Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients

Abstract

Myeloid sarcoma (MS) is a rare neoplasm whose knowledge is largely based on case reports and/or technically dated contributions. Ninety-two MSs in adulthood with clinical data available were evaluated both morphologically and immunohistochemically. Seventy-four cases were also studied by fluorescent in situ hybridization on tissue sections and/or conventional karyotyping on bone marrow or peripheral blood. Histologically, 50% of the tumors were of the blastic type, 43.5% either monoblastic or myelomonocytic and 6.5% corresponded to different histotypes. CD68/KP1 was the most commonly expressed marker (100%), followed by myeloperoxidase (83.6%), CD117 (80.4%), CD99 (54.3%), CD68/PG-M1 (51%), CD34 (43.4%), terminal-deoxy-nucleotidyl-transferase (31.5%), CD56 (13%), CD61/linker for activation of T cells (2.2%), CD30 (2.2%) and CD4 (1.1%). Foci of plasmacytoid monocyte differentiation were observed in intestinal cases carrying inv16. Chromosomal aberrations were detected in about 54% of cases: monosomy 7(10.8%), trisomy 8(10.4%) and mixed lineage leukemia-splitting (8.5%) were the commonest abnormalities, whereas t(8;21) was rare (2.2%). The behavior was dramatic irrespective of presentation, age, sex, phenotype and cytogenetics. Most if not all, long survivors received bonemarrow transplantation. The present report expands the spectrum of our knowledge showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal aberrations other than t(8;21), and requires supra-maximal therapy.

Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis

“…29,30 A number of studies have shown that CD30 is expressed by neoplastic cells in a small and distinct spectrum of human neoplasms, including embryonal carcinoma, Hodgkin lymphoma, anaplastic large cell lymphoma, and in rare cases of extramedullary myeloid sarcoma. [30][31][32] In addition, CD30 may be expressed in activated lymphoid cells in the course of viral infection, for example, infectious mononucleosis. 33 With the exception of single cases of myeloid sarcoma, CD30 expression has not been reported in the context of myeloid neoplasms so far.…”

Granulocytic Sarcoma of Colon in a Patient with Acute Promyelocytic Leukemia

“…Other tissues which can be involved include lymph nodes, the small intestine, the mediastinum, epidural sites, the uterus, and the ovaries. In the largest series so far, described by Pileri et al [1], the male-to-female ratio was 1.42:1. The median age was 55.8 years (range 16-87).…”

“…The clinical behavior and response to therapy do not seem to be influenced by any of the factors like age; sex; anatomic site; de novo presentation or clinical history related to AML, MDS, or MPN; histotype; phenotype; or cytogenetic findings [1]. Importantly, these patients have to be treated like any other acute myeloid leukemia and patients who undergo allogeneic or autologous bone marrow transplantation seem to have a higher probability of prolonged survival or cure.…”

“…A granulocytic sarcoma (chloroma, granulocytic sarcoma, extramedullary myeloid tumor), is a rare neoplastic condition consisting of immature myeloid cells and occurring at an extramedullary site that most frequently corresponds to the bone, skin, or lymph node, although any part of the body may be affected [1]. In other words, it is an extramedullary manifestation of acute myeloid leukemia; a solid collection of leukemic cells occurring outside of the bone marrow.…”

Myeloid Sarcoma: Presenting with Massive Cervical and Inguinal Lymphadenopathy

“…In patients with AML, MS is present in 2-8 % of these patients [5]. In adults, the true incidence of MS is unknown, although in roughly one third of cases it may occur concurrently with other myeloid diseases including myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN) or MDS/MPN [6][7][8][9][10][11], but such an association is not required. In pediatric AML however, MS occurs in approximately 10 % of cases [11].…”