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Pigmented dermatofibrosarcoma protuberans (Bednár tumor). An autopsy case with systemic metastasis
N Onoda1, Y Tsutsumi, K Kakudo
1Department of Pathology, Tokai University School of Medicine, Isehara, Japan.
Acta Pathologica Japonica
|December 1, 1990
Summary
This case report details a rare pigmented dermatofibrosarcoma protuberans (Bednár tumor) that metastasized widely. The study highlights the aggressive potential and changing morphology of this unusual sarcoma.
Area of Science:
- Oncology
- Dermatopathology
- Surgical Pathology
Background:
- Pigmented dermatofibrosarcoma protuberans, also known as Bednár tumor, is a rare cutaneous neoplasm.
- Systemic metastasis of this tumor is exceptionally uncommon, with limited documented cases.
Observation:
- A 45-year-old male presented with a pigmented dermatofibrosarcoma protuberans on his right upper arm.
- The tumor recurred twice over an 8-year period, eventually metastasizing to the skin, lungs, and brain.
Findings:
- Autopsy revealed widespread metastatic lesions in multiple organs including the skin, lungs, brain, thyroid, pancreas, stomach, small intestine, and thigh muscles.
- The primary tumor exhibited melanin-laden cells and a storiform pattern, while metastatic lesions lacked melanin and showed sarcomatous changes with a herring-bone pattern.
Implications:
- This case underscores the potential for aggressive behavior and widespread metastasis in pigmented dermatofibrosarcoma protuberans.
- The observed morphological changes in recurrent and metastatic tumors suggest dedifferentiation and highlight the importance of thorough histopathological examination.