Isolated Myeloid Sarcoma of the Vulva

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Myeloid sarcoma can involve any anatomic site, but involvement of the gynecologic tract is uncommon. In particular, vulvar myeloid sarcoma is extremely rare, with only 1 case report in the English-language medical literature. A 21-year-old, normally developed Japanese woman presented with a hard, gradually growing mass that involved the patient's right labia majora and whole right vulva. The mass was resected and histologically diagnosed as vulvar myeloid sarcoma, poorly differentiated type on the basis of the immunohistochemical studies. She had no history or simultaneous involvement of any other myeloid neoplasm. The patient had a complete remission (CR) after induction chemotherapy with cytarabine and daunorubicin, and she received another course of the same combination chemotherapy as a consolidation therapy and an allogeneic hematopoietic stem cell transplantation from an human leukocyte antigen-matched related donor. But a first medullar relapse of acute myeloid leukemia (AML) developed, and she needed salvage chemotherapy with daunomycin and cytarabine. She achieved a second CR after the salvage therapy and received a second allogeneic hematopoietic stem cell transplantation from the same donor. However, AML relapsed again and she received reinduction chemotherapy, which consisted of behenoyl cytarabine and aclarubicin, which resulted in a third remission of AML. Three years after the initial resection, the patient is alive, without any evidence of recurrent extramedullary disease. To the best of our knowledge, this is the second report of the vulvar myeloid sarcoma in the English-language medical literature. The correct diagnosis of myeloid sarcoma particularly on an isolated mass is important so that appropriate therapy can be instituted.

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