Bilateral adnexal masses: A case report of acute myeloid leukemia presenting with myeloid sarcoma of the ovary and review of literature

Abstract

Here, we report a case of myeloid sarcoma (extramedullary myeloid tumor) of the left ovary in a female with known acute myeloid leukemia (AML), who was discovered to have bilateral adnexal masses during treatment planning and subsequently underwent a total robotic hysterectomy with bilateral salpingo-oophorectomy. Myeloid sarcoma (MS) has been reported in 2–8% of patients with AML and can involve any extramedullary site. MS is most commonly diagnosed simultaneously with AML (Avni and Koren-Michowitz, 2011); however, it can precede or occur after the diagnosis of AML, and may be the sign of transformed/blast crisis of myelodysplastic and/or myeloproliferative neoplasm. In 2008, the World Health Organization (WHO) classified MS as “a tumor mass consisting of myeloid blasts with or without maturation occurring at an anatomic site other than the bone marrow” with an update in 2017 of “myeloid sarcoma” as the terminology used for extramedullary myeloid tumors (Pileri et al.). MS is more common in tumors with mononcytic differentiation. There is no consensus on the treatment of MS thus the mainstay of treatment of MS is AML-type chemotherapeutics. The pathogenesis of female reproductive organ involvement with MS is not fully understood; the most common site for myeloid sarcoma is the skin, subcutaneous tissues, and lymph nodes (Hernández et al., 2002, Wilson and Medeiros, 2015). Neural cell adhesion molecule (NCAM; CD56), which is present in normal ovarian, testicular, and gastrointestinal tissue, is suspected to play a role in the migration of MS to these tissues (Byrd and Weiss, 1994). Additionally, reproductive organs have barriers, which are thought to be sanctuary sites for leukemic cells to proliferate despite systemic therapy such as chemotherapy (Bakst et al., 2011).