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Assoc. Professor Jan Laco, MD, PhD

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1 Assoc. Professor Jan Laco, MD, PhD
Salivary gland tumors Assoc. Professor Jan Laco, MD, PhD

2 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)

3 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

4 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

5 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

6 Diagnostics clinical features ultrasonography FNAC extirpation
!!! definite diagnosis and assessment of surgical margins - pathologist !!!

7 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

8 Myoepithelioma 1.5 % SG tumors clinical features similar to PA
myoepithelial cells spindle, plasmacytoid, epithelioid, clear recurrence ( PA) malignant transformation

9 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)

10 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

11 Oncocytoma 1 % SG tumors predominant oncocytes + clear cell variant
oncocytosis older patients acinar / ductal disperse / focal / multifocal

12 Cystadenoma 4-5 % SG tumors, more common in MSG multicystic (80 %)
+ oncocytic variant papillary x mucinous variants

13 Ductal papillomas 1. inverted ductal papilloma
lower lip, cheek, 2 cm 2. intraductal papilloma lips, cheek, 2 cm 3. sialoadenoma papilliferum palate recurrence – % !!!

14 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

15 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%

16 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

17 Polymorphous (low-grade) adenocarcinoma
common MSG malignant tumor palate (60 %) painless ulcer cytologically uniform x polymorphous architecture dif. dg. ACC relatively good prognosis

18 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

19 Carcinosarcoma extremely rare
malignant transformation of both epithelial and mesenchymal PA component

20 Metastasizing PA microscopically like PA
dg. in retrospect in case of metastasis skeleton, lungs, lymph nodes variable interval (1-55 years)

21 Epithelial-myoepithelial carcinoma
1 % SG tumors, females epithelial cells (ducts) + rim of clear myoepithelial cells recurrence – 40 %

22 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

23 Newly described entities and tumors
Sclerosing polycystic adenosis Secretory carcinoma (MASC) Cribriform adenocarcinoma of MSG/tongue

24 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

25 Mesenchymal tumors hemangioma congenital, childhood lipoma

26 Hematologic malignancies
parotid (75 %), older patients MALT lymphoma extranodal marginal zone B-cell lymphoma background of lymphoepithelial sialadenitis (SS) good prognosis FL, MCL, DLBCL

27 Secondary tumors = metastases 5 % malignancies
parotid + intra-/peri-glandular LN carcinomas of lungs, breast, kidney squamous cell carcinoma melanoma

28 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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