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[Dermatofibrosarcoma protuberans developing for 25 years with 27 recurrences. Histological and ultrastructural study]

N Mandalenakis, L Venne, J G Paquin

    Annales D'Anatomie Pathologique
    |January 1, 1980
    PubMed
    Summary

    This study details a rare, aggressive protuberant dermatofibrosarcoma with 27 relapses over 25 years. Ultrastructure reveals myofibroblastic and histiocytic features, aiding in understanding this challenging skin cancer.

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    Area of Science:

    • Dermatopathology
    • Oncology
    • Surgical Pathology

    Background:

    • Protuberant dermatofibrosarcoma is a rare skin neoplasm.
    • Understanding its histological and ultrastructural characteristics is crucial for diagnosis and management.

    Observation:

    • A case of protuberant dermatofibrosarcoma with a 25-year history and 27 relapses was studied.
    • Histology revealed a dermal, non-encapsulated tumor invading the hypodermis, composed of storiform fusiform cells in a myxoid stroma.
    • Later relapses showed increased cellularity, compact zones, and mitoses, with areas of cystic degeneration.

    Findings:

    • Ultrastructural analysis identified tumor cells with developed endoplasmic reticulum, Golgi apparatus, and pinocytic vesicles.
    • Cells exhibited features of myofibroblastic (cytoplasmic filaments, contractile elements) and histiocytic (lysosomes, phagolysosomes) differentiation.

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  • Cellular junctions and amorphous extracellular material were also observed.

    • Implications:

    • The findings support classifying this tumor within myofibroblastic and histiocytic proliferating lesions.
    • This detailed case study provides insights into the aggressive behavior and cellular origins of protuberant dermatofibrosarcoma.
    • Further research into similar aggressive variants may improve therapeutic strategies for this rare sarcoma.