Salivary Gland Anlage Tumour

Salivary gland anlage tumours are rare tumours of neonates that arise from minor salivary glands of the nasopharynx and are also known as congenital pleomorphic adenoma [13, 38, 73]. The firm, polypoid, pedunculated midline masses can reach up to 3 cm in size and cause respiratory distress and feeding problems. Salivary gland anlage tumours are multinodular and usually solid tumours. Histologically, they are characterised by an epithelial proliferation imitating embryonic salivary glands (Fig. 6.2). The epithelial proliferation can be extensive with solid squamous areas with focal keratini-sation, keratinised nests, cyst and pearls. Calcification within the cysts occurs. Salivary gland anlage tumours may contain branching ductal and glandular structures, occasionally with complex intraluminal papillations. The surrounding stroma may be loose and myxoid with numerous inflammatory cells, but may also show some fibrosis. Other salivary gland anlage tumours consist predominantly of densely packed sheet and nodules of small fusiform spindle cells with occasional regular mitoses. Rare keratinised duct and cystic structures are seen in these areas. Haemorrhage and focal necrosis can be seen [125]. Immunohistochemically, all cells stain positive for salivary gland amylase. The spindle cells are immunoreactive with antibodies to vimentin, cytokera-tins, EMA and smooth muscle actin, but are negative for S-100 and GFAP. The epithelial structures stain for cy-tokeratin and EMA. The exact classification of the salivary gland anlage tumour as a hamartoma or true (benign) neoplasm has not yet been resolved. Dehner and colleagues favour a hamartomatous origin, although the present name indicates a tumourous growth [38].

Hairy polyps (or dermoids) are found in the naso- and oropharynx of neonates or young infants. About 60% of the roughly 140 reported cases arose as single, pedunculated or sessile, 0.5 to 6 cm polyps in the lateral vault of the nasopharynx near the Eustachian tube orifice and very rarely within the Eustachian tube and middle ear [17, 78, 109]. The remaining cases occurred in the tonsillar region (for tonsillar and bilateral location see Sect. 6.3.2). Hairy polyps cause respiratory distress or feeding problems. Simultaneous congenital abnormalities such as cleft palate are more common than ipsilat-eral branchial sinus, congenital atresia of the carotid artery, osteopetrosis and malformations of the auricle [36, 68]. Treatment is simple surgical resection. A hairy polyp is covered by keratinised or glycogenated squamous epithelium containing hair with sebaceous glands and sweat glands. The mesenchymal core consists of mature fibroadipose tissue and blood vessels with oc casional smooth muscle and striated muscle fibres. The stalk may contain foci or plates of hyaline cartilage [27]. Hairy polyps fulfil the definition of a choristoma, although the literature refers to hairy polyps mistakenly often as "teratoma".

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