive and has an increased probability to recur or metastasize if not resected with disease free margins. While rare to be evaluated and diagnosed in the outpatient setting, it is even more uncommon to have syncope as the presenting complaint for a soft tissue tumor. At the time of presentation, the size, associated functional impairment and resulting hemodynamic instability required immediate medical intervention and subsequent surgical treatment, highlighting this case as an extreme example of this tumor’s aggressive nature.