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Journal of Clinical Oncology, Vol 22, No 10 (May 15), 2004: pp. 2026-2027 © 2004 American Society of Clinical Oncology. DOI: 10.1200/JCO.2004.06.103
Soft Tissue SarcomasCASE 1. Granulocytic Sarcoma: Presentation With Nodal and Skin InvolvementUniversity of Texas Medical Branch, Galveston, TX. A 70-year-old white woman presented with a 3-month history of a left chest-wall skin lesion, that gradually increased in size. Her medical history was significant for ovarian cancer in 1988 that was treated with surgery and chemotherapy, and melanoma in 1988, treated surgically. Her mother, sister, and daughter had died as a result of breast cancer. Her father and brother had died as a result of lung cancer, and a brain tumor, respectively. Physical examination revealed a 3.5-cm erythematous, ulcerated skin lesion with rolled margins below the left breast, lateral to the midclavicular line (Fig 1). There was a 4.0-cm ovoid rubbery left axillary mass and a 1.0-cm palpable left supraclavicular lymph node.
A CBC showed leukocytes 9,200/µL, and the differential count included 78% segmented neutrophils, 10% lymphocytes, 9% monocytes, 2% eosinophils, and 1% basophils. Hemoglobin measured 9.2 g/dL, and the platelet count was 348,000/µL. Computed tomography scan of the chest and abdomen demonstrated multiple bilateral lung nodules; a 3.6-cm left axillary mass; and extensive lymphadenopathy in the subcarinal, prevascular, pretracheal, periaortic, retrocrural, and mesenteric regions (Fig 2). A punch biopsy specimen of the chest wall lesion showed an extensive, diffuse, atypical infiltrate involving the deep dermis and subcutaneous tissue. The atypical cells were intermediate in size, with immature nuclear chromatin and folded nuclei. Numerous mitotic figures were present. The overlying epidermis was not involved by the neoplasm. Immunohistochemical staining showed that the atypical cells were positive for leukocyte common antigen (CD45), vimentin, and monocyte markers CD68, lysozyme, and CD43. However, CD1A, CD3, CD20, CD21, CD34, S-100 protein, keratin (AE1/3), and myeloperoxidase were negative. Histologic skin specimens were consistent with an extramedullary myeloid cell tumor of monocytic origin. Left axillary lymph node excisional biopsy revealed abundant small cells with relatively low nuclear cytoplasmic ratios, pale and slightly granular cytoplasm, and round-to-oval nuclei with powdery blastlike chromatin with absence of prominent nucleoli (Fig 3). Some nuclei had a monocytic appearance with nuclear folding. Moderate numbers of mitoses were present. Chromosome analysis from the lymph node showed an abnormal karyotype as follows: 52,XX,+add(1)(p10),+add(1)(p10),+i(2)(q10),t(3,6)(p21;q21),+6,+7,+9[5]/46,XX[15]. These chromosomal abnormalities are consistent with a clonal evolution, but are not associated with any known malignancy. Flow cytometry showed a population of immature cells positive for CD45/HLA-DR/CD13/CD11c. The cells were negative for CD33, CD34, myeloperoxidase, terminal deoxynucleotidyl transferase, and B- and T-cell markers. These findings suggested extramedullary myeloid cell tumor (granulocytic sarcoma) consistent with acute myelogenous leukemia. Bone marrow biopsy and aspiration revealed normocellular marrow with no evidence of malignancy. The patient underwent induction chemotherapy with cytarabine. Idarubicin was not given because of poor cardiac function. Her hospital course was complicated by severe sepsis resulting in death.
Granulocytic sarcoma (also called myeloblastoma or myeloid sarcoma) is a rare solid tumor composed of immature myeloid cells. It was first described in 1811.1 Such tumors often display a greenish color due to the enzymatic action of myeloperoxidase in the tumor cells; hence, the term "chloroma" was given to this lesion in 1853.2,3 The WHO classification separates these tumors into myeloid or monocytic sarcomas. Most tumors have been observed in patients with acute myelogenous leukemia and myeloproliferative disorders3; however, they have been described in patients with myelodysplasia4 and chronic lymphocytic leukemia.5,6 Granulocytic sarcomas may also occur in the absence of leukemia,7 as noted in our patient. The most common sites of involvement are bone, periosteum, soft tissue, lymph node, and skin.3 Rare occurrences in muscle,8 meninges,9 breast,10 mediastinum, and ovary11 have been reported. The present case demonstrates the unusual extramedullary presentation with lymph node and skin involvement. This often predates involvement of the marrow. Granulocytic sarcoma is treated as acute myelogenous leukemia with systemic chemotherapy. Local radiation therapy may be necessary in selected cases, such as those involving the spine. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Burns A: Observations of Surgical Anatomy, Head and Neck. Edinburgh, United Kingdom, Thomas Royce and Co, 1811, pp 364-366 2. King A: A case of chloroma. Monthly J Med 17:97, 1853 3. Neiman RS, Barcos M, Berard C, et al: Granulocytic sarcoma: A clinicopathologic study of 61 biopsied cases. Cancer 48:1426-1437, 1981[CrossRef][Medline] 4. List AF, Gonzalez-Osete G, Kummet T, et al: Granulocytic sarcoma in myelodysplastic syndromes: Clinical marker of disease acceleration. Am J Med 90:274-276, 1991[Medline]
5. Majumdar G, Singh AK: Cord compression: A rare complication of chronic lymphocytic leukaemia. J Clin Pathol 45:258-259, 1992 6. Michalevicz R, Burstein A, Razon N, et al: Spinal epidural compression in chronic lymphocytic leukemia. Cancer 64:1961-1964, 1989[Medline] 7. Buckland ME, Scolyer RA, Donellan MB, et al: Spinal chloroma presenting with triplegia in an aleukaemic patient. Pathology 33:386-389, 2001[Medline] 8. Bassichis B, McClay J, Wiatrak B: Chloroma of the masseteric muscle. Int J Pediatr Otorhinolaryngol 53:57-61, 2000[Medline] 9. Binder C, Tiemann M, Haase D, et al: Isolated meningeal chloroma (granulocytic sarcoma): A case report and review of the literature. Ann Hematol 79:459-462, 2000[Medline] 10. Ngu IWY, Sinclair EC, Greenaway S, et al: Unusual presentation of granulocytic sarcoma in the breast: A case report and review of the literature. Diagn Cytopathol 24:53-57, 2001[Medline] 11. Sreejith G, Gangadharan VP, Elizabeth K, et al: Primary Granulocytic sarcoma of the ovary. Am J Clin Oncol 23:239-240, 2000[Medline]
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Copyright © 2004 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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