Fournier's Gangrene is known to be one of the rarest and potentially lethal necrotizing infections of the perineum and genital region with high mortality. We report an unusual case of a 34-year-old male presenting to Saint Antoine Hospital in June 2020 after 6 months of severe pain, a mass in the anal and perineal area with blackish discoloration, and painful swelling of the scrotum and the penis. A likely diagnosis of necrotizing fasciitis associated with high-grade fever and sepsis was made. An immediate surgical debridement, colostomy, and suprapubic catheter were done, along with broad-spectrum antibiotics. Per medical history, in 1999, he had an amputation of the left foot after diagnosis with osteosarcoma and lung metastasis, followed by chemotherapy. A new evaluation was done in 2003 and showed progressive lung lesions treated with right lobectomy. In January 2010, the patient presented a blood test with pancytopenia and 14% of circulating blasts. Bone marrow aspiration (BMA) was done and showed an AML secondary to chemotherapy (t-AML) with complex karyotype of chromosome 5 and chromosome 17. Induction chemotherapy with 4 cycles of Vidaza + Revlimid from January 2010 to April 2010 led to partial response. In May 2010, bone marrow biopsy showed signs of dysmyelopoiesis with young elements of a dispersed blastic appearance of myeloid origin without excess blasts and no signs of osteosarcoma. In June 2010, the patient had a successful haploidentical allotransplant from his brother. After presenting with Fournier's Gangrene in June 2020, his blood tests showed anemia (Hb 8.6g/dL), thrombocytopenia (127g/L), and blast cells (12%). Biopsies of the genital mass showed diffuse proliferation of malignant cells, and immunohistochemistry tests came back as CD34+, CD117+, MPO-, Glycophorine +/-, CD68-/+, ACL-/+, CD163-, AE1/AE3-, Ki67%, and EBER-. BMA showed a rich marrow invaded by 50% of blasts with a cytologic aspect of AML. After controlling the Fournier's Gangrene by surgery and antibiotics, the patient was started on a new line of treatment with Vidaza/Venetoclax (July 2020). A new BMA done in September showed a complete biologic response with 1% blasts. The decision was made to continue 4 cycles of chemotherapy. The recovery phase was uneventful with satisfactory cosmetic results.
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