Cases reported "Salivary Gland Neoplasms"

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1/62. family with low-grade neuroendocrine carcinoma of salivary glands, severe sensorineural hearing loss, and enamel hypoplasia.

    Four sibs in a family on the Isle of Man, two brothers and two sisters ranging in age from 33 to 45 years, presented with low-grade malignant tumors of the submandibular gland in three cases and of the nasal cavities and maxillary sinuses in one. The neoplasms were all of the same histological type, apparently hitherto undescribed, showing well-differentiated neoplastic ducts, surrounded by neoplastic myoepithelial cells, together with sheets of epithelial cells expressing neuroendocrine markers by immunohistochemistry. Cervical neck node metastases have developed in all four cases. In the sib with a primary sinonasal neoplasm, widespread bloodstream metastases also became manifest and a single such metastasis in his brother. All four sibs have severe enamel hypoplasia and the same lesion is present in 5 of their 11 children. In the two male patients, severe sensorineural hearing loss has developed in adult life, unilateral in the left ear in one brother, bilateral in the other. In the brother with bilateral sensorineural hearing loss, magnetic resonance imaging revealed a vestibular schwannoma on the left side, which is currently under treatment. The inherited hearing loss is thought to be unilateral in this case also.
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2/62. The place of irradiation in the treatment of malignant tumors of the salivary glands.

    1. radiation therapy is not indicated after surgical removal with adequate margins of low-grade tumor. 2. radiation therapy is indicated with a) Inadequate surgical margins in low-grade tumors b) All high-grade tumors c) All recurrent malignant tumors 3. Irradiation of nerve pathways is indicated with demonstrated nerve and perineural invasion and/or with adenoicystic carcinoma. 4. Irradiation of the entire ipsilateral neck is indicated a) Wtih high-grade tumors unless radical neck dissection shows negative nodes b) In the place of radical neck dissection.
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3/62. Pleomorphic adenoma of the nasal septum.

    Pleomorphic adenoma is the commonest benign tumour of the major salivary glands. It can also occur in minor salivary glands, mainly in the oral cavity, but also in other sites in the head and neck both within and outwith the upper aerodigestive tract. We present a rare case of pleomorphic adenoma of the nasal septum with consideration of the clinical management and a review of the literature.
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4/62. Intraductal papilloma of the salivary gland. A report of two cases with diagnosis by fine needle aspiration biopsy.

    BACKGROUND: Intraductal papillomas are rare, benign tumors most commonly encountered in minor salivary glands. They are cystic, solitary neoplasms that arise from ductal epithelium and produce painless swellings. CASES: Two cases arose in major salivary glands. The first case was a superficial, firm mass at the superior edge of the parotid, cytologically evocative of an adnexal tumor. A firm, submandibular mass in the second case was diagnosed as a papillary neoplasm. Fluid was aspirated from both cases. Three-dimensional epithelial clusters, some with a papillary configuration and histiocytes, were the main cellular components. The majority of cells showed oncocytic differentiation; however, benign-appearing ductal cells in honeycomb sheets were also present. The first case also had occasional cells suggestive of sebaceous differentiation. The excised lesions were unilocular cystic papillary neoplasms consistent with intraductal papilloma; focal sebaceous differentiation was noted in the first case. CONCLUSION: awareness of the cytologic features of intraductal papilloma of the salivary glands should prompt its inclusion in the differential diagnosis of papillary lesions of the head and neck.
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5/62. Vallecular acinic cell carcinoma in a 9-year-old girl: report of an unusual case.

    An unusual case of acinic cell tumour of the vallecula is presented. Acinic cell carcinoma occurs usually in the major salivary glands. Minor salivary gland location is unusual and vallecular origin exceptional. This peculiar histologic tumour should now be classified as an low grade carcinoma and adequate treatment has to be initiated. The patient, a 9-year-old girl, had undergone a suprahyoid access for total tumor removal with a bilateral neck exploration. Postsurgical radiotherapy has to be done in case with perineural invasion, invaded margins, node invasion or high grade tumor. The clinical and histopathological findings are discussed in the light of the literature.
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6/62. liposarcoma involving the periodontal tissues. A case report.

    Liposarcomas constitute 15 to 20% of all soft tissue tumors. They are extremely rare in the head and neck and in the oral cavity. A 30-year-old patient was seen for a soft, painless mass in the right palate. Through panoramic radiography it was possible to observe a radiolucent area with sharp margins in the right upper quadrant. The lesion, after an incisional biopsy, was diagnosed as a "myxoid liposarcoma." The patient underwent a wide excision of the lesion with bone laminectomy and he is well at a 4-year follow-up. The differential diagnosis included salivary gland tumors and palatal abscess.
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7/62. Turban tumour with involvement of the parotid gland.

    Familial autosomal dominant cylindromatosis (FADC, turban tumour syndrome, Brooke-Spiegler-syndrome and many more, MacKusick catalogue numbers 123850, 313100) is a rare hereditary disease usually presenting in the second or third decade. With female preponderance dermal cylindromas predominantly arise in hairy areas of the body with approximately 90 per cent on the head and neck. Transformation to malignancy seems to be scarce. Although cylindromas of the skin resemble basal cell adenomas of the salivary gland, there is usually no salivary gland involvement. On the other hand, patients with basal cell adenomas of a salivary gland usually do not show dermal lesions. We report one of the rare cases of FADC combined with multiple basal cell adenomas of the parotid glands and present a review of the literature.
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8/62. Adenoid cystic carcinoma of the tongue.

    Malignant tumours of the salivary glands in children are extremely rare. We present here a 12-year-old girl initially diagnosed as pleomorphic adenoma on fine needle aspiration biopsy, and adenoid cystic carcinoma (ACC) after the lesion was excised and examined by histopathology. A wide resection of the lesion and bilateral supraomyohyoid neck dissection was performed. To our knowledge this is one of the youngest patients with ACC of the minor salivary glands. Due to its benign histological appearance, the biological agressiveness of ACC is usually underestimated. Although fine needle aspiration cytology (FNAC) is very valuable in diagnosis, cytological variations of pleomorphic adenoma must be considered. ACC of the tongue in a young age group should be treated with wide resection and selective neck dissection if the tumour is localized in places where the risk of metastasis is increased and if there is a clinically palpable lymph node. In such cases the clinician should not avoid radical operations even in a young patient.
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9/62. Pleomorphic adenoma of the pterygopalatine fossa: a case report.

    Pleomorphic adenoma (PA) is the most common benign tumor of the major salivary glands. It can also Occur in the minor salivary glands, mainly in the oral cavity, and in other sites in the head and neck region. We present a very rare case of PA in the pterygopalatine fossa. Surgical resection of the tumor was performed via the transmaxillary approach. The patient has experienced neither surgical complications nor recurrence in the past 3 years. This case suggests that a localized benign tumor in the pterygopalatine fossa can be removed safely and efficaciously via a transmaxillary approach.
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10/62. Unusual neck mass in an adolescent: benign basal cell adenoma of the minor salivary glands of the piriform sinus.

    With an incidence of less than 3%, salivary gland tumors are rare in the head and neck. The percentage of basal cell adenomas within the group of salivary gland tumors is even less (0.2-2%). Salivary gland tumors occur very rarely in adolescents. The basal cell adenoma commonly affects older persons and occurs most frequently in the major salivary glands. We present the unusual case of a benign basal cell adenoma of the minor salivary glands of the piriform sinus in a 14 year old male patient. Unusual are the age of the patient, the histological type, size and localisation of the tumor.
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