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• W2051003954 abstract "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). Radiographs were obtained, and a subsequent ultrasound examination was performed (Figs 1-3).Fig 1: Anteroposterior radiograph of right shoulder.Fig 2: Axillary view radiograph of right shoulder.Fig 3: Ultrasound of right proximal humerus.Based on the history, physical findings, and imaging studies, what is the differential diagnosis? RADIOGRAPHIC INTERPRETATION 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. DIFFERENTIAL DIAGNOSIS Osteomyelitis Granulomatous disease Ewing's sarcoma Primitive neuroectodermal tumor Metastatic neuroblastoma Embryonal rhabdomyosarcoma Langerhans cell histiocytosis Malignant lymphoma Leukemia Based on the clinical examination and the imaging findings, an open biopsy was performed. Photomicrographs of the specimen are shown (Figs 4-7).Fig 4: Low power view of biopsy specimen. (Stain, hematoxylin and eosin; magnification, × 100).Fig 5: High power view of biopsy specimen. (Stain, hematoxylin and eosin; magnification, × 400).Fig 6: Photomicrograph of the biopsy specimen. Stain, myeloperoxidase; magnification, × 400).Fig 7: Photomicrograph of biopsy specimen. (Stain, naphthol esterase; magnification, × 400).Based on the history, physical findings, radiographic studies, and histologic pictures, what is the diagnosis and how should this patient be treated? HISTOLOGY 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. DIAGNOSIS Granulocytic sarcoma (chloroma). DISCUSSION General 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, Dock3 described its association with leukemia. The tumor originally was named chloroma by King9 in 1853 because of the occasional greenish color of freshly cut tumor tissue. Clinical Presentation 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.4,14,15 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. Patient Demographics The authors found 62 documented cases of isolated granulocytic sarcoma in patients without evidence of systemic disease.1,2,4-6,10,11,14,17,18 The age distribution of patients with granulocytic sarcoma is shown in Figure 8. Thirteen of 62 patients had skeletal involvement. Six patients were younger than 10 years of age. Three of these children had skeletal lesions involving the orbit, temporal bone, and maxillary sinus. Granulocytic sarcoma of a long bone in a child without evidence of systemic disease is rare (Fig 8).Fig 8: Age at presentation of patients with isolated granulocytic sarcoma who do not have evidence of systemic disease.Skeletal Distribution Skin, soft tissue, and lymph nodes are the most common locations of granulocytic sarcoma. Lesions of the larynx, breast, uterus, bladder, bowel, peritoneum, and brain have been reported.14,15 Bone is a well-described location of granulocytic sarcoma.7,13,15 Osseous lesions are most common in the skull and orbit. Lesions have been described in vertebrae, sacrum, rib, pelvis, sternum, clavicle, scapula, humerus, femur, and tibia.15 The preponderance of axial locations may be attributable to the presence of hematogenous marrow in these sites. There was no significant difference in the distribution of location of skeletal sites except in the five patients with known chronic myelogenous leukemia, in whom the lesions occurred in the femur and tibia.6,16 The majority of patients with osseous granulocytic sarcomas present with skeletal pain referable to the location of the lesion. Radiographic Appearance The usual finding on radiographs is the lytic destruction of the cortex and medulla with adjacent soft tissue mass and periosteal reaction.16 Sclerotic lesions are unusual.12 Bone scans show increased radionucleotide uptake.6 In the current case, concern about the possibility of infection lead to an ultrasound examination, which showed subperiosteal soft tissue mass extension. This finding initially was interpreted as a subperiosteal fluid collection attributable to osteomyelitis. Computed tomography (CT) scan usually shows a mixed sclerotic and lytic process but also provides better imaging of the often associated soft tissue mass.12 Magnetic resonance imaging has been helpful in imaging vertebral and cranial granulocytic sarcomas.8,12 Histologic Findings The tumor cells have abundant cytoplasm with a variable mitotic rate, and a starry sky pattern often is evident. Variable amounts of eosinophilic myelocytes are seen.15 Myeloperoxidase and naphthol chloracetate esterase stains are specific for cells of the granulocytic series and are necessary for the correct diagnosis. Without these special stains, this tumor may be identified incorrectly as Ewing's sarcoma, nonHodgkin's lymphoma, or Langerhans cell histiocytosis.5 Treatment and Prognosis The current patient was treated with local radiation and acute myelogenous leukemia protocol chemotherapy. The destructive lesion in the humerus resolved and became asymptomatic. The patient remains free of recurrence of granulocytic sarcoma and has not had acute myelogenous leukemia develop at 10 months. The prognosis for patients who present with isolated granulocytic sarcoma is improving. Most patients receive aggressive chemotherapy on acute myelogenous leukemia protocols. Radiation can be used to treat patients with symptomatic bone lesions. Orthopaedic surgeons may be called on to assist in the treatment of patients with granulocytic sarcomas. As with other hematologic malignancies in children, chemotherapy is the primary mode of local tumor control. Treatment of patients with symptomatic or unstable skeletal segments may include radiation or application of rods, plates, or screws; but treatment should be individualized according to the needs of the patient. Patients frequently have neutropenia while receiving chemotherapy for granulocytic sarcoma, and surgery is avoided wherever possible. In the current patient, no attempt was made to stabilize the affected proximal humerus, despite a partially displaced pathologic fracture. The patient had solid healing of the fracture and resolution of her symptoms shortly after the beginning of chemotherapy. Bone marrow transplantation has been described as a therapeutic option with good early results.10 Long-term, disease free survival of 21, 50, 64, and 67 months has been shown in patients with acute myelogenous leukemia who were treated with chemotherapy.1,4,5,17 Granulocyte sarcoma should be considered in the differential diagnosis of patients with osteolytic skeletal lesions. The diagnosis is commonly missed. In patients with a known myeloproliferative disorder or acute myelogenous leukemia, granulocytic sarcoma most often heralds a blastic crisis or recurrence. The prompt and accurate diagnosis of granulocytic sarcoma in patients without systemic evidence of disease is important because of the improvement in survival of patients with acute myelogenous leukemia who are treated with chemotherapy." @default.
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• W2051003954 title "Shoulder Pain in an 8-Year-Old Girl" @default.
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