Granulocytic Sarcoma (Chloroma) - Case Report

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An 8-year-old, right hand dominant girl complained of right shoulder pain of 2 weeks' duration. She had decreased range of motion (ROM) and was using the arm significantly less. Four days before the onset of pain, she had a sore throat, fever, and pharyngeal exudate. The results of pharyngeal cultures were negative. She was treated with azithromycin and her condition improved. A low grade temperature, decreased appetite, and increased fatigue persisted. She could recall no history of trauma. Her medical history was unremarkable.

Physical examination revealed minimal erythema and swelling over the lateral aspect of the proximal humerus and a focal point tenderness over the biceps tendon. There was substantially decreased active and passive ROM secondary to pain. The results of the neurovascular examination were normal. Laboratory tests revealed a normal leukocyte count of 6500 / µL. The differential was normal except for an elevated level of eosinophils (11.8%, normal 0%-6%). The erythrocyte sedimentation rate was 45 mm/hour (normal, 0-15 mm/hour).

The plain radiographs showed a lytic lesion involving the posterolateral aspect of the metaphysis of the proximal humerus. The cortex was obliterated, and the process extended to the physis. There was a permeative type of destruction involving the metaphysis with a wide zone of transition. There was no sclerosis or periosteal new bone formation. Ultrasound examination was interpreted as showing the destructive process of the proximal humerus with a subperiosteal fluid collection and possible necrotic debris in the medullary cavity.

Based on the clinical examination and the imaging findings, an open biopsy was performed. Photomicrographs of the specimen are shown

An incisional biopsy provided soft tissue and bone specimens from the right proximal humerus. The microscopic examination revealed a malignant tumor composed of large cells with irregular nuclear borders, vesicular nuclei, one or more nucleoli, and abundant cytoplasm. The mitotic rate was high and a starry sky pattern was evident. An occasional eosinophilic myelocyte was apparent. The tumor appeared to involve the spongiosa, and reactive bone formation was present. Stains for myeloperoxidase and naphthol chloracetate esterase were positive.

Granulocytic sarcoma (chloroma).

Granulocytic sarcoma is a solid tumor of immature granulocytes that most commonly occurs in a patient with leukemia or some other myeloproliferative disorder but occasionally may occur, such as in the current case, in an otherwise healthy individual. In 1893, Dock described its association with leukemia. The tumor originally was named chloroma by King in 1853 because of the occasional greenish color of freshly cut tumor tissue.

This tumor usually develops (1) in an individual with no known disease where it is a harbinger of acute myelogenous leukemia; (2) in an individual who already has acute myelogenous leukemia; and (3) in an individual with a known myeloproliferative disorder.15 In the current case, the symptoms of pharyngitis that preceded the discovery of the bone lesion are of uncertain significance. However, the possibility of osteomyelitis was considered based on the clinical history and initial plain radiographs, which prompted the treating physician to perform an ultrasound as the first imaging study. Subsequent evaluation of the peripheral blood and bone marrow biopsy in the current patient showed this to be granulocytic sarcoma without systemic evidence of acute myelogenous leukemia. The appearance of this tumor in a previously healthy individual presents a significant diagnostic challenge. Seventy-five percent of patients with granulocytic sarcoma initially are misdiagnosed. Work up and staging of a patient with granulocytic sarcoma is similar to the work up for a patient with acute myelogenous leukemia. After the biopsy specimen is obtained, a bone marrow aspiration and spinal fluid analysis are needed. A bone scan or skeletal survey is needed to identify any additional bone lesions, although virtually all cases of granulocytic sarcoma in bone are solitary lesions.

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