Non-Neoplastic Disorders of Salivary Glands: Overview and Clinical Presentations

1. Non-neoplasticdisordersof salivary glands Assoc. Professor Jan Laco, MD, PhD

2. Congenitaldisorders • very rare • agenesis • congenital polycystic degeneration of parotis • girls, childhood, fluctuating swelling • congenital sialectasia of parotis • anomalies of main excretory ducts • accessory gland • ectopic gland (Stafne bone cavity)

3. Acutebacterialsialadenitis • streptococci A, staphylococci, anaerobs • routes • ascendent from oral cavity (sialodochitis  sialadenitis) • hematogennous (in sepsis) • lymphogennous (from intra- and peri-parotid LN) • predisposition • poor oral hygiene , inflammation in oral cavity, hyposialia • drugs: tricyclic antidepressants • clinically • painful swelling, reddening f skin, pus discharge from duct • Mi: acute purulent sialadenitis + abscesses

4. Acutebacterialsialadenitis • treatment: ATB, event. absces drainage • CAVE!!! • dehydration, cachexia, terminal status in malignancies • Kussmaul´s disease (sialodochitis fibrinosa) • “signum mali ominis“ • acute postoperative sialadenitis 30% mortality !

5. Viralsialadenitides • sialotropic viruses • hematogennous spread during viremia • clinically evident sialadenitis • persistent infection  reactivation • in saliva only (polio, lyssa, influenza, …) • Mi: interstitial non-purulent inflammation • dg.: serology

6. Parotitis epidemica • “mumps“ • paramyxovirus, childhood • air droplets  upper airways + LN  blood – viremia  salivary glands • incubation period cca 18-21 days • clinically (up to ½ cases are inapparent !) • headache • uni- / bi-lateral swelling of parotis, event. submandibularis • symptoms for 10 days x infectious for 4-6 weeks ! • lifelong immunity

7. Parotitis epidemica • complications • in adults • 10-30 % orchitis (bilateral  sterility?) • oophoritis • pancreatitis • meningoencephalitis • treatment: symptomatic • vaccination

8. Cytomegalovirussialadenitis • children • transplacental • per partum by cervical mucus • adults • immunosupression • generalized infection • saliva, transplantation, dialysis • + CMV hepatitis, myocarditis, polyradiculoneuritis • Mi: intranuclear inclusions in ductal epithelium “owl eye“

9. HIV in salivary glands • children (20 %), almost pathognomic for HIV ! • pathogenesis unclear • clinically • parotis swelling (in 60% bilateral), xerostomia • micro: • lymphoepithelial sialadenitis CD8+ (incl. LE islands) + lymphoid follicles • multiple lymphoepithelial cysts • diff. dg.: Sjögren´s syndrome

10. Chronicsialadenitides • chronic recurrent sialadenitides • chronic sclerosing sialadenitis • obstructive sialadenitis • pneumoparotitis • postactinic sialadenitis • specific sialadenitides • lymphoepithelial sialadenitis / Sjögren´s syndrome • sarcoidosis

11. Chronicrecurrentsialadenitides • from acute phase x de novo • parotis • changes of ducts • congenital, immunologic, postinfectious, obstruction • hyposialia + dyschylia + microliths • microscopy • lymphocytic inflammation + fibrosis + acinar atrophy • attack (7-10 days) + remission (months); trigger ???

12. Chronicrecurrentsialadenitides • children (Payen) • 3rd year  peak 7th-10th year  spontanneous regression • painful • adults • dg.: sialography – sialectasias of small ducts • treatment: rather conservative

13. Chronicsclerosingsialadenitis • Küttner´s “tumor“ (1896) • middle-aged and old patients • submandibular gland • clinically (diff. dg. malignancy) • bilateral x unilateral; focal x diffuse • firm painless swelling • microscopy • cellular fibrosis, lymphoplasmacellular inflammation, phlebitis • member of “IgG4-related disease“ !!! • treatment: corticosteroids, event. exstirpation

14. Obstructivesialadenitis • obstruction of ducts / papilla • sialolithiasis (most common) • tumors • injury • MSG – compression by dentures, smokers

15. Pneumoparotitis • increase of intraoral pressure • norm 3 mmHg; smt. up to 150 mmHg !!! • glassblowers, trumpeters • OC  Stenon´s duct  parotis  constriction of facial muscles • event. subcutaneous emphysema • clinically • crepitations upon swollen parotis + air bubbles in saliva

16. Postactinicsialadenitis • complication of radiotherapy of HNSCCs • most vulnerable serous acini • hyposialia • 15 Gy – 50% of normal • 60 Gy – 10% of normal • risk factor for ascendent bacterial sialadenitis • MSG – xerostomia  carries  osteomyelitis • treatment: symptomatic, artificial saliva

17. Specificsialadenitides • tuberculosis • extremely rare • lymphogennous from oral cavity • --------------------------- • actinomycosis

18. Lymphoepithelialsialadenitis • = benign lymphoepithelial lesion (BLL), myoepithelial sialadenitis (MESA), Mikulicz´s disease (1892 ?) • Johann Freiherr von Mikulicz-Radecki (1850-1905) • x Mikulicz´s syndrome = swelling • + lymphoma, leukemia, … • parotis – 80-90% • clinically ± SS

19. Lymphoepithelialsialadenitis • microscopy • lymphoplasmacellular inflammation (CD4+) around small dilated ducts (MALT-like)  proliferation of basal cells  lymphoepithelial island = almost pathognomonic finding • variable acinar atrophy  xerostomia • complications • extranodal marginal B-cell lymphoma (MALT lymphoma) – risk up to cca 20x higher (after7-10 years)!!! • carcinoma from lymphoepithelial islands • malignant lymphoepithelial lesion • Inuits • undifferentiated carcinoma

20. Sjögren´s syndrome • Henrik Samuel Conrad Sjögren (1899-1986) • = sicca syndrome, keratoconjunctivitis sicca • clinical syndrome: xerostomia ± xerophthalmia • middle-aged females (F : M …3-10 : 1) • variable involvement of all exocrine glands • autoimmune disease • Ab x ribonucleoprotein (RNP) • primary (40 %) • secondary (60 %) • + RA (10 % pts), SLE (30 % pts), sclerodermia, vasculitides

21. Sjögren´s syndrome • clinically in OC • difficult eating, swalloving, speaking • dry red mucosa • frequent infections, Candida • carries • risk of acute purulent parotitis • hyposialia • clinically in eyes • decrease production of tears, keratoconiunctivitis • sialography – sialectasias • laboratory findings • ABs anti-RO (SS-A) and anti-La (SS-B); RFs

22. Sjögren´s syndrome • microscopy • like lymphoepithelial sialadenitis • diagnostics: clinical findings + microscopy • labial MSG – so-called foci • treatment • artificial saliva, cholinesterase inhibitors • hygiene, fluorids and chlorhexidin locally • artificial tears • risk of MALT lymphoma !!! • SG damage is permanent !!!

23. Differential diagnosticsof xerostomia • organic causes • Sjögren´s syndrome • radiotherapy • HIV infection • sarcoidosis • amyloidosis • functional causes • dehydration • psychogennic (depression) • side effect of drugs

24. Sialolithiasis • middle-aged males (M : F … 2 : 1) • submandibular gland (85%)  parotis (6-10%) • due to anatomy of Wharton´s duct • chemical composition of saliva • intraductal close to orifice (50%)  intraductal  intraparenchymatous • ethiology • changes in chemical composition of saliva due to infection and inflammation, Ca salts + nidus • clinically • salivary colic x asymptomatic • treatment – incision of duct orifice, exstirpation

25. Sialadenosis • non-inflammatory hypertrophy and hyperplasia of SG • disorder of innervation  dyschylia • metabolic, endocrine, neurogennic disorders • malnutrition • alcoholism, liver cirrhosis • diabetes mellitus • dysfunction of thyroid gland • dysfunction of adrenal cortex • pregnancy, menopause • bulimia • drugs (antihypertensives, β-sympatomimetics, psycho-drugs)

26. Sialadenosis • bilateral painless swelling of parotis • recurrent x permanent • microscopy – 3x increased acini x NO inflammation • treatment ?

27. Cysts and fistulas • lymphoepithelial cyst • from epithelial inclusions in LN • middle-aged patients • unilateral, solitary • slowly growing painless swelling • x acute pain if secondary infection • microscopy • squamous / respiratory epithelium + lymphoid tissue in the wall

28. Cysts and fistulas • retention duct cysts • old males • well circumscribed painless swelling in parotis • blockage of the duct by detached epithelial cells • traumatic cysts / pseudocysts • mucoceles / ranulas • preauricular + cervicoaural fistulas

29. Necrotizingsialometaplasia • spontannous x iatrogennic microtraumata • middle-aged males, smokers • MSG of OC (hard palate), of nasal cavity, of larynx • ischemic necrosis (focal x diffuse)  inflammation + reparation + squamous metaplasia of acini and ducts • clinically and microscopically mimics SCC !!! • painless ulcer about 2 cm with irregular margins • spontanneous healing within -2 months

30. Adenomatoid “hyperplasia“ • rare • middle-aged males • painless swelling of MSG on hard palate • clinically mimics tumor • microscopy • normal MSG, NO inflammation