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Childhood onset dermatofibrosarcoma protuberans.

B D Sathyanarayana¹

¹Department of Skin &STD, Government Medical College, Mysore, India. belagolasathyanarayana@yahoo.com

Indian Journal of Dermatology, Venereology and Leprology

|July 24, 2007

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Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer. Diagnosis relies on clinical and histopathological features, with wide surgical excision being the preferred treatment to prevent recurrence.

Area of Science:

Dermatology
Oncology
Pathology

Background:

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, locally aggressive dermal tumor.
Its histogenesis is debated, and it has a significant tendency for local recurrence after treatment.

Purpose of the Study:

To present a case of DFSP in a young female.
To highlight the diagnostic challenges and emphasize key features for accurate diagnosis.
To reinforce the recommended treatment approach for DFSP.

Main Methods:

Clinical presentation of a 16-year-old female with multiple lesions.
Dermoscopic and histopathological examination of biopsy specimens.
Analysis of lesion characteristics including size, chronicity, and indolent nature.

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Main Results:

Biopsy revealed a thinned epidermis and dermal infiltration by spindle cells in a storiform pattern.
A characteristic honeycomb pattern was observed due to tumor cells interdigitating with subcutaneous adipocytes.
Histochemical findings were noted as helpful but secondary to clinical and pathological features.

Conclusions:

Accurate diagnosis of DFSP requires consideration of chronicity, size, indolent behavior, and histopathology.
Wide and deep surgical excision is the gold standard treatment for DFSP to minimize recurrence risk.