BACKGROUND: Recent findings in molecular pathology suggest that genetic translocation and/or overexpression of oncoproteins is important in salivary gland tumorigenesis and diagnosis. We investigated PLAG1, SOX10, and Myb protein expression in various salivary gland neoplasm tissues. METHODS: A total of 113 cases of surgically resected salivary gland neoplasms at the National Cancer Center from January 2007 to March 2017 were identified. Immunohistochemical staining of PLAG1, SOX10, and Myb in tissue samples was performed using tissue microarrays. RESULTS: Among the 113 cases, 82 (72.6%) were benign and 31 (27.4%) were malignant. PLAG1 showed nuclear staining and normal parotid gland was not stained. Among 48 cases of pleomorphic adenoma, 29 (60.4%) were positive for PLAG1. All other benign and malignant salivary gland neoplasms were PLAG1-negative. SOX10 showed nuclear staining. In normal salivary gland tissues SOX10 was expressed in cells of acinus and intercalated ducts. In benign tumors, SOX10 expression was observed in all pleomorphic adenoma (48/48), and basal cell adenoma (3/3), but not in other benign tumors. SOX10 positivity was observed in nine of 31 (29.0%) malignant tumors. Myb showed nuclear staining but was not detected in normal parotid glands. Four of 31 (12.9%) malignant tumors showed Myb positivity: three adenoid cystic carcinomas (AdCC) and one myoepithelial carcinoma with focal AdCC-like histology. CONCLUSIONS: PLAG1 expression is specific to pleomorphic adenoma. SOX10 expression is helpful to rule out excretory duct origin tumor, but its diagnostic value is relatively low. Myb is useful for diagnosing AdCC when histology is unclear in the surgical specimen.
Adenoma ; Adenoma, Pleomorphic ; Antibody-Dependent Cell Cytotoxicity ; Carcinogenesis ; Carcinoma, Adenoid Cystic ; Diagnosis ; Immunohistochemistry ; Oncogene Proteins ; Oncogene Proteins v-myb ; Parotid Gland ; Pathology, Molecular ; Salivary Gland Neoplasms ; Salivary Glands ; SOX Transcription Factors ; Translocation, Genetic

Canalicular adenoma is a rare benign tumor that arises in the minor salivary gland. Clinically, it usually presents as an asymptomatic nodule on the upper lip or buccal mucosa. It is necessary to histopathologically differentiate canalicular adenoma from basal cell adenoma and adenoid cystic carcinoma. Canalicular adenoma shows tubular structures composed of 1 or 2 layers of columnar cells and intraluminal hemorrhage. Squamous morules, which seem to represent metaplasia, are a unique finding for this tumor. Immunohistochemical staining is often helpful for the diagnosis of canalicular adenoma, which is positive for S-100 and CK (AE1/3). Herein, we report a case of canalicular adenoma in a 71-year-old man who presented with an asymptomatic nodule on the upper lip. As far as we know, this is the first report of canalicular in Korean Dermatology Journal.
Adenoma* ; Aged ; Carcinoma, Adenoid Cystic ; Dermatology ; Diagnosis ; Hemorrhage ; Humans ; Lip* ; Metaplasia ; Mouth Mucosa ; Salivary Glands, Minor

Salivary adenoid cystic carcinoma (SACC) is a common malignant tumor in the oral and maxillofacial region and accounts for approximately 3%-5% of all head and neck carcinomas. SACC always occurs in the palatal salivary gland and parotid gland. The tumor has the characteristics of strong invasion, perineural invasion, high hematogenous metastasis, and low lymph node metastasis rate. The biological characteristics of SACC determine the specificity of clinical treatment. Thus far, few clinical trials have investigated the efficacy of systemic therapy owing to the rarity of SACC with lung metastasis. Moreover, long-term results are poor, and no consensus on standard treatment has been reached yet. This systematic review aims to provide a retrospective analysis of treatment options and prognosis for SACC with lung metastasis and evidence for future clinical treatment.
Carcinoma, Adenoid Cystic ; diagnosis ; Cell Line, Tumor ; Humans ; Neoplasm Invasiveness ; Prognosis ; Retrospective Studies ; Salivary Gland Neoplasms ; diagnosis

Adenoid cystic carcinoma (ACC) is a malignant neoplasm of glands commonly occurs in salivary glands. Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of ACC that primarily presents on the skin. Herein, we represent a rare case of PCACC occurred in the umbilicus in a 66-year-old Korean male patient. The patient visited our center with erythematous indurated patch on the umbilicus diagnosed as ACC by incisional biopsy at another center. The diagnosis of PCACC was confirmed by additional histopathologic examination and imaging study. We proceeded Mohs micrographic surgery and reconstructed umbilicus with tacked purse string suture. Local recurrence and distant metastasis were not observed during 30-month follow-up. We report this rare case of PCACC on the umbilicus so that dermatologist can aware of the rare disease. Furthermore, we recommend MMS and tacked purse string suture as effective methods for treatment of PCACC and immediate umbilical reconstruction.
Adenoids ; Aged ; Biopsy ; Carcinoma, Adenoid Cystic ; Diagnosis ; Follow-Up Studies ; Humans ; Male ; Mohs Surgery ; Neoplasm Metastasis ; Rare Diseases ; Recurrence ; Salivary Glands ; Skin ; Sutures ; Umbilicus

PURPOSE: Orbital exenteration is a psychologically and anatomically disfiguring procedure which indicated in some patients with malignant or progressive diseases of orbital and periorbital area. In this study, we reviewed 176 patients that underwent orbital exenteration. METHODS: This was a retrospective study of medical records from all patients who underwent orbital exenteration from March 1991 to March 2014 in oculoplastic department at an eye care center. Demographic data, diagnosis, site of primary involvement and technique of surgery were determined in patients. RESULTS: One hundred seventy-six cases of orbital exenteration were included that had documented histopathology. The age of patients ranged from 1 to 91 years (mean age ± standard deviation, 55.43 ± 27 years). Ninety-seven (55.11%) males and 79 (44.88%) females were included. Fifteen different tumors were identified. The most common indication was patients with basal cell carcinoma 49 (28%) followed by 41 (23.5%) squamous cell carcinomas, 35 (20%) retinoblastoma, and 13 (7%) adenoid cystic carcinomas. In total, adnexal malignancies were the most common tumors, secondarily involving the orbit. Eyelids 89 (50.5%) and the globe 43 (24%) were the most frequent site of involvement. Three types of exenteration were performed, based on available data of 129 operation sheets, 46 (35.7%) subtotal, 62 (48.1%) total, and 21 (16.3%) cases of extensive exenterations. In total 97 cases were evaluated pathologically for perineural involvement, of which perineural invasion was noted in 9 (7%) reports. CONCLUSIONS: Frequency of exenteration in our center has increased in past 3 years and the majority of cases were eyelid basal cell carcinoma. Patient education considering periocular lesions can help in earlier diagnosis of malignant lesions and therefore reducing the number of exenteration.
Carcinoma, Adenoid Cystic ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Diagnosis ; Eyelids ; Female ; Humans ; Male ; Medical Records ; Orbit ; Patient Education as Topic ; Retinoblastoma ; Retrospective Studies

Here we reported a case of bronchial adenoid cystic carcinoma from Peking University Third Hospital. A 40-year-old female presented with dry cough for 1 year and nocturnal paroxysmal attacks of wheezing for 4 months. She was a non-smoker, and did not have past histories of asthma or allergy. On physical examination, no stridor, wheezing and cyanosis were present and the general appearance was good. The results of the laboratory analysis, including blood eosinophils count, immunoglobulin E level and chest X-ray were normal. Spirometry revealed reversible airflow obstruction, and post-bronchodilator forced expiratory volume in one second (FEV1) showed an increase of 12% and 230 mL from baseline. Bronchial asthma was diagnosed, however, she responded poorly despite the adequate anti-asthma therapy including high dose inhaled corticosteroid plus long-acting beta2-agonist, theophylline and montelukast. Then chest computed tomography (CT) was performed which showed a polypoid mass occupying the lumen of left main bronchus. Then the bronchoscopy revealed a polypoid endo-bronchial mass arising from the left main bronchus, causing subtotal obstruction of the lumen. Biopsy was carried out through the bronchoscopy, the pathological findings showed characteristic cribriform and tubular pattern which was formed by two-layered cells with ductal and myoepithelial phenotypes, which were consistent with adenoid cystic carcinoma. Re-examining the patient, the lung was clear without any wheeze when she was seated. However, inspiratory wheeze was heard in her left upper lung when she was supine, and disappeared after sitting up again. Subsequently the patient underwent a resection surgery. At the operational site, the tumor was seen on the anterolateral wall of the left main bronchus, without submucosally expanding histologically. Therefore, a sleeve resection surgery of the left main bronchus was performed. Following surgery, chest CT scan revealed complete resolution of the tumor. Her symptoms improved significantly, as did her pulmonary function tests, although all the medicines for asthma were stopped. Now, two years after the operation, the patient remained asymptomatic, and spirometry was performed again which showed normal completely. The presenting case report emphasizes the fact that not all wheezes and reversible airflow obstruction are asthma. It is critical to bear in mind that if a "difficult asthma" patient does not respond to appropriate anti-asthma therapy; localized obstructions should be differentiated.
Adrenal Cortex Hormones ; Adult ; Asthma/diagnosis* ; Biopsy ; Bronchi ; Bronchoscopy ; Carcinoma, Adenoid Cystic/diagnostic imaging* ; Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms/diagnostic imaging* ; Radiography ; Respiratory Sounds ; Tomography, X-Ray Computed

Adenoid cystic carcinoma (ACC) of the sublingual gland is an extremely rare neoplasm. The clinicopathological characteristics of ACC are slow-growing swelling with or without ulceration, perineural spread, local recurrence, and distant metastasis. This report describes a 58-year-old male who had a slowly growing swelling without ulceration on the right side of the mouth floor that had been present for 1 month. In a radiological examination, the mass showed multilocular cystic features and no bony or tongue muscle invasion. No enlarged cervical lymph nodes were detected. Excisional biopsy and histological analysis showed that the lesion was ACC. In addition to reporting a rare case of ACC, this report also discusses the differential diagnosis and treatment of ACC with a review of the relevant literature.
Adenoids* ; Biopsy ; Carcinoma, Adenoid Cystic* ; Diagnosis, Differential ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Mouth Floor ; Neoplasm Metastasis ; Recurrence ; Salivary Glands ; Sublingual Gland* ; Tongue ; Ulcer

Central malignant salivary gland tumor of the mandible is rarely observed with adenoid cystic carcinoma, which only comprises a small proportion of cancer patients. In this study, a patient with central adenoid cystic carcinoma of the mandible is presented, and relevant literature is reviewed.
Carcinoma, Adenoid Cystic ; diagnosis ; Humans ; Mandible ; pathology ; Salivary Gland Neoplasms ; diagnosis

No abstract available.
Bone Marrow/*pathology/radiography/radionuclide imaging ; Carcinoma, Adenoid Cystic/*diagnosis ; Eye Neoplasms/*diagnosis ; Female ; Humans ; Lacrimal Apparatus/*pathology/radiography/radionuclide imaging ; Lacrimal Apparatus Diseases/*diagnosis ; Maxilla ; Middle Aged ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; *Positron-Emission Tomography ; *Tomography, X-Ray Computed

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