Clinical characteristics and management of primary granulocytic sarcoma of the oral cavity

Abstract

Introduction: Granulocytic sarcoma (GS) is a commonly occurring tumor comprising immature myeloid cells, which are usually related to acute or chronic myelocytic leukemia. The tumor rarely precedes leukemia without bone marrow involvement and is called primary GS. Although primary GS can occur in any body part, the involvement of the oral cavity is uncommon. Patient concerns: A 49-year-old woman hospitalized at the Department of Plastic and Maxillofacial Surgery presented with a growing mass in her left maxillary hard palate dating two months back. No obvious physical findings were noted during general examination. She was diagnosed with an oral ulcer at a local clinic, and received antibiotics. However, the symptoms did not improve; the mass became bigger and painful. Diagnosis: An incisional biopsy of the oral mass was performed, the immunohistochemistry showed that the tumor cells tested positive for myeloperoxidase, CD4, BCL-2, KI-67. Bone marrow aspiration was negative for malignant cells, and the laboratory test results revealed only monocytosis. Standard bone marrow cytogenetic analysis showed a normal karyotype and leukemia-related fusion gene detection was normal. Therefore, the final diagnosis was intraoral primary GS. Interventions: The patient was treated with a chemotherapy regimen based on idarubicin and cytarabine arabinoside. Outcomes: After 2 cycles of idarubicin and cytarabine arabinoside regimen chemotherapy, the patient achieved complete remission. The tumor was barely visible in the left maxillary hard palate. There has been no evidence of disease spread and progression after 1 year of follow-up. Conclusions: Careful morphological and immunohistochemical analyses, correlating with clinical data are necessary to establish the diagnosis of oral primary GS. Early aggressive systemic chemotherapy can effectively relieve symptoms, significantly reducing primary GS conversion into acute myelocytic leukemia and prolonging overall survival.

Intraoral Myeloid Sarcoma as an Early Manifestation of Acute Myeloid Leukemia: Case Report and Literature Review

“…Myeloid sarcoma is de ned as an isolated extramedullary tumor without bone marrow involvement, a concomitant hematologic malignancy, or the rst manifestation of relapse [7].Myeloid or granulocytic sarcoma, also known as chloroma due to its greenish surface color, is uncommon and di cult to detect [8].Burns described it in 1811, and King called it "chloroma" because of its greenish appearance when exposed to air, caused by the presence of myeloperoxidase in tumor cells [9].…”