Clinical characteristics and treatment outcomes of isolated myeloid sarcoma without bone marrow involvement: a single-institution experience

Jung Yeon Lee; Haerim Chung; Hyunsoo Cho; Ji Eun Jang; Yundeok Kim; Soo-Jeong Kim; Jin Seok Kim; Shin Young Hyun; Yoo Hong Min; June-Won Cheong

doi:10.5045/br.2017.52.3.184

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Original Article

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Blood Res 2017; 52(3):

Published online September 25, 2017

https://doi.org/10.5045/br.2017.52.3.184

Clinical characteristics and treatment outcomes of isolated myeloid sarcoma without bone marrow involvement: a single-institution experience

Jung Yeon Lee¹, Haerim Chung², Hyunsoo Cho³, Ji Eun Jang², Yundeok Kim², Soo-Jeong Kim², Jin Seok Kim², Shin Young Hyun¹, Yoo Hong Min², and June-Won Cheong^2*

Author Info. +

¹Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.

²Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

³Graduate School of Medical Science and Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea.

Correspondence to : June-Won Cheong, M.D., Ph.D. Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea. jwcheong70@yuhs.ac

Received: April 12, 2017; Revised: May 20, 2017; Accepted: June 17, 2017

Abstract

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Abstract

Background

Isolated myeloid sarcoma (MS) is a rare extramedullary tumor mass composed of malignant myeloid precursor cells without any evidence of leukemia in the peripheral blood and bone marrow. We describe the clinical characteristics and outcomes of patients diagnosed with isolated MS at our institution.

Methods

We retrospectively reviewed 9 of 497 acute myeloid leukemia (AML) patients (1.8%) with isolated MS. Isolated MS patients were divided into 2 groups according to the first-line treatment strategy: systemic treatment only (S) or local treatment with or without systemic treatment (LS).

Results

The most common site of MS occurrence was the head and neck area (N=4, 44.4%), followed by the anterior mediastinum (N=2, 22.2%) and the gastrointestinal tract (N=2, 22.2%). The tumors of 4 patients (44.4%) eventually evolved to AML, in a median time of 13.4 months (range, 2.4?20.1 mo). The number of patients achieving complete remission after first-line treatment was higher in the LS group (N=5, 83.3%) than in the S group (N=1, 33.3%) (P =0.226). All patients in the LS group survived, but those in the S group died (P=0.012).

Conclusion

Accurate and rapid diagnosis using various modalities and the early initiation of intensive combined treatment may be the optimal strategies to reduce the risk of isolated MS subsequently evolving to AML. To fully understand the characteristics of isolated MS, a larger number of patients from a multinational study is necessary.

Keywords Acute myeloid leukemia, Myeloid sarcoma, Treatment

Article

Original Article

Blood Res 2017; 52(3): 184-192

Published online September 25, 2017 https://doi.org/10.5045/br.2017.52.3.184

Clinical characteristics and treatment outcomes of isolated myeloid sarcoma without bone marrow involvement: a single-institution experience

Jung Yeon Lee¹, Haerim Chung², Hyunsoo Cho³, Ji Eun Jang², Yundeok Kim², Soo-Jeong Kim², Jin Seok Kim², Shin Young Hyun¹, Yoo Hong Min², and June-Won Cheong^2*

¹Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.

²Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

³Graduate School of Medical Science and Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea.

Correspondence to:June-Won Cheong, M.D., Ph.D. Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea. jwcheong70@yuhs.ac

Received: April 12, 2017; Revised: May 20, 2017; Accepted: June 17, 2017

Abstract

Background

Methods

Results

Conclusion

Keywords: Acute myeloid leukemia, Myeloid sarcoma, Treatment

Abstract

Fig 1.

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Figure 1.

Computed tomography (CT) findings of 56-year-old woman with primary gingival involvement (A) and 48-year-old man with nasal cavity and naso-oropharynx involvement by myeloid sarcoma (B, C). (A) Contrast-enhanced axial CT scan of the neck shows lytic destruction of the right side alveolar bone. (B) Contrast-enhanced axial CT scan of the neck shows diffuse thickening of the nasal cavity. (C)¹⁸Fluorine-labeled glucose axial PET scan shows strong focal uptake by the same lesions seen on the CT scan.

Blood Research 2017; 52: 184-192https://doi.org/10.5045/br.2017.52.3.184

Fig 2.

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Figure 2.

Computed tomography (CT) and magnetic resonance imaging (MRI) findings of a 24-year-old man with involvement of the right orbit by myeloid sarcoma who reported right eye proptosis. (A) Contrast-enhanced axial CT scan of the orbit shows a homogeneously enhancing soft tissue mass in the intra-conal and extra-conal spaces of the right orbit. (B) T1-weighted axial MRI scan of the orbit shows a right orbit mass iso-intense to the periorbital muscle. (C) T2-weighted axial MRI scan of the orbit shows a right orbit mass mildly hyper-intense to the periorbital muscle.

Blood Research 2017; 52: 184-192https://doi.org/10.5045/br.2017.52.3.184

Fig 3.

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Figure 3.

Magnetic resonance imaging (MRI) findings of a 34-year-old man with involvement of the second lumbar vertebral body by myeloid sarcoma who reported back pain and bilateral leg weakness. (A) Sagittal T1-weighted MRI scan of the spine shows an epidural and paravertebral mass iso-intense to muscle at the second lumbar vertebra level. (B) Sagittal T2-weighted MRI scan of the spine shows an epidural and paravertebral mass iso-intense or mildly hyper-intense to muscle at the second lumbar vertebra level. (C) Axial T2-weighted MRI scan of the spine shows a heterogeneously enhancing epidural mass with enhancement similar to or greater than that of normal muscle.

Blood Research 2017; 52: 184-192https://doi.org/10.5045/br.2017.52.3.184

Table 1 . The clinical and laboratory characteristics of the patients with isolated MS..

Abbreviations: ANR, above normal range; CR, complete remission; LDH, lactate dehydrogenase; NR, no response; OS, overall survival; PFS, progression-free survival; PR, partial remission; WNR, within normal range..

Table 2 . The clinico-pathological characteristics of all patients..

Abbreviations: Bcl-2, B-cell lymphoma 2; BM, bone marrow; CD, cluster of differentiation; IHC, immunohistochemistry; LCA, leukocyte common antigen; LN, lymph node; MPO, myeloperoxidase; NA, not available; NK, normal karyotype; TdT, terminal deoxynucleotidyl transferase..

Table 3 . The treatment outcomes and follow-up of all patients..

Abbreviations: Allo-HSCT, allogeneic hematopoietic stem cell transplantation; AML, acute myeloid leukemia; CCRTx, concurrent chemoradiotherapy; CR, complete remission; CTx, chemotherapy; MS, myeloid sarcoma; NR, no response; Op, operation; PR, partial remission; RTx, radiotherapy..

Table 4 . The clinical and laboratory characteristics of the patients divided into first-line treatment strategies..

Abbreviations: ANR, above normal range; CR, complete remission; LDH, lactate dehydrogenase; OS, overall survival; PFS, progression-free survival; WNR, within normal range..