Advanced Dermatofibrosarcoma Protuberans: an Updated Analysis of Cases From an Indian Sarcoma Clinic

Figures

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  Figure 1.. Histological picture from a case of dermatofibrosarcoma protuberans showing cells arranged in a storiform pattern. (A) High-power picture showing spindle cells that are showing mild nuclear atypia with elongated nuclei, finely dispersed chromatin and a moderate amount of eosinophilic cytoplasm. There is no mitosis and the overall mitotic activity is less than two per ten HPF (H&E ×100). (B) Histological picture from a case of fibrosarcomatous DFSP showing cells arranged in interlacing fascicles in a herringbone pattern. The individual tumor cells exhibit moderate nuclear atypia, a high nucleo-cytoplasmic ratio, hyperchromatic nuclei and scanty cytoplasm. Mitotic activity of around 8–10/10 HPF was noted (H&E ×200). (C) Immunostaining for CD34 showing diffuse loss of its expression in the spindle cell population in a case of fibrosarcomatous DFSP (H&E ×200). DFSP: Dermatofibrosarcoma protuberans; H&E: Hematoxylin and eosin staining; HPF: High-power field.

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  Figure 2.. Contrast-enhanced computerized tomography images of patient 6, with dermatofibrosarcoma protuberans with fibrosarcomatous transformation lesions over the chest wall, in the axillary soft-tissue and in the lungs. After progressing on imatinib (top panel: June 2017), his disease has remained stable on pazopanib for 42 months (bottom panel: June 2020).

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  Figure 3.. Contrast-enhanced computerized tomography images of patient 5, with a paraspinal mass and lung metastases, who continues to be progression free on imatinib at 53 months of therapy. Top image from July 2016 and bottom image from December 2019.

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  Figure 4.. Kaplan–Meier curve for progression-free survival. (A) Overall, for all cases combined. (B) For patients with locally advanced versus metastatic disease. (C) For patients with DFSP versus DFSP-FS. DFSP: Dermatofibrosarcoma protuberans; DFSP-FS: Dermatofibrosarcoma protuberans with fibrosarcomatous transformation; PFS: Progression-free survival.