Granulocytic sarcoma of mesentery in acute myeloid leukemia with CBFB/MYH11 fusion gene but not inv(16) chromosome: case report and review of literature.
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.
Fujieda
Atsushi
A
Division of Hematology and Oncology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan.
Nishii
Kazuhiro
K
Tamaru
Tomoki
T
Otsuki
Shoichiro
S
Kobayashi
Kazuhiko
K
Monma
Fumihiko
F
Ohishi
Kohshi
K
Nakase
Kazunori
K
Katayama
Naoyuki
N
Shiku
Hiroshi
H
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
C095514:CBFbeta-MYH11 fusion protein; D015514:Oncogene Proteins, Fusion