A malignant myoepithelioma is one of the rarest salivary gland neoplasms which may either arise de novo or develop within a preexisting pleomorphic adenoma or benign myoepithelioma. The parotid gland is the most common primary site and the palate the most common intra-oral site of occurrence. Herein is present a case of a malignant myoepithelioma arising in the hard palate of a 79-year-old woman. The lesion had been examined by biopsy at another hospital, and diagnosed as a poorly differentiated squamous cell carcinoma. The patient underwent a wide local tumor resection. Examination of the resection specimen showed the characteristic histopathological and immunohistochemical features of a malignant myoepithelioma. Five months after the operation, the patient was well without evidence of recurrence or metastasis.
Aged ; Female ; Humans ; Immunohistochemistry ; Myoepithelioma/*diagnosis/*pathology/surgery ; Palatal Neoplasms/*diagnosis/*pathology/surgery ; Palate/*pathology/*surgery

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.
Biomarkers, Tumor ; analysis ; Chondroma ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Immunophenotyping ; Mesenchymoma ; pathology ; surgery ; Middle Aged ; Myoepithelioma ; pathology ; surgery ; Palatal Neoplasms ; pathology ; surgery

Adenocarcinoma, Clear Cell ; metabolism ; pathology ; surgery ; Aged ; Diagnosis, Differential ; Female ; Humans ; Keratins ; metabolism ; Male ; Mucin-1 ; metabolism ; Palatal Neoplasms ; metabolism ; pathology ; surgery ; Palate, Hard ; surgery ; Tongue Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo explore the clinical characteristics, diagnosis, treatment and prognosis of adenoid cystic carcinoma of the palate.

METHODSA retrospective review was conducted in 42 patients with adenoid cystic carcinoma of the palate treated in our hospital from 1967 to 1998. Statistical analysis was performed using the Kaplan-Meier method. Prognostic factors were analyzed by Log Rank test.

RESULTSThe overall 5-, 10-, 15-year accumulative survival rates were 85.0%, 61.8% and 28.1%, and the 5-, 10-, 15-year accumulative disease-free survival rates were 52.4%, 32.7% and 22.4%, respectively. The 5-, 10-, 15-year accumulative local recurrence rates were 25.2%, 50.9% and 73.1%, and the 5-, 10-, 15-year accumulative distant metastasis rates were 28.5%, 50.4% and 66.0% respectively. T stage, bone invasion, extent of tumor, local recurrence and distant metastasis were significant prognostic factors. Treatment method, dose and area covered by radiotherapy were related to the prognosis.

CONCLUSIONSurgery alone can be done for early lesions, and combination of surgery with radiotherapy should be adopted for advanced lesions in adenoid cystic carcinoma of the palate. Radiotherapic salvage could prolong the survival of the locally recurrent patients.

Adolescent ; Adult ; Aged ; Carcinoma, Adenoid Cystic ; diagnosis ; secondary ; therapy ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms ; drug therapy ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Palatal Neoplasms ; diagnosis ; pathology ; therapy ; Palate ; radiation effects ; surgery ; Prognosis ; Retrospective Studies ; Salvage Therapy ; Survival Rate