Cutaneous Myeloid Sarcoma: Natural History and Biology of an Uncommon Manifestation of Acute Myeloid Leukemia

Abstract

We conducted a retrospective study of patients with cutaneous myeloid sarcoma, from 2 tertiary care institutions. Eighty-three patients presented, with a mean age of 52 years. Diagnosis of myeloid sarcoma in the skin was difficult due to the low frequency of myeloperoxidase and/or CD34+ cases (56% and 19% of tested cases, respectively). Seventy-one of the 83 patients (86%) had ≥ 1 bone marrow biopsy. Twenty-eight (39%) had acute myeloid leukemia with monocytic differentiation. Twenty-three had other de novo acute myeloid leukemia subtypes. Thirteen patients had other myeloid neoplasms, of which 4 ultimately progressed to an acute myeloid leukemia. Seven had no bone marrow malignancy. Ninety-eight percent of the patients received chemotherapy, and approximately 89% died of causes related to their disease. Cutaneous myeloid sarcoma in most cases represents an aggressive manifestation of acute myeloid leukemia. Diagnosis can be challenging due to lack of myeloblast-associated antigen expression in many cases, and difficulty in distinguishing monocyte-lineage blasts from neoplastic and non-neoplastic mature monocytes.

Recurrence of a t(8;21)-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge

“…GS associated with translocations t(8;21)(q22;q22)/RUNX1-RUNX1T1 transcript frequently locates in the orbital region in children [9], while those with inv(16) (p13.1q22)/t(16;16)(p13.1;q22) have a high incidence of gastrointestinal tract or breast involvement, especially in the adults [18, 19]. Although to be confirmed, there is also evidence of an association between trisomy 8 positive AML and cutaneous localization of GS [20]. …”

Treatment of blastic plasmacytoid dendritic cell neoplasm

“…As pictured in Figure 1C, chromatin is typically dispersed, and nuclear membranes are irregular with prominent nucleoli and increased mitotic activity. [17][18][19] The clinical and histologic presentation are most similar to myelomonocytic (M4) or monoblastic/monocytic (M5) leukemia with skin involvement, typically referred to as LC, aleukemic myeloperoxidase LC (MPO-LC), 17,20 or more rarely CD4…”

Cutaneous Hematopathology

“…Monosomy 7; MLL (myeloid/lymphoid or mixed-lineage leukemia) rearrangement; trisomy 4; monosomy 16, 16q, 5q, and 20q; and trisomy 11 are some of the reported abnormalities ). Trisomy of chromosome 8 and inversion 16 are more common in MS involving the skin (Dachary et al 1986 ;Cronin et al 2009 ;Hurley et al 2013 ). NPM1 mutations are seen in 16 % of cases (Falini et al 2005(Falini et al , 2007.…”

“…Males are more affected than females with a ratio of 1.2:1. Median age is 56 years but is also seen in older patients (Falini et al 2007 ;Pileri et al 2007 ;Hurley et al 2013 ). In one series of 61 biopsied cases, 13 had skin involvement (Neiman et al 1981 ).…”