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. 2016 May 28;26(3):e1711.
doi: 10.5812/ijp.1711. eCollection 2016 Jun.

Extramedullary Relapse of Acute Myeloid and Lymphoid Leukemia in Children: A Retrospective Analysis

Jee Young Kim  1 , Soo Ah Im  1 , Ju Hyun Lee  1 , Jae Wook Lee  2 , Nak Gyun Chung  2 , Bin Cho  2
Affiliations

Extramedullary Relapse of Acute Myeloid and Lymphoid Leukemia in Children: A Retrospective Analysis

Jee Young Kim et al. Iran J Pediatr. .

Abstract

Background: Extramedullary relapse (EMR) is a recurrence of leukemia in sites other than the bone marrow, and it exhibits a relatively rare presentation of relapse of acute leukemia. However, EMR is an important cause of treatment failure among patients with acute leukemia. Therefore, early detection of these relapses may improve the prognosis.

Objectives: To describe the disease-related demographic and clinical features and radiologic findings for children diagnosed with EMR in acute leukemia.

Patients and methods: The study was based on 22 children (M: F = 14: 8; mean age 7.30 (2.1 - 15.7) years) with 8 acute myeloid leukemia (AML) and 14 acute lymphoid leukemia (ALL) who had experienced an EMR. Age, gender, clinical symptoms, initial extramedullary disease (EMD), French-American-British (FAB) morphology, cytogenetics, time to and site of EMR, concurrent bone marrow relapse (BMR), radiologic findings, and outcomes were evaluated.

Results: No definite relationship was found between initial EMD and EMR. A predilection for AML to relapse in the central nervous system (CNS), except for the CSF and bone, and for ALL to relapse in the CSF and kidney seemed to occur. Patients with EMR had a significantly higher incidence of t(8: 21) cytogenetics and FAB M2 and L1 morphologies. EMR accompanied with concurrent BMR occurred in 31.8% of the patients, who exhibited a relatively grave clinical course. Radiologic findings were nonspecific and had a great variety of structure involved, including bulging enhancing mass in the CT scan, hypoechoic mass in the US, and enhanced mass-like lesion in the MRI.

Conclusions: Knowledge of the potential sites of EMR, their risk factors, and their clinical and radiologic features may be helpful in the early diagnosis of relapse and planning for therapy.

Keywords: Extramedullary Leukemia; Leukemia; Pediatrics; Relapse.

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Figures

Figure 1.
Figure 1.. MRI at Diagnosis of Extramedullary Leukemia
A, Axial T2 and B, axial T1 showing a round mass in the periphery of the right cerebellum with surrounding edema (arrows). C, Axial T1 and D, coronal T1 with gadolinium reveal a homogenous enhancement of lesion (arrows).
Figure 2.
Figure 2.. MRI at Diagnosis of Extramedullary Leukemia
Axial T1 showing a fusiform enlargement of the left brachial plexus (arrows).
Figure 3.
Figure 3.. MRI at Diagnosis of Extramedullary Leukemia
A, Axial T2 and B, axial T1 showing a large bulging mass on the left mastoid (arrow) and similar lesions involving the skull base and orbits (arrowheads). C, Axial T1 and D, coronal T1 with gadolinium reveal a relatively homogenous enhancement with a central non-enhancing area (arrow, arrowheads).
Figure 4.
Figure 4.. CT at Diagnosis of Extramedullary Leukemia
Enhanced CT showing a hypodense mass of mesentery root and the pancreas head (arrows) (A), and hypodense oval lesions on the right kidney (arrowheads) (B). Abdominal pain occurred with increased serum amylase and lipase level in this patient.
Figure 5.
Figure 5.. US at Diagnosis of Extramedullary Leukemia
Longitudinal scan through the scrotum showing an ill-defined hypoechoic mass posteriorly (arrows) (A) and Doppler sonography showing hypervascular flow signals (B).
See this image and copyright information in PMC

References

    1. Fritz J, Vogel W, Bares R, Horger M. Radiologic spectrum of extramedullary relapse of myelogenous leukemia in adults. AJR Am J Roentgenol. 2007;189(1):209–18. doi: 10.2214/AJR.06.1500. - DOI - PubMed
    1. Yoshihara S, Ikegame K, Kaida K, Taniguchi K, Kato R, Inoue T, et al. Incidence of extramedullary relapse after haploidentical SCT for advanced AML/myelodysplastic syndrome. Bone Marrow Transplant. 2012;47(5):669–76. doi: 10.1038/bmt.2011.163. - DOI - PubMed
    1. Clark WB, Strickland SA, Barrett AJ, Savani BN. Extramedullary relapses after allogeneic stem cell transplantation for acute myeloid leukemia and myelodysplastic syndrome. Haematologica. 2010;95(6):860–3. doi: 10.3324/haematol.2010.025890. - DOI - PMC - PubMed
    1. Vega-Ruiz A, Faderl S, Estrov Z, Pierce S, Cortes J, Kantarjian H, et al. Incidence of extramedullary disease in patients with acute promyelocytic leukemia: a single-institution experience. Int J Hematol. 2009;89(4):489–96. doi: 10.1007/s12185-009-0291-8. - DOI - PMC - PubMed
    1. Freyer DR, Devidas M, La M, Carroll WL, Gaynon PS, Hunger SP, et al. Postrelapse survival in childhood acute lymphoblastic leukemia is independent of initial treatment intensity: a report from the Children's Oncology Group. Blood. 2011;117(11):3010–5. doi: 10.1182/blood-2010-07-294678. - DOI - PMC - PubMed

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