Granulocytic Sarcoma (Chloroma) in HIV/AIDS

October 4, 2015

Chest Wall Mass & Altered • Xray of the Week

This HIV positive 63 year old female patient had an enlarging left anterior chest wall mass which was subsequently biopsied. She presented 2 weeks later with altered mental status, and an MRI brain was performed.

Left image: Left parasternal anterior chest wall mass.

Right image and image below: Axial T2 FLAIR PROPELLER showing mass in the left lateral aspect of the posterior fossa which is isointense to slightly T2 hyperintense to brain. There is vasogenic edema involving the left cerebellar hemisphere, with mass effect. The mass is causing deviation of the fourth ventricle to the right, as well as partial fourth ventricle effacement.

Left image: Axial exponential apparent diffusion coefficient (ADC) map demonstrating low signal at the site of the mass indicating restricted diffusion.

Right image: Axial diffusion weighted image (DWI) demonstrating high signal at the site of the mass indicating restricted diffusion, likely reflecting increased cellularity. 

Differential considerations include lymphoma, metastatic disease, as well as atypical meningeal tumors such as hemangiopericytoma.

Above: The patient had a CT guided biopsy of the chest wall mass which proved to be granulocytic sarcoma. 

Above: Photomicrographs of the biopsy done in this case. Courtesy of Dennis Kasimian, MD - Chair of Pathology at Valley Presbyterian Hospital

Left: H&E- Diffuse infiltration of soft tissue by dis-cohesive, immature mononuclear cells with irregular, hyperchromatic nuclei and a moderate amount of faintly granular cytoplasm, consistent with granulocytic sarcoma.

Right: Myeloperoxidase immunohistochemical stain: Positive cytoplasmic staining with myeloperoxidase stain confirms myeloid origin. 

 

Granulocytic Sarcoma is also known as myeloid sarcoma, chloroma, extramedullary myeloblastoma, and extramedullary myeloid tumor. It is a rare solid tumor composed of primitive precursors of the granulocytic series of white blood cells that include myeloblasts, promyelocytes, and myelocytes The tumor is an extramedullary manifestation of acute myeloid leukemia (AML).

 

History:

•1811: First described by the British physician A. Burns.

•1853: King initially called it chloroma, because typical forms have a green color caused by high levels of myeloperoxidase in the immature cells.

•1966: Rappaport renamed it granulocytic sarcoma, because not all of the cells are green.

 

Granulocytic Sarcoma Associated with:

•Acute myelogenous leukemia

•Chronic myelogenous leukemia

•Myelofibrosis with myeloid metaplasia

•Hypereosinophilic syndrome

•Polycythemia vera

 

Epidemiology:

•Occurs in 2.5-9.1% of patients with acute myelogenous leukemia.

•Occurs in <2% of patients with chronic myelogenous leukemia.

•Same rate of occurrence in both sexes.

•60% of patients are younger than 15 years old.

 

Location:

•May involve any part of the body.

•Often occur in multiples and preferentially involve orbits and subcutaneous tissue.

•Paranasal sinuses, lymph nodes, bone, spine, brain, pleural and peritoneal cavities, breast, thyroid, salivary glands, small bowel, lungs, various pelvic organs.

 

Granulocytic Sarcoma in HIV:

In the highly active antiretroviral therapy (HAART) era, the overall survival of patients with AIDS is improving dramatically and, as a result, perhaps the occurrence of malignancies not typically associated with HIV infection, especially those malignancies such as AML in which the incidence increases with age, may become more prevalent as the HIV-infected population ages.

 

Prognosis:

•Patients with granulocytic sarcomas who have chronic leukemia or myeloproliferative disorders have a negative prognosis, because these tumors often occur during acute transformation.

•Very sensitive to focal irradiation or chemotherapy; they generally resolve completely in less than 3 months.

•Poor prognosis in AIDS patients with median survival of 7.5 months if treated and 1 month if not treated.

 

References: 

1. Krause JR, and Aburiziq I. Granulocytic sarcoma and HIV. Proc (Bayl Univ Med Cent) 2011;24(4):306–308

2. Navarro WH, Kaplan LD. AIDS-related lymphoproliferative disease. Blood. 2006;107(1):1–13.

3. Rizzo M, Magro G, Castaldo P, Tucci L. Granulocytic sarcoma (chloroma) in HIV patient: a report. Forensic Sci Int. 2004;146(Suppl):S57–S58. 

4. Aboulafia DM, Meneses M, Ginsberg S, Siegel MS, Howard WW, Dezube BJ. Acute myeloid leukemia in patients infected with HIV-1. AIDS. 2002;16(6):6–865.

 

 

Related posts: Pneumocystis pneumonia in AIDS

 

 

Kevin M. Rice, MD is the president of Global Radiology CME 

Dr. Rice serves as the Chair of the Radiology Department of Valley Presbyterian Hospital in Los Angeles, California and is a radiologist with Renaissance Imaging Medical Associates. Dr. Rice has made several media appearances as part of his ongoing commitment to public education. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. In 2015 Dr. Rice founded Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field.

 

All Posts by Kevin Rice, MD

 

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