Myeloid sarcoma

Abstract

Purpose of review Myeloid sarcoma; also known as granulocytic sarcoma and chloroma, often occurs concomitantly with AML, and rarely without bone marrow involvement. In this article, we review the recent literature on myeloid sarcoma, focusing on treatment approach for this rare disease, and addressing the prognostic and therapeutic role of molecular and cytogenetic aberrations. Recent findings Molecular testing and cytogenetics are important adjunct to conventional diagnostic methods. The significance of cytogenetic and molecular abnormalities in myeloid sarcoma is not completely established, but testing for targetable mutations on myeloid sarcoma cells is feasible, imperative, and may guide treatment decisions. Outcomes in myeloid sarcoma largely depend on the background of its development. Almost all patients with myeloid sarcoma eventually develop AML typically in a short period after its diagnosis; therefore, remission induction treatment using AML type chemotherapy has been the standard of care. Postremission therapy is controversial; allogenic SCT, radiotherapy or consolidation chemotherapy should be considered according to patient risk. Summary Further research is required to understand the nature of myeloid sarcoma, and inclusion of patients with this condition in clinical trials should be considered to better identify the best diagnostic, prognostic, and therapeutic approach in managing this rare disease.

Extramedullary Involvement in Acute Myeloid Leukemia. A Single Center Ten Years Experience

“…Complex karyotype occurs in about 17%–39% of patients. 17 In our series, no difference for complex karyotype was observed between EMI and non-EMI patients; differently trisomy of chromosome 8, which was the most frequent cytogenetic finding, was significantly more frequent in EMI patients.…”

“…22 There is no consensus on the treatment of AML patients with EMI because of the disease's rarity and the lack of randomized controlled studies. 17 In most singleinstitution series previously published, AML-like induction therapy, followed by consolidation with either chemotherapy or allo-HSCT, is the current standard of care in fit patients. 23 Kaur et al, 23 in a retrospective review of 23 patients treated for EMI, reported that the overall survival was significantly improved for patients who achieved a complete response to induction chemotherapy.…”

Pancreatic Myeloid Sarcoma

“…MS is an extramedullary blast cell proliferation of one or more of the myeloid lineages, resulting in disruption of the normal architecture of the tissue in which it originates [5]. While MS is listed in the World Health Organization (WHO) as a subtype of AML, in reality it is a distinct entity that warrants further pathological and genetic analysis in an attempt to define its own WHO subclassification [6].…”

“…Primarily, MS precedes AML, or both pathologies are discovered simultaneously. MS may also present de novo, as a relapse of AML, or as a progression of a prior MDS, chronic myeloid leukemia, and other myeloproliferative neoplasms [3,5,6]. Isolated MS is extremely rare, with an overall incidence of less than 1% as compared with 2.5-9% in patients with AML [7].…”

“…The presence of eosinophilic metamyelocytes may hint more toward granulocytic differentiation, whereas monocytic neoplasms are more difficult to recognize. In either case, cytochemistry, immunophenotyping, cytogenetics, and molecular studies are imperative [5,15,16]. The most frequently expressed antigens by MS include CD11c, CD13, CD33, CD34, CD43, CD45, CD56, CD68, CD117, lysozyme, and myeloperoxidase.…”