Primary granulocytic sarcoma
Primary Granulocytic Sarcoma
Primary granulocytic sarcoma, also known as chloroma, is a rare extramedullary tumor composed of immature myeloid cells. It is associated with acute myeloid leukemia (AML) but can occur in the absence of overt leukemia, which is when it is termed "primary." This condition is significant due to its potential to precede or coincide with the development of systemic leukemia.
Pathophysiology[edit | edit source]
Granulocytic sarcoma arises from the proliferation of myeloid precursor cells outside the bone marrow. These cells can infiltrate various tissues, forming a mass. The tumor is often greenish in color due to the presence of myeloperoxidase, an enzyme found in myeloid cells, which gives it the name "chloroma" (from the Greek "chloros," meaning green).
Clinical Presentation[edit | edit source]
Patients with primary granulocytic sarcoma may present with a variety of symptoms depending on the location of the tumor. Common sites include the skin, lymph nodes, bone, and soft tissues. Symptoms can range from painless masses to more severe symptoms if the tumor compresses vital structures.
Diagnosis[edit | edit source]
Diagnosis of primary granulocytic sarcoma involves a combination of imaging studies, histopathological examination, and immunohistochemistry. Biopsy of the lesion typically reveals a dense infiltrate of immature myeloid cells. Immunohistochemical staining is positive for myeloid markers such as myeloperoxidase, CD68, and CD117.
Treatment[edit | edit source]
The treatment of primary granulocytic sarcoma often involves systemic chemotherapy similar to that used for acute myeloid leukemia. Radiation therapy may be used for localized control of the tumor. In some cases, hematopoietic stem cell transplantation is considered, especially if the patient develops systemic leukemia.
Prognosis[edit | edit source]
The prognosis of primary granulocytic sarcoma varies depending on whether it is associated with systemic leukemia. Isolated granulocytic sarcoma without leukemia has a better prognosis, but there is a high risk of progression to acute myeloid leukemia.
Also see[edit | edit source]
- Acute myeloid leukemia
- Myeloid sarcoma
- Extramedullary hematopoiesis
- Hematopoietic stem cell transplantation
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Contributors: Prab R. Tumpati, MD
