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Neurology India, Vol. 53, No. 2, April-June, 2005, pp. 221-223 Case Report Spinal granulocytic sarcoma (chloroma) presenting as acute cord compression in a nonleukemic patient Kalayci Murat, Sü mer Murat, Yenidü nya Sibel, Özdölap Senay, Açikgoz Bektas Departments of Neurosurgery, Zonguldak Karaelmas University Medical School, Kozlu, Zonguldak Correspondence Address: Bahcelievler Mah, Elmas Sok, Kardelen Apt. No:13/3, 67100 Zonguldak, drkalayci@yahoo.co Date of Acceptance: 31-Dec-2004 Code Number: ni05069 ABSTRACT The case of a previously healthy 24-year-old man diagnosed with extradural thoracic granulocytic sarcoma with no evidence of bone marrow or other hematological involvement is described. The tumor was removed totally by microsurgery. The histopathological examination was consistent with granulocytic sarcoma. Granulocytic sarcomas are most commonly found in the context of an acute myelogenous leukemia or in chronic myelogenous leukemia. They rarely have been reported in otherwise healthy patients without any evidence of systemic disease. A review of the literature revealed only 14 more nonleukaemic cases with granulocytic sarcoma causing thoracic spinal cord compression.Keywords: Chloroma, epidural mass, granulocytic sarcoma, spine Granulocytic sarcomas (chloroma) originate from malignant myeloid blast cells and infiltrate the spinal extradural area. [1],[2],[3],[4] They are rare manifestations of acute and chronic leukemia and can occasionally precede the development of systemic disease by weeks to years.[1],[2],[4],[5] Granulocytic sarcomas may occur in the extramedullary spinal canal and cause spinal cord compression or cauda equina syndrome. Only a few cases of granulocytic sarcoma presenting with symptoms of spinal cord compression in patients without myelogenous leukemia have been reported.[2],[4],[5],[6],[7],[8],[9],[10],[11] Case report A 24-year-old man had localized back pain with bilateral lower extremity weakness, numbness, and urinary retention for about 45 days. His medical history was otherwise unremarkable. Physical examination revealed no abnormality. Sensory assessment revealed hypoesthesia below T5 and motor examination revealed a 3/5 paraparesis. He was unable to stand or walk independently. Deep tendon reflexes were hyperactive and Babinski signs were bilaterally positive. Laboratory evaluation revealed a white blood cell count of 4.100/mm3, a hemoglobin level of 12.6 g/dl, and an hematocrit of 37.2%. Magnetic resonance imaging (MRI) of the thoracolumbar spine revealed a posterior epidural mass between T3 and T5 [Figure - 1]A.High-dose (8 g/day) methylprednisolone was given initially, and he underwent emergent spinal cord decompression with T3 and T4 laminectomy. A soft and grayish tumor compressing the spinal cord was identified. Gross total tumor resection was carried out [Figure - 1]B. Postoperatively, the patient underwent a bone marrow biopsy, which revealed normocellular bone marrow with no evidence of lymphoma or leukemia. Peripheral blood examination was normal. Histologically the tumor was composed of a relatively uniform population of immature cells [Figure - 2]. Cells with bright eosinophilic, occasionally granular cytoplasm, consistent with myeloid lineage were present. The myeloblast had a slight to moderate rim of basophilic cytoplasm, fine nuclear chromatin, and two to four nucleoli. Eosinophil myelocytes were abundant. Because of a prominent component of myelocyte, with round, eccentric nuclei, and moderately abundant cytoplasm, and because of an associated chronic inflammatory cell infiltrate that contained mature plasma cells, the mass was initially misinterpreted as plasmacytoma. Because of the presence of a uniform population of primitive cells with scant cytoplasm, malignant lymphoma was also suspected. The presence of immature eosinophils and maturing neutrophils indicated the true nature of the tumor. Immunohistochemical staining revealed the expression of myeloperoxidase, lysozyme, and leukocyte common antigen and not of B-cell-specific or T-cell-specific antigens [Figure - 3]. The final pathological diagnosis of the specimen was granulocytic sarcoma. Although bone marrow biopsy results were inconsistent with leukemia, we preferred antileukemic therapy with a standard chemotherapeutic protocol and radiation therapy to the spinal axis and the tumor bed, because of the possibility of late development of leukemia. With an aggressive physical therapy regimen, he gradually gained strength in his lower extremities and regained the ability to walk with a cane. Bladder function was normal. No evidence of acute myelogenous leukemia has emerged over 8 months of follow up. DISCUSSION Granulocytic sarcomas represent a diagnostic challenge, particularly those occurring in patients without evidence of systemic disease.[2],[6],[11] Granulocytic sarcomas have been associated with 3.1-9.1% of acute myelogenous leukemia cases.[2] Rarely, they can occur alone without peripheral blood or bone marrow evidence of leukemia.[5] Granulocytic sarcomas are often the initial presenting feature of acute myelogenous leukemia, even when both are present at diagnosis.[2],[4],[6] Late development of acute myelogenous leukemia frequently occurs. The interval between initial diagnosis and the onset of acute leukemia has been reported between 1 and 49 months (average: 10 months).[2] We could find only 14 patients with primary epidural granulocytic sarcoma of the spine in the literature.[2],[4],[5],[6],[7],[8],[9],[10],[11]Granulocytic sarcomas usually occur in the bone, skin, and lymph nodes. Bony involvement is most common in the orbit, sacrum, spine, and ribs.[3],[5],[6] The origin of the tumor is thought to be the bone marrow, with migration to extraosseous locations achieved via the Haversian canals.[2] Macroscopically, granulocytic sarcoma is usually green in appearance. The green color, which is due to the presence of myeloperoxidase in the leukemia cells, is not present in all tumors of this type. This color rapidly fades when exposed to air.[2],[4],[5] The diagnosis is often difficult in granulocytic sarcoma cases when the myeloblastic cells are poorly differentiated and the tumor lacks the characteristic green color. The most common misdiagnoses included large cell lymphoma, malignant lymphoma, reticulum cell lymphoma, and histiocytic lymphoma.[2],[5] A diagnosis of granulocytic sarcoma is not ruled out when bone marrow biopsy specimens show no evidence of leukemia infiltration.[4] Granulocytic sarcoma should be considered in the differential diagnosis of an epidural mass in patients with or without acute leukemia, because early diagnosis followed by appropriate combined chemotherapy and radiation may obviate surgical intervention and eventually prevent leukemic transformation.[2],[4],[5],[6] Differential diagnosis includes non-Hodgkin′s lymphoma, neuroblastoma, Ewing′s sarcoma, rhabdomyosarcoma, and osteosarcoma.[1],[6] The incidence of granulocytic sarcomas is rising, due mostly to the longer survive of acute myelogenous leukemia patients.[2],[6] Treatment options include surgical decompression, chemotherapy, radiation therapy or any combination of these treatment methods.[1],[2],[4],[5] Surgery is generally preferred for cases of acute spinal cord compression. Pathological examination of the obtained tissue from the mass is very important in diagnosis especially in cases without systemic evidence of leukemia.[2],[4],[6] Granulocytic sarcomas are radiosensitive and are often treated by local radiotherapy and systemic chemotherapy. Newly diagnosed patients with isolated granulocytic sarcomas are usually treated with aggressive chemotherapy as if they have acute myelogenous leukemia; cures are not attained with radiation therapy alone.[2] REFERENCES
Copyright 2005 - Neurology India The following images related to this document are available:Photo images[ni05069f2.jpg] [ni05069f3.jpg] [ni05069f1.jpg] |
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