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.

DI VEROLI, A., Micarelli, A., Cefalo, M., Ceresoli, E., Nasso, D., Cicconi, L., et al. (2013). 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. CASE REPORTS IN HEMATOLOGY, 1-5 [10.1155/2013/245395].

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;CEFALO, MARIAGIOVANNA;OTTAVIANI, FABRIZIO;VENDITTI, ADRIANO;AMADORI, SERGIO
2013-09-13

Abstract

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.
13-set-2013
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/31 - OTORINOLARINGOIATRIA
Settore MED/15 - MALATTIE DEL SANGUE
English
Con Impact Factor ISI
DI VEROLI, A., Micarelli, A., Cefalo, M., Ceresoli, E., Nasso, D., Cicconi, L., et al. (2013). 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. CASE REPORTS IN HEMATOLOGY, 1-5 [10.1155/2013/245395].
DI VEROLI, A; Micarelli, A; Cefalo, M; Ceresoli, E; Nasso, D; Cicconi, L; Mauramati, S; Ottaviani, F; Venditti, A; Amadori, S
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/79889
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