The authors analyzed retrospectively the 36 patients with malignant salivary gland tumors who were treated at Department of Oral and Maxillofacial Surgery, Pusan National University Hospital from February, 1989 to September, 1997. The results obtained were as follows: 1. There were 14 males( 38.9%) and 22 females(61.1%). The peak age of patients with major salivary gland tumors was the 6th decade, but with minor salivary gland was the 5th decade. 2. Of all salivary gland tumors, 5 cases arose in the parotid glands, 2 cases in the submanibular glands, 1 case in the sublingual gland and 28 cases in the minor salivary glands. 3. The incidence according to the anatomic primary site for minor salivary glands was 15 cases in the palate, 5 in the floor of mouth and 2 cases each arising in the tongue, lip, retromolar area and buccal mucosa. 4. Of all salivary gland tumors, adenoid cystic carcinoma was 5 cases in the major salivary glands and 8 in the minor salivary glands. mucoepidermoid carcinoma was 2 cases in the major salivary glands and 14 in the minor salivary glands and others were 2 cases of adenocarcinoma, 4 malignant mixed tumors and 1 undifferentiated carcinoma. 5. The incidence of cervical lymph node metastasis was 100%(2/2) in the submandibular glands, 80%(4/5) in the floor of mouth, 50%(1/2) in the tongue and 20%(1/5) in the parotid glands. The highest incidence of lymph node metastasis according to histopathological classfication was found in the high grade of mucoepidermoid carcinoma and tubular type of adenoid cystic carcinoma. 6. Nerve invasion was common in the adenoid cystic carcinoma. 7. The lung was the commonest site for distant metastasis comprising 7 cases among 7 cases in which distant spread occurred.
Adenocarcinoma ; Busan ; Carcinoma ; Carcinoma, Adenoid Cystic ; Carcinoma, Mucoepidermoid ; Humans ; Incidence ; Lip ; Lung ; Lymph Nodes ; Mixed Tumor, Malignant ; Mouth Floor ; Mouth Mucosa ; Neoplasm Metastasis ; Palate ; Parotid Gland ; Retrospective Studies ; Salivary Glands* ; Salivary Glands, Minor ; Sublingual Gland ; Submandibular Gland ; Surgery, Oral ; Tongue

The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.
Basal Cell Nevus Syndrome* ; Crowns ; Dentigerous Cyst ; Genes, Dominant ; Humans ; Hypertelorism ; Jaw Cysts ; Nevus ; Odontogenic Cysts* ; Tooth, Unerupted