Larisa Yarindy Jesús Mejenes1*, Mendoza-Revilla Diego Antonio2, Jiménez-Madrigal Víctor Gabriel3, Navarro-Munguía Jazmín2, Margareth Sharon Hall Mondragón4 and Aguilera-López Ana Karina1
1 Department of Pediatrics. Regional General Hospital 1. Mexican Institute of Social Security, Querétaro, Mexico
2 Department of Clinical Immunology and Allergy. Regional General Hospital 1. Mexican Institute of Social Security, Querétaro, Mexico
3Department of Pediatrics. General Hospital of Zone 11. Mexican Institute of Social Security. Nuevo Laredo, Tamaulipas, Mexico
4Department of Clinical Immunology and Allergy. La Raza National Medical Center. Mexican Institute of Social Security, Mexico City, Mexico
Submission: August 11, 2020;Published: August 19, 2020
*Corresponding author: Jesus-Mejenes LY, Department of Pediatrics. Regional General Hospital 1. Mexican Institute of Social Security, Querétaro, Mexico
How to cite this article: Larisa Yarindy Jesús Mejenes1*, Mendoza-Revilla Diego Antonio2, Jiménez-Madrigal Víctor Gabriel3, Navarro-Munguía Jazmín2,
Margareth Sharon Hall Mondragón4, Aguilera-López Ana Karina1. Myeloid Sarcoma (Chloroma) An Uncommon Entity. JOJ Orthoped Ortho Surg. 2020;
2(5): 555597. DOI: 10.19080 10.19080/JOJOOS.2020.02.555597
Granulocytic Sarcoma or Myeloid Sarcoma (MS) is a solid tumor of myeloid origin and extramedullary location. Its incidence is estimated between 2.5 - 9.11% of patients with Acute Myeloid Leukemia, 60% of MS cases occur in children under 15 years of age. The clinical characteristics depend on its location and range from an asymptomatic lesion to organ dysfunction due to infiltration. It appears as a circumscribed or diffuse soft tissue mass infiltrating the bone, skin and/or peritoneum. Definitive diagnosis requires biopsy and immunohistochemical study, the most sensitive markers are CD43 and lysozyme, with expression in up to 100% of cases, myeloperoxidase, is expressed in 66-96% of cases. Treatment includes radiation therapy, surgery, or both, combined with a specific systemic chemotherapy regimen or allogeneic bone marrow transplantation. Recognition of this entity, a timely diagnosis and early treatment is a diagnostic challenge when it occurs in isolation.
Granulocytic Sarcoma (GS), Myeloid Sarcoma (MS) or also known as Chloroma is a solid tumor of myeloid origin and extramedullary location [1,2]. The term Chloroma (green tumor) is derived from the greenish staining of the lesion attributable to myeloperoxidase in cells of the granulocytic lineage first described by Burns in 1811. In 2002, the World Health Organization abandoned its original name and gave it is current nomenclature (Myeloid Sarcoma) . Its estimated incidence is 2.5 - 9.11% of patients with Acute Myeloid Leukemia (AML), especially the M2 subtype, 60% of cases occur in children under 15 years of age. [4-7] Common sites of appearance include but are not limited to the skin, soft tissues, bone, peritoneum, lymph nodes, and digestive tract. Additionally, one can find lesions around the orbit, skull, and epidural spaces [2,7,8]. Its formation is believed to be due to extramedullary hematopoiesis secondary to proliferation of hematopoietic progenitor cells and an increase in the expression
of the adhesion molecule CD56 in malignant myeloid cells, associated with the genetic translocation t (8; 21) .
Asymptomatic presentations are reported in up to 50% of patients. The clinical characteristics of MS depend on its location, where the tumor can create a displacing effect or organ dysfunction due to infiltration and tumor burden .
MS usually appears as a diffuse or circumscribed soft tissue mass. Computed Tomography is the initial study of choice; however, Magnetic Resonance Imaging is preferred when central nervous system involvement is suspected [11,12].
Definitive diagnosis of MS requires biopsy and immunohistochemical studies. Morphologically, infiltration of immature cells of myeloid lineage is observed, receiving their name according to the myeloid cell involved. The diagnosis is confirmed with an immunohistochemical study by observing
surface markers such as CD68-KP1, myeloperoxidase (MPO),
CD117, CD99, CD68 / PG-M1, lysozyme, CD34, CD56, CD61,
CD30, CD4, CD43, CD45. In 2008, the WHO established the stains
required for the diagnostic study for MS and these should include
chloroacetate esterase, myeloperoxidase, and non-specific
It has been reported that the most sensitive markers for MS
are CD43 and lysozyme, with expression in up to 100% of cases,
however, they are not specific markers. MPO, which is expressed
in 66-96% of MS cases, is used to differentiate MS from lymphoma.
CD68 and CD117 are also part of the panel most used for diagnosis
[13,16]. Cytogenetic alterations are found in approximately 50% of
patients. The most common mutation described in MS is mutated
NPMI. The most common translocation is t (8; 21). Inv (16) is also
a common abnormality associated with extramedullary disease in
Treatment depends on the time of presentation of the
disease, that is, if it is an isolated tumor, concomitant with AML
or if it presents as a relapse of AML. Treatment includes radiation
therapy, surgery, or both, combined with systemic chemotherapy
specific to AML. Allogeneic bone marrow transplantation is also a
therapeutic option in achieving remission of the disease or in case
of relapse [19-23].
Zhang XH, Zhang R, Li Y (2010) Granulocytic sarcoma of abdomen in acute myeloid leukemia patient with inv (16) and t (6;17) abnormal chromosome: case report and review of literature. Leuk Res 34(7): 958-961.