|Year : 2019 | Volume
| Issue : 2 | Page : 41-43
S Saivignesh1, MP Brundha2
1 Department of Pathology, Saveetha Dental College and Hospital, Saveetha Institute of Medical and Technical Science, Chennai, Tamil Nadu, India
2 Department of General Pathology, Saveetha Dental College and Hospital, Saveetha Institute of Medical and Technical Science, Chennai, Tamil Nadu, India
|Date of Submission||14-Sep-2019|
|Date of Acceptance||17-Oct-2019|
|Date of Web Publication||11-Dec-2019|
M P Brundha
Department of General Pathology, Saveetha Dental College and Hospital, Saveetha Institute of Medical and Technical Science, 162, Poonamalle High Road, Velapanchavadi, Chennai - 600 077, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is usually accompanied by acute myeloid leukemia, although in some rare cases, it may present in nonleukemic patients. It is a rare entity characterized by the occurrence of one or more tumor myeloid masses occurring at an extramedullary site. It may occur at any site, leading to very varied clinical presentations. However, the most common locations are soft tissues, bone, peritoneum, and lymph nodes. The aim of this review is to summarize about pathogenesis, diagnostic test, prognosis, and treatment of myeloid sarcoma.
Keywords: Bone, granulocytic cells, lymph nodes, medullary tumor, pathogenesis
|How to cite this article:|
Saivignesh S, Brundha M P. Myeloid sarcoma. Int J Clinicopathol Correl 2019;3:41-3
| Introduction|| |
Myeloid sarcoma is a neoplastic condition which consists of immature cells of granulocytic series. It is called as chloroma owing to its green color attributed to the enzyme myeloperoxidase. It is also called as granulocytic sarcoma, myeloblastoma, and extramedullary myeloid cell tumor. It is a pathologic diagnosis for an extramedullary proliferation of blasts of one or more of the myeloid lineages that disrupt the normal structure of the tissue in which it is found. It consists of myeloblasts, with or without features of promyelocytic or neutrophilic maturation, that partially or totally efface the tissue architecture. Myeloid Sarcoma may occur in association with Acute Myeloid Leukemia, before or after as a relapse. It is less often associated with myeloproliferative neoplasm or myelodysplastic disorder. Multiple sclerosis (MS) occurs at any age both in pediatric and elderly patients.,
| Prevalence and Clinical Presentation|| |
The incidence of the isolated Myeloid Sarcoma in adults is 2%. The age of patients at MS presentation is highly variable, with cases being reported in patients 1–81. It mostly occurs in soft tissues, bone, peritoneum, lymph nodes, and gastrointestinal system. Other sites include genitourinary system of males and females and the central nervous system. In children with newly diagnosed AML, extramedullary involvement was most common in the skin (in 54%), with orbital involvement being the second most common site. MS size at diagnosis is highly variable ranging from 2 to 20 cm. It is reported in 2%–8% of patients with AML either as a single or as a multifocal tumor.
| Diagnosis|| |
Diagnosis of MS with known AML or other hematologic malignancies is relatively easy. However, the differential diagnosis of primary MS is relatively difficult for a pathologist. The rate of misdiagnosis is 75% and mostly misdiagnosed as large cell lymphoma followed by malignant lymph proliferative disorders. The misdiagnoses are non-Hodgkin lymphoma, histolytic lymphoma, thymoma, myeloma, eosinophilia sarcoma, extramedullary hematopoiesis, mucosa-associated lymphoid tissue, Ewing sarcoma and carcinoma, undifferentiated cancer, malignant melanoma, extramedullary hematopoiesis, and inflammation. The characteristic microscopic growth pattern of myeloid cells is either a diffuse or an Indian file pattern. Although the fine needle aspiration is used for diagnosis in the literature, tissue biopsy is the preferred method. The morphologic appearance on H and E varies according to differentiation of the cells. It mainly consists of infiltration by myeloblasts. It is recommended to send the specimen to immunohistochemistry, flow cytometry, fluorescence in situ hybridization, and molecular analysis followed by bone marrow biopsy, and aspiration should be performed to rule out other hematological malignancies. Based on localization of the tumor, magnetic resonance or computed tomography is performed. These techniques differentiate from abscess and hematomas in patients with AML.
| Prognosis|| |
The prognosis of isolated MS is not well examined in large prospective studies, but it has a poor prognosis. In some series with subgroup analysis of isolated MS performed in children, it was demonstrated that it had a better prognosis than children with AML, without MS, and patients concomitant with AML., Another study conducted by Tsimberidou et al. have stated that isolated MS patients with chromosome 8 abnormalities had a worse prognosis, and intensive chemotherapies were needed in this group.
| Local Approaches|| |
The role of radiotherapy in addition to systemic chemotherapy is not established, although it is often given. Studies have shown that patients treated only with radiotherapy had a high rate of progression to AML after a short no-leukemic period. It could also be used as a consolidation therapy after systemic chemotherapy while considering the possible toxicities due to the localization of the tumor., The study conducted by Tsimberidou et al. stated that the failure-free survival in 21 patients with no leukemic granulocytic sarcoma was lower with the combination treatment of chemoradiotherapy. In other study, it had no effect on survival in multivariate analyses. Hence, it could be used in conjunction with systemic therapies or patients who need a rapid relief of symptoms, or it could also be used as a consolidation therapy.
The patients who undergo surgery, almost always relapse or progress to AML, and die. The incidence of AML or extramedullary relapse was significantly higher in patients who were treated with surgery only., It was also demonstrated by Yamauchi et al. that the time interval to progression to AML was higher in patients who did not receive systemic therapy. Most of the patients (81%) who did not receive systemic treatment were shown to progress to AML in the first 11 months. Therefore, surgery is not an effective treatment strategy for primary MS, and surgery should be considered before the systemic treatment in acute situations. It could also be used to confirm the diagnosis in rare cases.
Bone marrow transplantation
Allogeneic stem cell transplantation was an efficient treatment modality with a no-relapsing mortality rate of 17%. Nearly half of the patients (47%) in this study got transplantation in the first remission. The 5-year overall survival was 33%, and leukemia-free survival was 30%. It was concluded that hematopoietic stem cell transplantation (HSCT) as a first-line effective therapeutic option with longer overall survivals.
There is no targeted therapy for patients with MS but new agents such as the nucleoside analogs, FLT3 inhibitors, farnesyltransferase inhibitors, histone deacetylase inhibitors, and DNA methyltransferase inhibitors are currently being tested for AML treatment and may change treatment options and prognosis.
| Conclusion|| |
The optimal treatment of the myeloid sarcoma is not clear since there is not enough data and large prospective studies in the literature. Systemic chemotherapies used in AML remission induction treatment are mostly suggested therapy with or without radiotherapy. HSCT should also be considered in relapsed or refractory patients after reinduction and used as a consolidation treatment in suitable patients. New large prospective studies and new agents are needed for the treatment.
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Conflicts of interest
There are no conflicts of interest.
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