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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Wiley
2013-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2013/245395 |
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| author | Ambra Di Veroli Alessandro Micarelli Mariagiovanna Cefalo Eleonora Ceresoli Daniela Nasso Laura Cicconi Simone Mauramati Fabrizio Ottaviani Adriano Venditti Sergio Amadori |
| author_facet | Ambra Di Veroli Alessandro Micarelli Mariagiovanna Cefalo Eleonora Ceresoli Daniela Nasso Laura Cicconi Simone Mauramati Fabrizio Ottaviani Adriano Venditti Sergio Amadori |
| author_sort | Ambra Di Veroli |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-1249c1cfe6f340f6b92ca6f01ba5f2f8 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
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| series | Case Reports in Hematology |
| spelling | doaj-art-1249c1cfe6f340f6b92ca6f01ba5f2f82025-02-03T05:59:45ZengWileyCase Reports in Hematology2090-65602090-65792013-01-01201310.1155/2013/245395245395Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic ChallengeAmbra Di Veroli0Alessandro Micarelli1Mariagiovanna Cefalo2Eleonora Ceresoli3Daniela Nasso4Laura Cicconi5Simone Mauramati6Fabrizio Ottaviani7Adriano Venditti8Sergio Amadori9Department of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Otorhinolaryngology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Otorhinolaryngology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Otorhinolaryngology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyDepartment of Hematology, “Tor Vergata” University, Viale Oxford 81, 00133 Rome, ItalyGranulocytic 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.http://dx.doi.org/10.1155/2013/245395 |
| spellingShingle | Ambra Di Veroli Alessandro Micarelli Mariagiovanna Cefalo Eleonora Ceresoli Daniela Nasso Laura Cicconi Simone Mauramati Fabrizio Ottaviani Adriano Venditti Sergio Amadori 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 |
| 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 |
| url | http://dx.doi.org/10.1155/2013/245395 |
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