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Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge

Granulocytic sarcoma (GS) is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML) as well as following treated AML. The incidence of GS in AML patients is 3–8% but it significantly rises...

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Autores principales: Di Veroli, Ambra, Micarelli, Alessandro, Cefalo, Mariagiovanna, Ceresoli, Eleonora, Nasso, Daniela, Cicconi, Laura, Mauramati, Simone, Ottaviani, Fabrizio, Venditti, Adriano, Amadori, Sergio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787645/
https://www.ncbi.nlm.nih.gov/pubmed/24109526
http://dx.doi.org/10.1155/2013/245395
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author Di Veroli, Ambra
Micarelli, Alessandro
Cefalo, Mariagiovanna
Ceresoli, Eleonora
Nasso, Daniela
Cicconi, Laura
Mauramati, Simone
Ottaviani, Fabrizio
Venditti, Adriano
Amadori, Sergio
author_facet Di Veroli, Ambra
Micarelli, Alessandro
Cefalo, Mariagiovanna
Ceresoli, Eleonora
Nasso, Daniela
Cicconi, Laura
Mauramati, Simone
Ottaviani, Fabrizio
Venditti, Adriano
Amadori, Sergio
author_sort Di Veroli, Ambra
collection PubMed
description Granulocytic sarcoma (GS) is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML) as well as following treated AML. The incidence of GS in AML patients is 3–8% but it significantly rises in M2 FAB subtype AML. This variety of AML harbors t(8;21) in up to 20–25% of cases (especially in children and black ones of African origin) and, at a molecular level, it is characterized by the generation of a fusion gene known as RUNX1-RUNX1T1. Approximately 10% of M2 AML patients will develop GS, as a consequence, the t(8;21) and the relative transcript represent the most common cytogenetic and molecular abnormalities in GS. FLT3-ITD mutation was rarely described in AML patients presenting with GS. FLT3 ITD is generally strongly associated with poor prognosis in AML, and is rarely reported in patients with t(8;21). GS presentation is extremely variable depending on organs involved; in general, cranial bones and sinus are very rarely affected sites. We report a rare case of GS occurring as a recurrence of a previously treated t(8;21), FLT3-ITD positive AML, involving mastoid bones and paravertebral tissues.
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spelling pubmed-37876452013-10-09 Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge Di Veroli, Ambra Micarelli, Alessandro Cefalo, Mariagiovanna Ceresoli, Eleonora Nasso, Daniela Cicconi, Laura Mauramati, Simone Ottaviani, Fabrizio Venditti, Adriano Amadori, Sergio Case Rep Hematol Case Report Granulocytic sarcoma (GS) is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML) as well as following treated AML. The incidence of GS in AML patients is 3–8% but it significantly rises in M2 FAB subtype AML. This variety of AML harbors t(8;21) in up to 20–25% of cases (especially in children and black ones of African origin) and, at a molecular level, it is characterized by the generation of a fusion gene known as RUNX1-RUNX1T1. Approximately 10% of M2 AML patients will develop GS, as a consequence, the t(8;21) and the relative transcript represent the most common cytogenetic and molecular abnormalities in GS. FLT3-ITD mutation was rarely described in AML patients presenting with GS. FLT3 ITD is generally strongly associated with poor prognosis in AML, and is rarely reported in patients with t(8;21). GS presentation is extremely variable depending on organs involved; in general, cranial bones and sinus are very rarely affected sites. We report a rare case of GS occurring as a recurrence of a previously treated t(8;21), FLT3-ITD positive AML, involving mastoid bones and paravertebral tissues. Hindawi Publishing Corporation 2013 2013-09-12 /pmc/articles/PMC3787645/ /pubmed/24109526 http://dx.doi.org/10.1155/2013/245395 Text en Copyright © 2013 Ambra Di Veroli et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Di Veroli, Ambra
Micarelli, Alessandro
Cefalo, Mariagiovanna
Ceresoli, Eleonora
Nasso, Daniela
Cicconi, Laura
Mauramati, Simone
Ottaviani, Fabrizio
Venditti, Adriano
Amadori, Sergio
Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title_full Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title_fullStr Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title_full_unstemmed Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title_short Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge
title_sort recurrence of a t(8;21)-positive acute myeloid leukemia in the form of a granulocytic sarcoma involving cranial bones: a diagnostic and therapeutic challenge
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787645/
https://www.ncbi.nlm.nih.gov/pubmed/24109526
http://dx.doi.org/10.1155/2013/245395
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