Myeloid sarcoma is associated with poor clinical outcome in pediatric patients with acute myeloid leukemia

Xu, Lü-Hong; Wang, Yin; Chen, Zhiyuan; Fang, Jian‐Pei

doi:10.1007/s00432-020-03128-7

Cited by 19 publications

(29 citation statements)

References 33 publications

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“…The best diagnostic approach to recognizing MS is a biopsy of the tumor and immunohistochemical examination. However, the standard immunohistochemical panel in diagnosing MS is lacking which makes it difficult to distinguish MS from other neoplasms, in particular non-Hodgkin lymphomas [14,15]. In our case, the patient was coagulopathic and thrombocytopenic; hence, biopsy was not possible, and he was too restless and sick for CMR, so echo and cardiac CT were performed together with the histological evaluation of bone marrow.…”

Section: Discussionmentioning

confidence: 92%

“…MS (also termed extramedullary AML, extramedullary myeloid tumor, and granulocytic sarcoma) is a rare manifestation of AML characterized by the occurrence of 1 or more myeloid tumor masses (or infiltrations) at an extramedullary site. It can occur almost anywhere in the body and can occur at any time over the course of the disease, de novo, or as relapse [4][5][6][7][8][9][13][14][15][16][17][18]. It may be difficult to diagnose because of its similarity to other neoplasms [2,3,5].…”

Section: Discussionmentioning

confidence: 99%

“…It is most commonly caused by melanoma, lymphoma, leukemia, and carcinoma of the lung, breast, and esophagus. Interestingly, over than 90% of secondary cardiac tumors remain asymptomatic [9][10][11]15].…”

Section: Discussionmentioning

confidence: 99%

“…Not only can CT provide anatomic information and tissue characterization such as calcification, but this modality offers more comprehensive assessment of patients with suspected metastatic disease. So, cardiac CT can serve as an ideal alternative to MR imaging, especially in patients with contraindications [9][10][11]15].…”

Section: Discussionmentioning

confidence: 99%

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Acute Myeloid Leukemia Presenting as a Cardiac Mass

Mohamed¹,

Saleh²,

Alzarawani³

et al. 2020

Dubai Med J

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Leukemias are the most common childhood malignancy. It is a systemic disease presenting with different signs and symptoms. Extramedullary manifestations, in particular myeloid sarcoma (MS), are extremely rare, often challenging to identify, even more challenging to diagnose, and more than often misdiagnosed owing to its similarity with other solid tumors. We describe a previously healthy boy, aged 4 years, who had a cardiac mass lesion, presumed to be a MS, noted in the right atrium at the time of initial routine echocardiography. The lesion resolved without surgical intervention following the institution of multi-agent chemotherapy.

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Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Acute Myeloid Leukemia Presenting as a Cardiac Mass

Mohamed¹,

Saleh²,

Alzarawani³

et al. 2020

Dubai Med J

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“…6 The incidence of EMI in adults with AML is reported as 2% to 5%, while it is significantly more frequently found, about 7% to 49% in pediatric AML patients. 3,[9][10][11]13 The definition in terms of accepted inclusion criteria and prognostic role of EMI is still controversial, 7,10,14,15 and there is even more limited information in the pediatric population. 2 There is a lack of consensus in various studies whether hepatosplenomegaly and lymph node involvement should be considered as EMI, 3,15,16 some arguing that those findings should not be regarded as EMI since they are a manifestation of organ infiltration by myeloblasts rather than discrete mass formation.…”

mentioning

confidence: 99%

Extramedullary Infiltration in Pediatric Acute Myeloid Leukemia on Surveillance Magnetic Resonance Imaging and its Relationship With Established Risk Factors

Kim

Im²,

Lee

et al. 2021

Journal of Pediatric Hematology/Oncology

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Purpose: Extramedullary infiltration (EMI) is a rare condition defined by the accumulation of myeloid tumor cells beyond the bone marrow. The clinical significance is still controversial. This study was aimed to evaluate the incidence, characteristics, and prognostic significance of EMI on complete magnetic resonance imaging (MRI) investigation in newly diagnosed pediatric acute myeloid leukemia (AML) patients who are asymptomatic without clinical evidence to suspect EMI.Materials and Methods: Retrospective clinical and radiologic review of 121 patients with MRI examination at the time of initial diagnosis of AML without any clinical evidence suggestive of EMI was performed. Patients were divided into 2 groups according to the presence or absence of EMI, and the relationship between EMI and established risk factors was analyzed. Initial white blood cell count, the occurrence of an event (including relapse, death, and primary refractory disease), survival status, and detailed information on cytogenetic/molecular status was performed by a thorough review of electronic medical records system. All patients underwent full imaging evaluation with the contrast-enhanced whole body and some regional MRI at the time of initial diagnosis. Results:The median age at diagnosis was 10.77 years (range, 0.37 to 18.83 y). Based on the risk stratification system of AML, 36, 45, and 40 patients are classified as low-risk, intermediate-risk, and high-risk groups, respectively. MRI at the time of the initial diagnosis of AML revealed 35 of 121 patients (28.9%) with EMI. The most common site of EMI was a skull, followed by the lower extremity bone and meninges of the brain. The median age at diagnosis was significantly younger in patients with EMI (7.87 vs. 11.08 y, P = 0.0212). Low incidence of FLT3/ITD mutation, low incidence of AML-ETO gene rearrangement, and the larger extent and more severe degree of bone marrow involvement was related with EMI. However, there was no significant prognostic difference in eventfree survival and overall survival regardless of the presence of EMI in the overall patient population and each risk group. The location of EMI occurrence was also not related to prognosis.Conclusions: Even if EMI symptoms are not evident, surveillance MRI scans at the initial diagnosis of pediatric AML patients are very helpful in detecting a significant number of EMIs. Younger age, some molecular features, and more severe bone marrow involvement of AML patients were related with EMI. However, there was no significant prognostic difference between patients with or without EMI regardless of risk group. Further prospective investigation is necessary to validate the prognostic effect of EMI in a larger group of patients with different risk groups.

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