DIAGNOSTIC CHALLENGES WITH INTRAORAL MYELOID SARCOMA: REPORT OF TWO CASES & REVIEW OF WORLD LITERATURE

Abstract

Background: Myeloid sarcomas (MS) are rare extramedullary tumors composed of blasts of myeloid lineage that either precede, follow or present concomitantly with acute myeloid leukaemia (AML) or myeloproliferative neoplasms. The diagnosis of MS is especially challenging in patients without an antecedent history of leukemia. Methods: We present 2 cases of intraoral MS that presented as de novo lesions. A detailed review of cases of intraoral MS that either preceded or presented along with leukemia has been done with emphasis on diagnostic criteria used. Results: Two male patients aged 28 and 5 years presented with MS with one patient presenting with concomitant AML. A combination of morphological and immunohistochemical methods was used for diagnosis. A thorough review of world literature revealed 44 cases of intraoral MS that presented as de novo lesions. Conclusion: Intraoral MS is a rare tumor with poor prognosis. It may be diagnostically challenging due to its protean clinical manifestations and histological overlap with other tumors.

Intraoral granulocytic sarcoma as a manifestation of myelofibrosis: A case report and review of the literature

“…In the present case, the patient was an 81‐year‐old man, presenting a nodular, sessile lesion, measuring about 2 cm at its largest point, and was purple and hemorrhagic. In accordance with the literature, the patient was asymptomatic, but the mandible represented the majority of the oral manifestations, which is not consistent with this case; however, GS has been reported at almost all intraoral sites (Table ).…”

“…Although some studies have recommended that chemotherapy is more effective than radiotherapy or surgical resection, there is no consensus regarding the management. It was reported that GS associated with chronic myeloid leukemia (CML) or myelodysplastic syndrome has a worse clinical outcome when compared to MS with AML . In addition, GS prognosis may be poor, with survival times varying among different studies …”

Myeloid sarcoma of the oral cavity: A case report and review of 89 cases from the literature

“…Considering the 89 cases of oral MS previously published in the English literature, the average age of the patients is 45 years (ranging from 1 to 89 years), with slight predilection for females (1,2:1). The mandible is the most common affected site (28 cases), followed by maxilla (20 cases), gingiva (13 cases), and palate (seven cases) ( 1 - 15 ). MS affected multiple intraoral anatomical sites in eight cases, six of them with concomitant involvement of maxilla and mandible ( 5 , 7 , 8 , 11 , 13 - 15 ).…”

“…APL is characterized by the predominance of abnormal promyelocytes in the bone marrow and identification of a specific chromosomal translocation t(15;17)(q24.1;q21.1) resulting in a fusion transcript between the genes promyelocytic (PML on chromosome 15) and retinoic acid receptor alpha (RARA on chromosome 17). The most common diseases associated with oral MS are AML (43 cases), followed by myelodysplastic syndrome (11 cases), and chronic myeloid leukemia (seven cases), nevertheless 14 patients with oral MS did not present nor developed associated leukemia or myelodysplastic neoplasms ( 1 - 15 ).…”

“…Oral involvement by MS is uncommon. To the best of our knowledge, only 89 cases of oral MS have been published in the English-language literature so far, and only four of them were associated with acute promyelocytic leukemia ( 1 - 15 ). Herein, we report an additional case of oral MS in a 24-year-old female with acute promyelocytic leukemia, including a review of the literature.…”