Myeloid sarcoma: current approach and therapeutic options

Abstract

Myeloid sarcoma is a rare disease that can present as an isolated extramedullary leukemic tumor, concurrently with or at relapse of acute myeloid leukemia. Owing to the rarity of this disorder, most of the literature comprises small retrospective studies and case reports. The aim of this review is to summarize the current published data regarding the clinical presentation, morphological, cytogenetic and molecular features, prognosis and treatment of myeloid sarcoma.

Myeloid Sarcoma: Presenting with Massive Cervical and Inguinal Lymphadenopathy

“…The reported age range for myeloid sarcoma is 1-81 years old [5]. In patients with AML, MS is present in 2-8 % of these patients [5].…”

“…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.…”

Isolated myeloid sarcoma

“…Randomized prospective trials are lacking. The current recommended treatment regimen in patients presenting with MS is conventional AML-type chemotherapeutic protocols 10 . But according to the report of Yamauchi and Yasuda cytarabine may be an important agent in the treatment of MS since they found a prolonged DFS in the patients treated with cytarabine contained regiment compare with those who were initially misdiagnosed and treated with agents used to treat lymphoma, sarcoma or multiple myeloma 11 Due to a higher rate of progression to AML in isolated MS patients, localized treatment like radiotherapy and surgical treatment is not recommended 3 .…”

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“…MS could occur isolated, although most often precedes, relapse or coincides with acute myeloid leukemia (AML), or less commonly represents an acute blastic transformation of myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN) or MDS/MPN. [1][2][3] In cases with granulocytic differentiation, eosinophilic metamyelocytes, erythroid precursors, and megakaryocytes are helpful clues for diagnosis, whereas MSs with monocytic differentiation are more difficult to recognized and more easily to be misdiagnosed as lymphoma. The most common site of involvement by MS is the skin.…”

“…The most common site of involvement by MS is the skin. 2,4 Central nervous system (CNS) or lymph nodes involvement is relatively rare.…”