title: Granulocytic sarcoma of mesentery in acute myeloid leukemia with CBFB/MYH11 fusion gene but not inv(16) chromosome: case report and review of literature.
abstract: Granulocytic sarcoma (GS) is a rare extra-medullary tumor and usually involves the skin, soft tissue, and lymph nodes. GS is found in 8% of acute myeloid leukemia (AML) patients, especially patients with t(8;21)AML. It has been suggested that GS is a poor prognostic factor in t(8;21)AML. Compared to t(8;21)AML, GS is rare in cases of inv(16)AML. Thus, the characteristics of inv(16) with GS are not well understood. Here, we describe a patient with AML and mesentery GS. The chromosomal analysis was normal, but molecular analysis detected the CBFB/MYH11 fusion gene in the blasts. A complete remission was achieved with standard induction therapy followed by high-dose cytarabine consolidation. We have also summarized 12 reported cases of inv(16)AML with GS and found that GS was commonly found in abdominal lesions. These observations suggest that when abdominal GS is diagnosed, an analysis of the CBFB/MYH11 fusion gene is necessary to make an appropriate decision regarding treatment options, even if no chromosomal abnormalities are found.
authors.affiliation: Division of Hematology and Oncology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Fujieda
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Nishii
authors.forename: Tomoki
authors.initials: T
authors.lastname: Tamaru
authors.forename: Shoichiro
authors.initials: S
authors.lastname: Otsuki
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Monma
authors.forename: Kohshi
authors.initials: K
authors.lastname: Ohishi
authors.forename: Kazunori
authors.initials: K
authors.lastname: Nakase
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Katayama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shiku
mesh_terms: D000208:Acute Disease; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence / Q000379:methods; D007951:Leukemia, Myeloid / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D008297:Male; D008643:Mesentery / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000032:analysis / Q000235:genetics*; D012074:Remission Induction; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D023981:Sarcoma, Myeloid / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: C095514:CBFbeta-MYH11 fusion protein; D015514:Oncogene Proteins, Fusion