BACKGROUND: Granulocytic sarcomas (GS) have been associated with t(8;21). The prognosis of patients with GS is generally regarded as being less favorable than of patients with acute myeloblastic leukemia (AML). GS occurs relatively commonly in Africa and has been reported to affect 10-25% of black children presenting with AML. We sought to establish the incidence of GS in our pediatric population, to determine whether an association with t(8;21) existed, and to report on the outcome of these cases in a single series. PROCEDURE: The records of consecutive pediatric patients treated for de novo AML in Johannesburg between January 1985-December 1995 were reviewed. Fifteen cases of GS among a total of 88 cases of AML presented to the Paediatric Haematology/Oncology Clinics of the Johannesburg and Baragwanath Hospitals. Fourteen (93%) of these patients were black male children. RESULTS: All 9 cases of orbital GS (60%) and almost all cases with concurrent AML M2 had t(8;21). This translocation was present in only 4 n(8.5%) of the remaining 47 AML cases without GS for which cytogenetic data were available. One case presented with a complex chromosomal translocation not previously associated with GS. The median disease-free survival of the GS patients, using conventional chemotherapy treatment protocols, was significantly better than for the patients with AML and no GS (P = 0.0004). CONCLUSIONS: Our data support a strong association between orbital GS, t(8;21), and AML M2 in the pediatric population. This entity occurred virtually exclusively in black male children at presentation. One third of these children who presented with AML had a GS. The favorable prognosis noted in our GS patients on standard induction and intensification therapy without local irradiation conflicts with some previous reports but is consistent with the favorable outcome documented in AML with t(8;21).
BACKGROUND:Granulocytic sarcomas (GS) have been associated with t(8;21). The prognosis of patients with GS is generally regarded as being less favorable than of patients with acute myeloblastic leukemia (AML). GS occurs relatively commonly in Africa and has been reported to affect 10-25% of black children presenting with AML. We sought to establish the incidence of GS in our pediatric population, to determine whether an association with t(8;21) existed, and to report on the outcome of these cases in a single series. PROCEDURE: The records of consecutive pediatric patients treated for de novo AML in Johannesburg between January 1985-December 1995 were reviewed. Fifteen cases of GS among a total of 88 cases of AML presented to the Paediatric Haematology/Oncology Clinics of the Johannesburg and Baragwanath Hospitals. Fourteen (93%) of these patients were black male children. RESULTS: All 9 cases of orbital GS (60%) and almost all cases with concurrent AML M2 had t(8;21). This translocation was present in only 4 n(8.5%) of the remaining 47 AML cases without GS for which cytogenetic data were available. One case presented with a complex chromosomal translocation not previously associated with GS. The median disease-free survival of the GSpatients, using conventional chemotherapy treatment protocols, was significantly better than for the patients with AML and no GS (P = 0.0004). CONCLUSIONS: Our data support a strong association between orbital GS, t(8;21), and AML M2 in the pediatric population. This entity occurred virtually exclusively in black male children at presentation. One third of these children who presented with AML had a GS. The favorable prognosis noted in our GSpatients on standard induction and intensification therapy without local irradiation conflicts with some previous reports but is consistent with the favorable outcome documented in AML with t(8;21).
Authors: Donna L Johnston; Todd A Alonzo; Robert B Gerbing; Beverly J Lange; William G Woods Journal: Pediatr Blood Cancer Date: 2011-05-25 Impact factor: 3.167
Authors: Jan-Niklas Eckardt; Friedrich Stölzel; Desiree Kunadt; Christoph Röllig; Sebastian Stasik; Lisa Wagenführ; Korinna Jöhrens; Friederike Kuithan; Alwin Krämer; Sebastian Scholl; Andreas Hochhaus; Martina Crysandt; Tim H Brümmendorf; Ralph Naumann; Björn Steffen; Volker Kunzmann; Hermann Einsele; Markus Schaich; Andreas Burchert; Andreas Neubauer; Kerstin Schäfer-Eckart; Christoph Schliemann; Stefan W Krause; Regina Herbst; Mathias Hänel; Maher Hanoun; Ulrich Kaiser; Martin Kaufmann; Zdenek Rácil; Jiri Mayer; Frank Kroschinsky; Wolfgang E Berdel; Gerhard Ehninger; Hubert Serve; Carsten Müller-Tidow; Uwe Platzbecker; Claudia D Baldus; Johannes Schetelig; Martin Bornhäuser; Christian Thiede; Jan Moritz Middeke Journal: J Hematol Oncol Date: 2022-05-13 Impact factor: 23.168