Objective: To study the clinicopathologic and genetic features of papillary cystic low-grade mucoepidermoid carcinoma (LG-MEC) and cystadenoma. Methods: A retrospective review was performed on salivary gland tumor patients with papillary cystic architecture who presented to department of oral pathology, Peking University School and Hospital of Stomatology between January 2010 and June 2022. Among this cohort, there were 17 males and 17 females with a range age of 23-82 years [(55.6±14.6) years]. Diagnosis was confirmed by histological, immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) analysis. Finally, 15 papillary cystic LG-MEC and 19 cystadenoma patients were included in the present study. All patients were followed clinically and radiologically, and the duration of follow-up ranged from 1 to 141 months. Results: All neoplasms showed papillary proliferation with multilocular or giant cystic tumors. Papillary cystic LG-MEC was characterized by epidermoid cells, intermediate cell and mucous cells with multiple lining-layers. Papillary cystic LG-MEC had mild cellular atypia and a pushing infiltration. Cystadenoma was characterized by cuboidal, columnar and ciliated pseudostratified columnar lining epithelium. Squamous metaplasia, mucinous metaplasia and acidophilic degeneration could also be observed focally in cystadenoma. For IHC staining, papillary cystic LG-MEC showed diffusely and strongly positive for mucin 4 (MUC4) (15/15) and mucin 5 Subtype AC (MUC5AC) (4/15) in the epidermoid cells, intermediate cell and mucous cells. The epidermoid cells and intermediate cells were diffusely positive for p40 and p63. The Ki-67 index was about 10%-15% in LG-MEC. As a contrast, p40 (17/19) and p63 (14/15) were only detected in the basal cells of cystadenoma. Cystadenoma showed focal MUC5AC (4/19)expression and MUC4 (19/19)diffuse expression. In addition, the Ki-67 index was 5%-10% in cystadenoma. The MAML2 gene translocation was detected in 11 LG-MEC patients, but none in cystadenoma. Conclusions: The differential diagnosis points between papillary cystic LG-MEC and cystadenoma included the specific epidermoid cells, intermediate cells and mucus cells in LG-MEC, cell atypia, the pushing-infiltration pattern, diffuse expression of p40 and p63 in the lining epithelium, and a MAML2 gene rearrangement. The molecular test of MAML2 should be recommended to reduce missed LG-MEC diagnoses.
Male ; Female ; Humans ; Carcinoma, Mucoepidermoid/pathology* ; In Situ Hybridization, Fluorescence ; Ki-67 Antigen/genetics* ; Biomarkers, Tumor/analysis* ; Salivary Gland Neoplasms/diagnosis* ; Transcription Factors/metabolism* ; Cystadenoma ; Metaplasia

Mucoepidermoid carcinoma is a malignant tumor that occurs mainly in the salivary glands of adults and children, but rarely in the esophagus. A surgical resection is the primary treatment for mucoepidermal carcinoma, and the prognosis has been reported to be poor. A 61-year-old man was diagnosed with an esophageal nodule in upper gastrointestinal endoscopy for the purpose of a medical examination without any special symptoms and an endoscopic resection was performed for an accurate diagnosis. An endoscopic mucosal resection was performed using a band ligation for a complete resection and continuous follow-up with no evidence of recurrence or metastasis. The unknown prognosis of endoscopic resection makes it necessary to follow the patient carefully.
Adult ; Carcinoma, Mucoepidermoid ; Child ; Diagnosis ; Endoscopy, Gastrointestinal ; Esophagus ; Follow-Up Studies ; Humans ; Ligation ; Middle Aged ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Salivary Glands

Mucoepidermoid carcinoma is a malignant tumor that occurs mainly in the salivary glands of adults and children, but rarely in the esophagus. A surgical resection is the primary treatment for mucoepidermal carcinoma, and the prognosis has been reported to be poor. A 61-year-old man was diagnosed with an esophageal nodule in upper gastrointestinal endoscopy for the purpose of a medical examination without any special symptoms and an endoscopic resection was performed for an accurate diagnosis. An endoscopic mucosal resection was performed using a band ligation for a complete resection and continuous follow-up with no evidence of recurrence or metastasis. The unknown prognosis of endoscopic resection makes it necessary to follow the patient carefully.
Adult ; Carcinoma, Mucoepidermoid ; Child ; Diagnosis ; Endoscopy, Gastrointestinal ; Esophagus ; Follow-Up Studies ; Humans ; Ligation ; Middle Aged ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Salivary Glands

Pulmonary mucoepidermoid carcinoma (MEC) is a rare form of lung cancer that originates from submucosal glands of tracheobronchial tree. Unlike low-grade tumor with benign nature, high-grade case is even rarer and has aggressive clinical features with no definite treatment option. Here, we report a case of high-grade pulmonary MEC with fulminant clinical course. A 74-year-old man presented with cough, sputum and mental change. Chest imaging showed massive mediastinal lymphadenopathy with obstructive pneumonia, and multiple metastases in lung and adrenal gland. Bronchoscopy showed polypoid masses obstructing right main bronchus and bronchus intermedius. Histopathology revealed a mixture of glandular structure lined with mucussecreting cells and nests of squamoid cells with nuclear atypia and pleomorphism, which is compatible with high-grade MEC. We intensively treated the patient with combination antibiotics and ventilator care. However, the patient did not respond to the treatment and rapidly deteriorated, and finally expired a month after diagnosis.
Adrenal Glands ; Aged ; Anti-Bacterial Agents ; Bronchi ; Bronchoscopy ; Carcinoma, Mucoepidermoid* ; Cough ; Diagnosis ; Humans ; Lung ; Lung Neoplasms ; Lymphatic Diseases ; Neoplasm Metastasis ; Pneumonia ; Sputum ; Thorax ; Ventilators, Mechanical

OBJECTIVETo explore the diagnostic value of MYB protein expression for adenoid cystic carcinoma and its differential diagnosis from other salivary gland tumors, and to further investigate the status of MYB gene copy number.

METHODSMYB expression was studied by immunohistochemistry in 34 adenoid cystic carcinomas, 55 non-adenoid cystic carcinomas (other salivary gland tumors) including 10 pleomorphic adenomas, 10 basal cell adenomas, 10 epithelial-myoepithelial carcinomas, 9 basal cell adenocarcinomas, 8 mucoepidermoid carcinomas, 4 carcinoma in pleomorphic adenomas, and 4 polymorphous low-grade adenocarcinoma. MYB gene copy number status was detected by FISH in MYB protein-positive cases.

RESULTS82.4% (28/34) of adenoid cystic carcinomas were MYB protein-positive, compared with 9.1% (5/55) of non-adenoid cystic carcinomas, and the difference between the two groups was statistically significant (P < 0.01). 2/18 of adenoid cystic carcinomas had duplication of MYB gene by FISH, and all non-adenoid cystic carcinomas were negative although the difference was not statistically significant (P = 0.435).

CONCLUSIONSMYB protein expression is a useful diagnostic marker for adenoid cystic carcinomas in its separation from other salivary gland tumors. In addition, duplication of MYB gene is no a major mechanism for the MYB protein overexpression.

Adenoma ; Adenoma, Pleomorphic ; Biomarkers, Tumor ; genetics ; metabolism ; Carcinoma, Adenoid Cystic ; diagnosis ; genetics ; metabolism ; Carcinoma, Mucoepidermoid ; Diagnosis, Differential ; Gene Dosage ; Humans ; Immunohistochemistry ; Proteomics ; Proto-Oncogene Proteins c-myb ; genetics ; metabolism ; Salivary Gland Neoplasms

OBJECTIVETo study the clinicopathologic characteristics of primary salivary gland-type lung carcinomas, and the immunophenotypic value of TTF-1, Napsin A and Ki-67 in their differential diagnosis.

METHODSTotally 48 special type lung cancer surgical removal specimens were collected in China-Japan Friendship Hospital during September 2009 to December 2014. A panel of immunohistochemical markers (TTF-1, Napsin A, Ki-67, CK5/6, CK7 and p63) were conducted on these specimens.

RESULTSThe 48 cases of special type lung cancer included 25 cases of primary salivary gland-type lung carcinoma (18 cases of adenoid cystic carcinoma and 7 cases of mucoepidermoid carcinoma), 5 cases pulmonary adenocarcinoma with mucoepidermoid carcinoma-like or adenoid cystic carcinoma-like structure, and 18 cases of pulmonary adenosquamous carcinoma. Compared with pulmonary adenocarcinoma with mucoepidermoid carcinoma-like or adenoid cystic carcinoma-like structure and pulmonary adenosquamous carcinoma, primary salivary gland-type lung carcinomas have special characteristics in median age, sex, location, tumor size, LN involvement and pleura invasion, with negative TTF-1 and Napsin A expression as well as lower Ki-67 index detected by immunohistochemistry. Primary salivary gland-type lung carcinomas usually have an indolent behavior.

CONCLUSIONSPrimary salivary gland-type lung carcinomas are low-aggressive entities. The origins of primary salivary gland-type lung carcinomas were different from that of pulmonary adenocarcinoma with mucoepidermoid carcinoma-like or adenoid cystic carcinoma-like structure and pulmonary adenosquamous carcinoma. Negative TTF-1 and Napsin A expression as well as Ki-67 index lower than 20% have special value for primary salivary gland-type lung carcinomas in their differential diagnosis.

Adenocarcinoma ; diagnosis ; Aspartic Acid Endopeptidases ; metabolism ; Biomarkers, Tumor ; metabolism ; Carcinoma, Adenoid Cystic ; diagnosis ; Carcinoma, Mucoepidermoid ; diagnosis ; China ; DNA-Binding Proteins ; metabolism ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; diagnosis ; Transcription Factors

Cystadenoma rarely arises in the parotid gland. It grows slowly without pain. Histopathologically, it is subdivided into papillary cystadenoma and mucinous cystadenoma. Mucinous cystadenoma arising from the salivary gland is a very rare benign tumor. A 25-year-old female patient visited our hospital with a left infra-auricular mass. Computed tomography scan showed a 3.1x2.3 cm size heterogeneous lesion with an irregular margin in the left parotid gland. Sono guided gun biopsy was performed to reveal fibrous tissue with atypical epithelial cell with mucin. Suspecting mucoepidermoid carcinoma, partial parotidectomy was performed for treatment and diagnosis. Histopathologic exam revealed mucinous cystadenoma. Cystadenoma of salivary gland has no distinct clinical feature, and is difficult to differentiate clinically from other salivary gland tumors as well as its malignancy. Thus, the possibility of malignancy must be considered for differential diagnosis.
Adult ; Biopsy ; Carcinoma, Mucoepidermoid* ; Cystadenoma ; Cystadenoma, Mucinous* ; Cystadenoma, Papillary ; Diagnosis ; Diagnosis, Differential ; Epithelial Cells ; Female ; Humans ; Mucins* ; Parotid Gland* ; Salivary Glands

PURPOSE: To analyze the clinical and radiologic findings and to evaluate the treatment and prognosis of epithelial tumor patients. METHODS: We retrospectively reviewed clinical and radiologic findings of 36 patients who had been histopathologically diagnosed with epithelial tumors of the lacrimal gland after biopsy and surgery at Seoul St. Mary's Hospital from May 2005 to October 2012. RESULTS: Among the patients with epithelial tumors of the lacrimal gland based on histopathological findings, there were 21 cases of pleomorphic adenoma, seven cases of dacryops, four cases of adenoid cystic carcinoma, two cases of adenocarcinoma, one case of benign oncocytoma, and one case of mucoepidermoid carcinoma. The characteristic clinical finding of epithelial tumors of the lacrimal gland was proptosis (52.7%). In contrast with benign epithelial tumors of the lacrimal gland, the characteristic clinical findings of malignant epithelial tumors of the lacrimal gland were limitation of motion (57.1%), diplopia (57.1%), ocular pain (42.9%), and decreased visual acuity (42.9%). The mean duration of the symptoms of malignant epithelial tumors (5.0 +/- 4.2 months) was shorter than that of benign epithelial tumors (11.2 +/- 11.1 months) (t-test, p = 0.034). In radiologic CT and MRI findings, there was minimal bony destruction in two cases of pleomorphic adenoma and calcification in one case of pleomorphic adenoma. Malignant epithelial tumors of the lacrimal gland, in contrast to benign tumors, showed characteristic bony destruction (57.1%), poorly marginated tumor outline (42.9%) and calcification (14.3%). The 57.1% of patients in this study with malignant tumors were treated with chemotherapy and radiotherapy after surgical treatment, and there was one case (14.3%) of recurrence after treatment. CONCLUSIONS: Careful analysis of clinical and radiologic findings can lead to early diagnosis of malignant tumors.
Adenocarcinoma ; Adenoma, Oxyphilic ; Adenoma, Pleomorphic ; Biopsy ; Carcinoma ; Carcinoma, Adenoid Cystic ; Carcinoma, Mucoepidermoid ; Diplopia ; Drug Therapy ; Early Diagnosis ; Exophthalmos ; Humans ; Lacrimal Apparatus* ; Magnetic Resonance Imaging ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Seoul ; Visual Acuity

Glandular odontogenic cysts (GOCs) are rare intrabony solitary or multiloculated cysts of odontogenic origin. The importance of GOCs lies in the fact that they exhibit a propensity for recurrence similar to keratocystic odontogenic tumors and that they may be confused microscopically with central mucoepidermoid carcinoma. Thus, the oral and maxillofacial radiologists play an important role in definitive diagnosis of GOC based on distinctive cases; though they are rare. In large part, this is due to the GOC's complex and frequently non-specific histopathology. This report describes a case of GOC occurrence in the posterior mandibular ramus region in a 17-year-old female, which is a rare combination of site, age, and gender for occurrence.
Adolescent ; Carcinoma, Mucoepidermoid ; Cone-Beam Computed Tomography ; Diagnosis ; Female ; Humans ; Odontogenic Cysts* ; Odontogenic Tumors ; Radiography, Panoramic ; Recurrence

Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid gland is a very rare carcinoma. We herein report on a case of SMECE with metastasis to bilateral lateral neck nodes, esophagus, and trachea. A 72-year-old woman presented with a neck mass found incidentally during a medical check-up. She had a history of cerebral stroke without sequelae 20 years ago and was taking aspirin regularly. The neck mass was confirmed as papillary thyroid carcinoma by fine needle aspiration biopsy. The patient underwent bilateral total thyroidectomy with central compartment and bilateral compartment lateral neck dissection. The right recurrent laryngeal nerve was sacrificed due to tumor invasion. The trachea wall and esophagus were also invaded by the cancer. Histologically, the tumor showed dense fibrohyaline stroma and a goblet cell, nested islands of squamoid cells, and marked stromal eosinophilia with perineural invasion and lymphovascular invasion, confirming the diagnosis of SMECE. After radiation therapy for three months, distant metastasis to the liver, lung, and bone were found on PET-CT. This case appears to be more aggressive than previously reported cases.
Aged ; Aspirin ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Mucoepidermoid* ; Diagnosis ; Eosinophilia* ; Esophagus ; Female ; Goblet Cells ; Humans ; Islands ; Liver ; Lung ; Neck ; Neck Dissection ; Neoplasm Metastasis* ; Recurrent Laryngeal Nerve ; Stroke ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Trachea