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Myeloid Sarcoma With CBFB-MYH11 Fusion Prevails in the Abdomen: A Clinicopathologic Analysis of 11 Cases and Review of the Literature

Objectives: Myeloid sarcoma with CBFB-MYH11 fusion may be enriched in abdominal sites. The clinicopathologic features of 11 cases are reported. Methods: We collected clinical features, morphology, immunophenotype, and bone marrow (BM) involvement of myeloid sarcoma cases with CBFB-MYH11 fusion. Resu...

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Published in:American journal of clinical pathology 2020-03, Vol.153 (3), p.333
Main Authors: Dalland, Joanna C, Meyer, Reid, Ketterling, Rhett P, Reichard, Kaaren K
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Language:English
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container_title American journal of clinical pathology
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creator Dalland, Joanna C
Meyer, Reid
Ketterling, Rhett P
Reichard, Kaaren K
description Objectives: Myeloid sarcoma with CBFB-MYH11 fusion may be enriched in abdominal sites. The clinicopathologic features of 11 cases are reported. Methods: We collected clinical features, morphology, immunophenotype, and bone marrow (BM) involvement of myeloid sarcoma cases with CBFB-MYH11 fusion. Results: Eleven of 29 total myeloid sarcoma cases were CBFB-MYH11 positive and all 11 involved abdominal sites. The blastic infiltrate was associated with eosinophils in four of 11 cases and plasmacytoid dendritic cell (pDC) nodules in four of six cases. CD34, CD117, and myeloperoxidase were expressed in eight of nine, 10 of 10, and 10 of 10 cases, respectively. Ten of 10 cases showed no BM involvement. Conclusions: Our current series, combined with a literature review, identifies a compelling series of 31 (94%) of 33 cases of myeloid sarcoma with CBFB-MYH11 fusion showing a marked predilection for abdominal sites. In addition, the lack of obvious associated eosinophils, presence of pDC nodules, and lack of concurrent BM involvement suggest that "myeloid sarcoma with CBFB-MYH11 fusion" may represent a unique phenomenon. Key Words: Myeloid sarcoma; Chloroma; M4; M4eo; Acute myeloid leukemia; Core binding factor
doi_str_mv 10.1093/AJCP/AQZ168
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The clinicopathologic features of 11 cases are reported. Methods: We collected clinical features, morphology, immunophenotype, and bone marrow (BM) involvement of myeloid sarcoma cases with CBFB-MYH11 fusion. Results: Eleven of 29 total myeloid sarcoma cases were CBFB-MYH11 positive and all 11 involved abdominal sites. The blastic infiltrate was associated with eosinophils in four of 11 cases and plasmacytoid dendritic cell (pDC) nodules in four of six cases. CD34, CD117, and myeloperoxidase were expressed in eight of nine, 10 of 10, and 10 of 10 cases, respectively. Ten of 10 cases showed no BM involvement. Conclusions: Our current series, combined with a literature review, identifies a compelling series of 31 (94%) of 33 cases of myeloid sarcoma with CBFB-MYH11 fusion showing a marked predilection for abdominal sites. In addition, the lack of obvious associated eosinophils, presence of pDC nodules, and lack of concurrent BM involvement suggest that "myeloid sarcoma with CBFB-MYH11 fusion" may represent a unique phenomenon. 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The clinicopathologic features of 11 cases are reported. Methods: We collected clinical features, morphology, immunophenotype, and bone marrow (BM) involvement of myeloid sarcoma cases with CBFB-MYH11 fusion. Results: Eleven of 29 total myeloid sarcoma cases were CBFB-MYH11 positive and all 11 involved abdominal sites. The blastic infiltrate was associated with eosinophils in four of 11 cases and plasmacytoid dendritic cell (pDC) nodules in four of six cases. CD34, CD117, and myeloperoxidase were expressed in eight of nine, 10 of 10, and 10 of 10 cases, respectively. Ten of 10 cases showed no BM involvement. Conclusions: Our current series, combined with a literature review, identifies a compelling series of 31 (94%) of 33 cases of myeloid sarcoma with CBFB-MYH11 fusion showing a marked predilection for abdominal sites. In addition, the lack of obvious associated eosinophils, presence of pDC nodules, and lack of concurrent BM involvement suggest that "myeloid sarcoma with CBFB-MYH11 fusion" may represent a unique phenomenon. 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The clinicopathologic features of 11 cases are reported. Methods: We collected clinical features, morphology, immunophenotype, and bone marrow (BM) involvement of myeloid sarcoma cases with CBFB-MYH11 fusion. Results: Eleven of 29 total myeloid sarcoma cases were CBFB-MYH11 positive and all 11 involved abdominal sites. The blastic infiltrate was associated with eosinophils in four of 11 cases and plasmacytoid dendritic cell (pDC) nodules in four of six cases. CD34, CD117, and myeloperoxidase were expressed in eight of nine, 10 of 10, and 10 of 10 cases, respectively. Ten of 10 cases showed no BM involvement. Conclusions: Our current series, combined with a literature review, identifies a compelling series of 31 (94%) of 33 cases of myeloid sarcoma with CBFB-MYH11 fusion showing a marked predilection for abdominal sites. In addition, the lack of obvious associated eosinophils, presence of pDC nodules, and lack of concurrent BM involvement suggest that "myeloid sarcoma with CBFB-MYH11 fusion" may represent a unique phenomenon. Key Words: Myeloid sarcoma; Chloroma; M4; M4eo; Acute myeloid leukemia; Core binding factor</abstract><pub>Oxford University Press</pub><doi>10.1093/AJCP/AQZ168</doi></addata></record>
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subjects Bone marrow cells
Diagnosis
Genetic aspects
Health aspects
Myosin
Sarcoma
Testing
Transcription factors
title Myeloid Sarcoma With CBFB-MYH11 Fusion Prevails in the Abdomen: A Clinicopathologic Analysis of 11 Cases and Review of the Literature
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