Chaper 11: Salivary gland pathology Flashcards

1
Q
  • Von Ebner glands
    - Blandin-­‐Nuhn
A

-­‐ Von Ebner glands: located in tongue beneath circumvalate and foliate papillae. Primarly serous.
-­‐ Blandin-­‐Nuhn glands: located in ventral tongue, serous

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2
Q

Salivary gland aplasia

A

-­‐ Isolated or associated with Treacher Collins, hemifacial microsomia and lacrimo-­‐auriculo-­‐dental-­‐ digital syndrome (LADD)
-­‐ LADD: FGF10 mutation. SG/LG aplasia, cup-­‐shaped ears, and dental/digital anomalies

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3
Q

Mucocele

A

-­‐ Superficial mucoceles: in soft palate and retromolar pad
-­‐ Seen in lichen planus, lichenoid drug reactions, GVHD, and tartar control toothpaste
-­‐ Mucous extravasation often causes granulation tissue

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4
Q

Ranula

A

-­‐ Arises from sublingual gland (body or ducts of Rivini).
-­‐ Shows tail sign on CT/MRI (only if from Sub-­‐MD gland)
-­‐ Plunging ranula: spilled mucin dissects through mylohyoid muscle

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5
Q

Salivary duct cyst (mucous retention cyst, sialocyst)

A

-­‐True epithelium-­‐lined cyst, frequent on upper lip
– Major glands: Parotid glands = most common
– Minor glands: Floor of the mouth, buccal mucosa, lips; appear bluish depending on depth of cyst below surface
If in floor of mouth, often arise adjacent to submandibular ducts, sometimes have amber color
-­‐Ductal ectasia: cyst-­‐like dilatation of salivary duct due to ductal obstruction and increased intraluminal pressure (probably not a true cyst). Possibly multifocal=presents as painful nodules with dilated ductal orifices, with pus. Tx with erythromycin and chlorhexidine
-­‐ Cuboidal, columnar or squamous epithelium + ductal ectasia with oncocytic metaplasia (may contain papillary folds: if so, named papillary cystadenoma)

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6
Q

Sialolithiasis

A

-­‐ Most in SubMD (tortuous duct and mucoid secretion)
– Most common in young and middle-aged adults
* Major gland sialoliths most frequently cause episodic pain or swelling of affected gland, esp at mealtime

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7
Q

Sialadenitis

A

-­‐ Infectious (viral-­‐mumps; or bacterial-­‐S.aureus) or non-­‐infectious (Sjogrens, RT, sarcoidosis).
-­‐ Bacterial retrograde spread from reduced flow (dehydration, medication) or block (sialolith, tumor). Fever and pus present, usually parotid
-­‐ Kuttner tumor: chronic sclerosing sialadenitis in subMD leading to gland enlargement. Pts should be evaluated for sclerosing pancreatitis.
-­‐ Surgical mumps: after abdominal sx, when pt is kept w/o food/fluids and received atropine
-­‐ Sialography: sausaging of Stensen’s duct (dilation + strictures from scar tissue alternating)
-­‐ Subacute necrotizing sialoadenitis: no squamous metaplasia, mixed acute and chronic inflamm, younger patients, does not ulcerate-
– Juvenile recurrent parotitis = Most common inflammatory salivary disorder of children in US, recurring episodes of unilateral or bilateral, non-suppurative parotid swelling, Unknown cause,
Multiple recurrences but usually resolves around time of puberty

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8
Q

Cheilitis glandularis

A

-­‐ Swelling and eversion of** lower lip **due to hypertrophy and inflammation of glands.
-­‐ Possible etiologies: UV damage, tobacco, syphilis, poor hygiene and hereditary
-­‐ Forms: simple, superficial suppurative (Baelz’s disease) or deep suppurative (CG apostematosa)
-­‐ Small beads of fluid may be extruded with pressure. String of mucus may form
-­‐ 18-­‐35% of CG transform into SCC

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9
Q

Sialorhhea

A

-­‐ True sialorrhea: caused by apthae, new dentures, GERD (“water brash”), rabies, heavy metal poisoning (acrodynia) and medications (Alzheimer’s)
-­‐ Relative sialorrhea (can’t keep saliva in mouth): Down’s, cerebral palsy, Parkinson’s, prior Sx
-­‐ Idiopathic paroxysmal sialorrhea: short episodes 2-­‐5min each, prodrome of nausea and epigastric pain

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10
Q

Xerostomia

A

-­‐ Complaint of 25% of the adult population
-­‐ Causes: developmental (SG aplasia), water/metabolite (reduced intake, vomit, hemorrhage), iatrogenic (drugs, RT, CT), systemic disorders (SS, sarcoidosis, diabetes, HIV, HCV, GVHD) and local (reduced mastication, smoking, mouth breathing)

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11
Q

Benign lymphoepithelial lesion (lymphoepithelial sialadenitis)

A

-­‐ Mikulicz disease = BLEL = Benign lymphoepithelial lesion = symmetric bilateral swelling of the lacrimal and salivary glands
-­‐ Mikulicz syndrome = SG enlargement from other causes (eg sarcoidosis)
-­‐ BLEL: painless enlargement SG (usually unilateral). Pts may or may not have clinical Sjogren
-­‐ Histo: lymphocytic sialadenitis + epimyoepithelial islands (ductal preservation with epith prolif)
-­‐ Malignant LEL (aka lymphoepithelial carcinoma): poorly differentiated salivary ca with prominent lymphoid stroma. EBV-­‐related. Arises de novo or from BLEL
-­‐ Increased risk MALT lymphoma

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12
Q

Sjogrens syndrome

A

– Female predilection, middle-aged
-­‐ 1ary (sicca syndrome=dry eyes+mouth) or 2ary (sicca syndrome+ RA, SLE, billiary cirrhosis)
-­‐ Dry eyes=keratoconjunctivitis sicca
-­‐ HLA-­‐DRw52 (both forms), DR3 and B8 (primary form only)
-­‐ Fruit-­‐laden, branchless tree on sialography
-­‐ Rose Bengal dye: shows defects on ocular surface epithelium
-­‐ Schirmer test: to confirm dry eyes (< 5mm +)
-­‐ Elevated ANAs, anti-­‐SSA (Ro), anti-­‐SSB (La), ESR, IgG, RF
-­‐ Histo: multifocal lymphocytic sialadenitis (T cells) leading to acini destruction.
-­‐ MSG biopsy: 2+ foci 50+ lymphocytes within a 4-­‐mm2 is supportive of SS. Smokers have less foci
-­‐ Increased risk of MALT lymphoma (40x)

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13
Q

HIV-­‐related parotid cysts

A

-­‐ Usually bilateral
-­‐ Histo: cystic spaces lined with squamous epithelium, with abundant reactive lymphoid stroma
-­‐ Epimyoepithelial islands and Warthin-­‐Finkeldey giant cells may be seen

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14
Q

Sialadenosis

A

– noninflammatory disorder
○ Salivary gland enlargement, particularly parotid glands
– Associated with underlying systemic problem - endocrine, nutritional, neurogenic in origin
-­‐ Risk factors: diabetes, malnutrition, alcoholism, and bulimia
-­‐ Aka sialosis: increased accumulation of secretory zymogen granules due to innervation disruption

-­‐ Sialography: leafless tree (compression of finer ducts by hypetrophic acinar cells)

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15
Q

Adenomatoid hyperplasia of the minor salivary glands

A

-­‐ Mostly on palate (“pseudotumor”). Possibly due to chronic trauma.
-­‐ Coalescing lobules of normal appearing mucinous acini in lamina propria and submucosa

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16
Q

Polycystic (dysgenetic) parotid disease

A

-­‐ Developmental abnormality histologically ~ to polycystic disease of the kidney
-­‐ Amost always in parotid, usually bilateral, usually childhood
-­‐ Histo: Parenchyma diffusely replaced by varying degrees of honeycombed cystic change, lined with a thin layer of flattened epithelium.
-­‐ DDx: cystadenoma/carcinoma (but these are localized masses)

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17
Q

Juxtaoral organ of Chievitz

A

-­‐ Vestigial organ of the developing parotid gland
-­‐ Located bilaterally within the ST overlying the angle of the mandible in the buccotemporal space
-­‐ May serve as a mechanosensor in the lateral wall of the oral cavity (avoid removal)
-­‐ 2-­‐10 islands of squamoid cells (but no keratin).
-­‐ Dark stained cells on periphery and clear cells in center. Glandular/organoid pattern
-­‐ DDx: WD SCC, MEC and metastatic tumor

18
Q

Necrotizing sialometaplasia

A

-­‐ Necrotizing sialometaplasia: due to local ischemia and infarction
-­‐ Factors: trauma, sx, infection, dental injection

19
Q

Sclerosing polycystic adenosis

A

-­‐ Pseudoneoplastic reactive inflammatory process ~ sclerosing adenosis of the breast
-­‐ Usually affects parotid
-­‐ Histo: multiple densely sclerotic lobules composed of hyalinized collagen surrounding multiple ducts with prominent cystic change. Apocrine, sebaceous-­‐like and paneth-­‐like cells seen.
-­‐ Snouts: intraluminal rounded projections

20
Q

Salivary gland tumors – general considerations

A

-­‐ 66-­‐75% of all SG tumors in parotid; 66-­‐75% are benign
-­‐ SG tumors occurrence: parotid (66%) > MSG (palate > upper lip > cheek) > SubMD > sublingual
-­‐ Parotid 66% benign, Submand 60%, MSG overall 50%, sublingual 10%
-­‐ MSG: Upper lip 75% benign; palate and buccal mucosa 50% benign; retromolar pad, FOM, tongue, lower lip 10% benign
-­‐ Parotid: most common benign=PA, most common malignat= mucoep
-­‐ Submand: PA and ACC
-­‐ Sublingual: Mucoep
-­‐ MSG overall: PA, PLGA/mucoep/ACC

21
Q

Pleomorphic adenoma

A

-­‐ Most common SG tumor, most common SG tumor in children
– Palate = most common site (MSG)
-­‐ Myoepithelioma: PA composed almost entirely of myoepithelial cells. Calponin, p63+
-­‐ Myxoid PAs have higher recurrence
-­‐ Recurrent PA: “string of pearls” on skin
-­‐ Tyrosine crystals seen in 6%
– PLGA1, HMGA2

22
Q

Oncocytoma

A

-­‐ Oncocytes show swollen granular cytoplasm due to increased mitochondria
-­‐ 90% in parotid. Thought to be derived from striated duct.

-­‐ Granules: PAS+, diastase sensitive (glycogen). PTAH stain can show granules
-­‐ Oncocytosis: aka multinodular oncocytic hyperplasia (not neoplasm). Small multiple nodule without tumor stroma, nested “zellballen-­‐like” oncocytes with vascular spaces and numerous connective tissue septa
-­‐ Diffuse hyperplastic oncocytosis: entire gland replaced (>70y, often incidental)
-­‐ Oncocytic carcinoma: r/o metastatic Hurthel cell adenoca from thyroid

23
Q

Warthin’s tumor

A

-­‐ Tail of parotid. 8x more in smokers. 10:1 M:F. 5-­‐17% bilateral (metachronous)
-­‐ Carcinoma ex-­‐papillary cystadenoma lymphomatosum: malignant Warthin’s

24
Q

Canalicular adenoma

A

-­‐ 75% upper lip, then cheek. Can be multifocal.
-­‐ Histo: Loose, vascular connective tissue stroma; cystic spaces of variable size; parallel strands of columnar cells with occasional “beading”; and occasional presence of multiple tumor foci

25
Q

Basal cell adenoma

A

-­‐ 75% parotid; then upper lip and cheek
-­‐ Histo: solid, trabecular, tubular and membranous. Basosquamous whorls distinctive pattern.
-­‐ Membranous aka dermal anlage tumor. Shows jigsaw puzzle and thick hyaline material.
-­‐ Brooke-­‐Spiegler syndrome: hereditary membranous BCA, dermal cylindromas, tricoepitheliomas
-­‐ Membranous worst prognosis (30% recurrence)

26
Q

Ductal papillomas

A

-­‐ Sialadenoma papilliferum (papilloma-­‐like, palate), intraductal papilloma (submucosal swelling, most MSG) and inverted ductal papilloma (nodule of lower lip and mandibular vestibule)
-­‐ Histo: SP-­‐ similar to papilloma; IP-­‐ unicystic structure with cuboidal/columnar epithelium; IDP-­‐ unicystic with squamous, bulbous epithelium
-­‐ Syringocystadenoma papilliferum: sialadenoma papilliferum of skin

27
Q

Sebaceous adenoma/lymphadenoma

A

-­‐ Sebaceous lymphadenoma more common than adenoma

28
Q

Sialoblastoma (embryoma)

A

-­‐ Congenital basaloid tumor

29
Q

Mucoepidermoid carcinoma

A

Mucoepidermoid carcinoma
-­‐ Most common malignant SG tumor in adults and children (US). Most in parotid, then MSG.
-­‐ Most common SG tumor of lower lip, FOM, tongue and retromolar pad (~ mucocele)
-­‐ Radiation most common etiologic factor
-­‐ MSG mucoeps lower grading and better prognosis; sub-­‐MD worse prognosis that parotid
-­‐ Intra-­‐osseous: from ectopic SG tissue, sinus lining or odontogenic epithelium from cyst lining
– Grading system: Auclair vs Brandweln

30
Q

Acinic cell adenocarcinoma

A

-­‐ 85% parotid. F>M.
-­‐ 2nd most common malignant tumor of childhood, most common to be bilateral
-­‐ PAS+, diastase resistant (so it’s not glycogen)
-­‐ Often cotains lymphocytes (TALP)

-­‐ Solid, cystic, microcystic, papillary-­‐cystic, well differentiated, de-­‐differentiated, follicular.
-­‐ Good prognosis (better if in MSG).

31
Q

Malignant mixed tumors

A

-­‐ CA ex-­‐PA (95%; most parotid, then MSG), carcinosarcoma (parotid, sub-­‐MD and MSG) and metastasizing PA (lungs or bones)
-­‐ Usually poorly dif adenoca or SDC
-­‐ Non-­‐invasive (in situ), minimally invasive (< 1.5 mm) and invasive (> 1.5 mm) (worse prognosis)
-­‐ Carcinosarcoma: adenoca NOS + chondorsarcoma (usually)

32
Q

Basal cell adenocarcinoma

A

-­‐ Arises de novo (most) or from BCA. 90% parotid.
-­‐ Types: solid, membranous, tubular and trabecular
-­‐ Invasive growth and/or perineural or vascular invasion importanto to diff from BCA

33
Q

Adenoid cystic carcinoma

A

-­‐ Most in palate, then parotid and subMD.
-­‐ CD43+ and CD117+
-­‐ Classic (cribiform-­‐ swiss cheese), tubular, solid and de-­‐differentiated
-­‐ Fine needle (Giemsa stained): metachromatic staining myxoid material
-­‐ Best prognosis: tubular, diploid; worse prognosis: solid; MX sinus and subMD gland
-­‐ Most common malignancy of sub-­‐MD

34
Q

Polymorphous low-­‐grade adenocarcinoma

A

-­‐ Almost exclusive MSG (palate).
-­‐ Weak CD43, CD117 (strong in AdCC) and GFAP (strong in PA)

35
Q

Epithelial-­‐myoepithelial carcinoma

A

-­‐ Biphasic: inner layer of epithelial cells, outer layer of myoepithelial (clear) cells
-­‐ 60-­‐80% parotid; remaining in sub-­‐MD, sinonasal tract and MSG
-­‐ Variants: oncocytic, double clear
-­‐ Myoepithelial-­‐predominant EMC and clear cell myoepithelial CA may overlap features
-­‐ Inner cels: AE1/3 and Cam 5.2+; outer: PAS, SMA, calponin and p63+ (latter is best)
-­‐ p63 participates in epidermal-­‐mesenchymal interactions during embryonic develop

36
Q

Hyalanizing clear cell carcinoma

A

-­‐ Low-­‐grade, monomorphic, glycogen-­‐rich (PAS+, diastase sensitive) carcinoma
-­‐ Dense fibrous stroma surrounding chords, nests, sheets and trabeculae of tumor cells
-­‐ Myoepithelial markers negative

37
Q

Salivary duct carcinoma

A

-­‐ Aggressive adenocarcinoma resembling high-­‐grade breast ductal carcinoma
-­‐ Most on parotid; 90% high grade
-­‐ Comedonecrosis within ducts and Roman bridge formation

38
Q

Sebaceous carcinoma/lymphadenocarcinoma

A

-­‐ Biphasic: 30’s and 80’s
-­‐ Lymphadenocarcinoma: malignant counterpart of sebaceous lymphadenoma (rarest – 6 cases)

39
Q

Mammary analogue secretory carcinoma

A

-­‐ Macro and microcystic architecture, secretion in cysts PAS+
-­‐ Prominent hob nailing seen, cells may show granular pink vacuolated cytoplasm
-­‐ DDx: papillary acinic cell ca and low-­‐grade cystadenocarcinoma
-­‐ Vimentin, S100+
-­‐ FISH: ETV6-­‐NTRK3

40
Q

Mucinous adenocarcinoma (colloid carcinoma)

A

-­‐ Tumor composed of epithelial clusters with large pools of extracellular mucin
-­‐ CK20+, CK7 neg, CDX-­‐2 neg, TTF-­‐1 neg
-­‐ Same markers as breast colloid carcinoma? (ER, PR, GCDFP-­‐15)