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Assoc. Professor Jan Laco, MD, PhD
Salivary gland tumors Assoc. Professor Jan Laco, MD, PhD
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General features incidence 0.4-13.5 (1.2) in 100,000
malignant SG tumors … 6 % head neck malignancies 55-80 % benign tumors parotis (superficial lobe; %) SM (7-11 %) MSG (palate; 9-23 %) SL adults – years; more common in females pleomorphic adenoma Warthin tumor mucoepidermoid carcinoma etiology: irradiation (Japan, RTG), smoking (WT)
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General features parotid submandibular MSG sublingual
78 % tumors of SG; 15 % malignant submandibular 12 % tumors of SG; 30 % malignant MSG 10 % tumors of SG; 45 % malignant sublingual 0.3 % tumors of SG; 86 % malignant
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Clinical features Benign tumor slow growth soft 85 % parotid tumors
NO skin ulceration NO paresis n. VII Malignant tumor rapid growth, acceleration ± firm 45 % MSG tumors ± skin ulceration ± bone invasion ± paresis n. VII
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Classification Benign tumors pleomorphic adenoma myoepithelioma
Warthin tumor basal cell adenoma oncocytoma cystadenoma ductal papillomas Malignant tumors mucoepidermoid carcinoma acinic cell carcinoma adenoid cystic carcinoma polymorphous (low-grade) adenocarcinoma carcinoma ex PA carcinosarcoma metastasizing PA epithelial-myoepithelial carcinoma salivary duct carcinoma
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Diagnostics clinical features ultrasonography FNAC extirpation
!!! definite diagnosis and assessment of surgical margins - pathologist !!!
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Pleomorphic adenoma “mixed tumor“, 75% of parotid tumors
slow growth (years), movable, ± multinodular, 2-5 cm epithelial component ducts, cords, tubuli, sheets, smt. keratinization mesenchymal-like component myxoid / chondroid, bone, fat, … recurrence (3-6%) – multifocal, myxoid myxoid consistence, variable tumor capsule thickness, often incomplete capsule, tumor cells within capsule incomplete removal malignant transformation (6 %) – carcinoma ex PA
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Myoepithelioma 1.5 % SG tumors clinical features similar to PA
myoepithelial cells spindle, plasmacytoid, epithelioid, clear recurrence ( PA) malignant transformation
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Warthin tumor “papillary cystadenoma lymphomatosum“
3.5% of SG tumors (locally up to 30 %!) almost exclusively in parotid; lower pole of superficial lobe multicentric (12-20 %); bilateral (5-15 %) smoking (8x increased risk); from LN inclusions ? painless swelling, 2-4 cm, ½-3 years encapsulated cystic nodule bilayered oncocytic epithelium (luminal + basal cells) lymphoid stroma metaplastic variant (7 % of WT)
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Basal cell adenoma 1-3 % SG tumors; parotid
basaloid cells + peripheral palisading 4 variants: solid trabecular tubular membranous – recurrence (25 %) !!! + skin cylindromas canalicular adenoma – upper lip
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Oncocytoma 1 % SG tumors predominant oncocytes + clear cell variant
oncocytosis older patients acinar / ductal disperse / focal / multifocal
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Cystadenoma 4-5 % SG tumors, more common in MSG multicystic (80 %)
+ oncocytic variant papillary x mucinous variants
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Ductal papillomas 1. inverted ductal papilloma
lower lip, cheek, 2 cm 2. intraductal papilloma lips, cheek, 2 cm 3. sialoadenoma papilliferum palate recurrence – % !!!
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Mucoepidermoid carcinoma
most common SG malignant tumor parotid (45 %) + SM + MSG (palate, cheek) epidermoid + mucinous + intermediate cells histopathological grade (WHO 2005): cystic component < 20% … 2 neural invasion … 2 necroses … 3 4 mitoses / 10 HPF … 3 anaplasia … 4 LG - 0-4; IG - 5-6; HG - >6 lymphogennous metastases; prognosis usually good
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Acinic cell carcinoma parotid (80 %) + MSG (17 %), 1-3 cm
acinic cell differentiation + other cell types ± lymphoid infiltrate (better prognosis ?) 4 variants: solid microcystic papillary-cystic follicular recurrence – 35%
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Adenoid cystic carcinoma
“cylindroma“ 10 % of SG tumors; parotid + SM + MSG ductal and myoepithelial (basaloid) differentiation perineural spread 3 variants: tubular cribriform (seeve-like) – most common solid – worse prognosis recurrence – up to 85% 5-year survival - 35% x 10-year survival – 10-20% lymphogennous + hematogennous (lungs) metastases
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Polymorphous (low-grade) adenocarcinoma
common MSG malignant tumor palate (60 %) painless ulcer cytologically uniform x polymorphous architecture dif. dg. ACC relatively good prognosis
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Carcinoma ex PA 4 % SG tumors; 12 % SG malignant SG tumors
complication of approx. 6 % PAs PA duration more than 3 years (usually to 10 years) 1-25 cm malignant transformation of epithelial PA component poorly differentiated adenocarcinoma (SDCa) classification intracapsular (non-invasive, in situ) – good prognosis minimally invasive – good prognosis invasive – poor prognosis
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Carcinosarcoma extremely rare
malignant transformation of both epithelial and mesenchymal PA component
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Metastasizing PA microscopically like PA
dg. in retrospect in case of metastasis skeleton, lungs, lymph nodes variable interval (1-55 years)
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Epithelial-myoepithelial carcinoma
1 % SG tumors, females epithelial cells (ducts) + rim of clear myoepithelial cells recurrence – 40 %
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Salivary duct carcinoma
9% of SG malignant tumors, old pts. similar to DIC of breast variants: spindle cell mucinous invasive micropapillary angioinvasion and perineural spread poor prognosis
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Newly described entities and tumors
Sclerosing polycystic adenosis Secretory carcinoma (MASC) Cribriform adenocarcinoma of MSG/tongue
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Genetics Mucoepidermoid carcinoma Adenoid cystic carcinoma
t(11;19) …CRTC3-MAML2 Adenoid cystic carcinoma t(6;9) … MYB-NFIB Secretory carcinoma t(12;15) … ETV6-NTRK3 Clear cell carcinoma t(12;22) … EWSR1-ATF1
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Mesenchymal tumors hemangioma congenital, childhood lipoma
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Hematologic malignancies
parotid (75 %), older patients MALT lymphoma extranodal marginal zone B-cell lymphoma background of lymphoepithelial sialadenitis (SS) good prognosis FL, MCL, DLBCL
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Secondary tumors = metastases 5 % malignancies
parotid + intra-/peri-glandular LN carcinomas of lungs, breast, kidney squamous cell carcinoma melanoma
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Tumors of childhood Sialoblastoma Salivary gland anlage tumor
very rare (23 cases), from blastematous cells? congenital / early childhood boys : girls (2 : 1); parotid recurrence (22 %), metastases (9 %) Salivary gland anlage tumor very rare (approx. 20 cases), embryologic development newborns, boys : girls (4 : 1) polypoid midline tumor in nasopharynx, 1-3 cm benign x suffocation !!!
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