Myeloid Sarcoma: The Clinician's Point of View

Myeloid Sarcoma may occur in patients with an acute or chronic myeloproliferative disorder as well as de novo, with no apparent sign or symptom of concomitant haematological disease. The patients are preferentially young male and the site of disease localization may vary from central nervous system to pleura and thorax, with a common involvement of the reticuloendothelial system. The disease often shows chromosomal rearrangements, involving chromosomes 7, 8 and 3 and sometimes a complex karyotype (more than 3 abnormalities) is detected at diagnosis. The prognosis of this disease is dismal and only high-dose chemotherapy with autologous or allogeneic stem cells transplantation (auto or allo-SCT) may be potentially curative. In the absence of definitive elements that can define the prognosis of extra-medullary localization of “standard risk” AML, Clinicians should pursue the collection of data from different Centres and design of homogeneous treatment strategies, that could integrate standard chemotherapy with specific approaches, such as radiotherapy, transplant procedures or, in selected cases (such as those displaying molecular abnormalities involving protein tyrosine-kinases), molecularly targeted therapies. Recently Al-Khateeb et al. reported a clinicopathologic, cytogenetic, and outcome analysis of 21 adult patients with Myeloid Sarcoma (MS) [1]. Briefly, they show that MS may occur in patients with an acute or chronic myeloproliferative disorder (13 patients) as well as de novo (8 cases), with no apparent sign or symptom of concomitant haematological disease. The patients are preferentially young male, and the site of disease localization may vary from central nervous system to pleura and thorax, with a common involvement of the reticuloendothelial system. The disease often shows chromosomal rearrangements, involving chromosomes 7, 8, and 3, and sometimes a complex karyotype (more than 3 abnormalities) is detected at diagnosis. The authors confirm that the prognosis of this disease is dismal and that only high-dose chemotherapy with autologous or allogeneic stem cells transplantation (auto- or allo-SCT) may be potentially curative. From a clinical point of view, we agree with the authors’ conclusions regarding the disease features and prognosis. As has been recently reviewed by Pileri et al. on 92 adult patients [2], development of a myeloid tumor at an extramedullary site can be either the sole evidence of a myeloid neoplasm or can happen concurrently or after an acute myeloid leukemia (AML) or other myeloproliferative neoplasms (MPN). In the