Primary oral myeloid sarcoma: Report of a case
A 29-year-old woman appealed to the faculty of Dentistry, Department of Maxillofacial Surgery in August 2009. The patient complained about a painful buccal gingival swelling and bilateral submandibular lymphadenopathy []. She had the history of these complaints for 3 months. The patient was initially treated for periodontitis by a dentist. On clinical examination, of gray-brown keratinized, severly painful, sessile lesion at the anterior upper labial gingiva accompanied with submandibular lymphadenopathy was noted []. There was no gingival bleeding on probing. In detailed medical history, we found out the patient had weight loss, malaise, night sweats and fever for a while. Radiological examinations did not show any bone pathology. The patient's blood test results were as follows: hemoglobin, 10.8 g/dl; hematocrit, 36.5%; white blood cell count was 9.2 × 103/μL (52.9%monocytes, 23.2% neutrophils, 22.7% lymphocytes, 0.8% eosinophils, 0.4% basophils); platelets, 146 × 103/μL. A bone marrow aspirate showed 80-90% blastic cell infiltration.\nAn incisional biopsy was performed from the labial vestibular fornix under local anesthesia and sent to Department of Oral Pathology.\nOn gross examination, the excised material showed a central grayish-white non-specific soft tissue material. Histologic examination of the biopsy specimen showed monomorphous diffuse infiltrate with a population of immature blast-like cells beneath the intact oral epithelium on panoramic view. The tumor cells were intermediate in size and round to oval in shape with mild to moderate basophilic cytoplasm without granules []. The tumor was composed of two different cell types. Some of these cells had pale nuclei containing conspicuous nucleoli, while the others had large angular shaped hyperchromatic nuclei. The tumor cells exhibited loss of cohesion with each other at these areas []. Also the tumor cells had an increased nuclear-to-cytoplasmic ratio. Rare mitotic figures were observed whereas eosinophilic myelocytes were not seen. Immunohistochemical staining were performed on the paraffin-embedded sections. Tumor cells demonstrated pale positive reaction to CD117(c-Kit) [] and strong positive reaction to myeloperoxidase (MPO) [] monoclonal antibodies; whereas, negative reactions to S-100, Melan-A, HMB-45, pan-keratin, kappa, lambda, alpha smooth muscle actin, desmin, CD3, CD15, CD20, CD34, CD45 and CD68 monoclonal antibodies was observed. Histopathological examination demonstrated an extramedullary myeloblastic malignancy. Diagnosis was MS.\nThe patient's submandibular lymph node induration and gingival swelling increased during the time period between biopsy and final histopatological diagnosis was made. After the diagnosis, we consulted the Medical Oncology Department.\nIn PET/CT (Positron emission tomography/computed tomography) scans, tumor masses were apparent in mandible and on the floor of nasal cavity []. The patient received combination of idarubicin and ARA-C (cytosine arabinoside) chemotherapy regimen at first treatment phase. Because of the enlargement of the masses and development of AML, in second chemotherapy session 3-5-7 (daunorubicin, etoposide, ARA-C) regimen was performed. Bone marrow transplantation was planned after the fourth chemotherapy session but during this hold on period other aggressive tumor masses were found in left lacrimal gland and left breast []. Histopathological diagnoses of the new lesions were also MS. Unfortunately, the patient died waiting for the appropriate bone marrow transplant and after 15 months of first diagnosis of MS.