11: Salivary pathology Flashcards
von ebner salivary glands – where, what secretions
tongue beneath circumvallate and foliate papillae; primarily serous
salivary glands in tongue beneath circumvalate and foliate papillae and their secretions
von ebner, primarily serous
ventral tongue salivary glands and secretions
blandin-nuhn, serous
blandin-nuhn salivary glands – where, what secretions
ventral tongue, serous
salivary gland aplasia syndromes
treacher collins, hemifacial microsomia, lacrimo-auriculo-dento-digital syndrome (LADD)
can also be isolated
LADD genetics and clinical
FGF10 mutation
salivary and lacrimal gland aplasia, cup shaped ears, dental/digital anomalies
salivary and lacrimal gland aplasia, cup shaped ears, dental/digital anomalies
what is and genetics
LADD
FGF10 mutation
superficial mucoceles location
soft palate and retromolar pad
superficial mucoceles which conditions
lichen planus, lichenoid drug reactions, GVHD, tartar control toothpaste
connective tissue reaction to extravasated mucus
granulation
origin of ranula
sublingual gland (body or ducts of Rivini)
CT/MRI sign of submandibular ranula
tail sign
plunging ranula dissects though
mylohyoid muscle
salivary duct cyst location
upper lip
systemic treatment for multifocal ductal ectasia
erythromycin and chlorhexidine
most common sialolithiasis and histo look
submandibular
tortuous duct and mucoid secretion
types of sialadenitis and causes
infections - viral-mumpsm bacterial - S aureus
non-infectious – Sjogrens, RT, sarcoid
chronic sclerosing sialadenitis in submandibular leading to gland enlargement – evaluate for what
Kuttner tumor (IgG4) – eval for sclerosing pancreatitis
how do bacteria cause sialadenitis
retrograde spread from reduced flow (dehydration, medication) or block (sialolith, tumor)
sialadenitis most commonly where and presentation
fever and pus, usually parotid
surgical mumps
after abdominal surgery, when patient is kept without food/fluids and receives atropine
after abdominal surgery, when patient is kept without food/fluids and receives atropine
surgical mumps
sausaging of stensen’s duct – what is, what modality, what diagnosis
dilation and strictures from scar tissue in sialadenitis on sialography
subacute necrotizing sialadenitis histo, demographic, ulceration
no squamous metaplasia, mixed acute and chronic inflammation, younger patients, does not ulcerate
cheilitis glandularis what is
swelling and eversion of lower lip due to hypertrophy and inflammation of glands
swelling and eversion of lower lip due to hypertrophy and inflammation of glands
cheilitis glandularis
cheilitis glandularis etiologues
UV damage, tobacco, syphilis, poor hygiene, hereditary
forms of cheilitis glandularis
simple, superficial suppurative (Baelz’s disease)m deep suppurative (cheilitis glandularis apostematosa)
Baelz’s disease
superficial suppurative cheilitis glandularis
cheilitis glandularis apostematosa aka
deep suppurative
true sialorrhea causes
aphthae, new dentures, GERD (water brash), rabies, heavy metal poisoning, medications for alzheimers
relative sialorrhea what is and causes
can’t keep saliva in mouth: Down’s, cerebral palsy, Parkinson’s, prior surgery
idiopathic paroxysmal sialorrhea presentation
short episodes 2-5min, prodrome of nausea and epigastric pain
xerostomia how ofetn
25% of adult population
xerostomia 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)
benign lymphoepithelial lesion aka
lymphoepithelial sialadenitis, Mikulicz disease
painless enlargement of salivary glands, usually unilateral
pts may or may not have clinical sjogren
mikulicz syndrome what is
salivary gland enlargement from other causes (eg sarcoidosis)
benign lymphoepithelial lesion histo
lymphocytic sialadenitis + epimyoepithelial islands (ductal preservation with epithelial proliferation)
malignant lymphoepithelial lesion aka, histo, cause
aka lymphoepithelial carcinoma
poorly differentiated salivary ca with prominent lymphoid stroma, EBV, de novo or from BLEL
primary sjogrens =
sicca syndrome = dry eyes + mouth
secondary sjogrens
sicca syndrome + RA, SLE, biliary cirrhosis
dry eyes in sjogrens aka
keratoconjunctivitis sicca
HLA in sjogrens (primary vs secondary)
HLA-DRw52 both
HLA-DR3 and B8 primary only
fruit laden branchless tree
sialography for sjogrens
rose bengal dye
shows defects on ocular surface epithelium in sjogrens
dye to show ocular surface epithelium defects in sjogrens
rose bengal
test to confirm dry eyes and what’s the metric in sjogrens
schirmer test - <5mm
labs in sjogrens
ANAs, anti-SSA (Ro), anti-SSB (La0, ESR, OgG, RF
sjogrens histo
multifocal lymphocytic sialadenitis (T cells)–> acini destruction
sjogrens biopsy criteria
2+ foci of 50+ lymphocytes within 4mm squared; smokers have fewer foci
risk with sjogrens
40x MALT lymphoma
HIV related parotid cysts presentation
usually bilateral
bilateral parotid cysts consider
HIV related parotid cysts
HIV related parotid cysts histo
cystic spaces lined with squamous epithelium; abundant reactive lymphoid stroma
epimyoepithelial islands and Warthin-Finkeldey giant cells
Warthin-Finkeldey cells
giant cells in HIV related parotid cysts
giant cells in HIV related parotid cysts name
Warthin Finkeldey
Sialadenosis aka, what is and why
sialosis
accumulation of secretory zymogen granules due to innervation disruption
accumulation of secretory zymogen granules due to innervation disruption
Sialadenosis
sialadenosis risk factors
diabetes, malnutrition, alcoholism, bulimia
sialadenosis sialgraphy
leafless tree (compression of finer ducts by hypertrophic acinar cells)
leafless tree sialography why and what
sialadenosis (compression of finer ducts by hypertrophic acinar cells)
adenomatoid hyperplasia of minor salivary glands where and why
mostly on palate (pseudotumor); poss 2/2 chronic trauma
adenomatoid hyperplasia of minor salivary glands histo
coalescing lobules of normal mucinous acini in lamina propria and submucosa
coalescing lobules of normal mucinous acini in lamina propria and submucosa; say, palate
adenomatoid hyperplasia of minor salivary glands
polycystic (dysgenetic) parotid disease where and who
almost always in parotid, bilateral, usually childhood
polycystic (dysgenetic) parotid disease histo
parenchyma diffusely replaced by varying degrees of honeycombed cystic change, lined with a thin layer of flattened epithelium
polycystic (dysgenetic) parotid disease ddx
cystadenoma/carcinoma but they are localized masses
juxtaoral organ of chievitz what, where, function
vestigial organ of the developing parotid gland
bilateral in soft tissue overlying angle of mandible in buccotemporal space
poss mechanosensor in lateral wall of oral cavity – avoid removal
juxtaoral organ of chievitz histo and ddx
2-10 islands of squamoid cells but no keratin
dark stained cells on periphery and clear cells in center
glandular/organoid pattern
ddx: well-diff SCC, MEC, met
necrotizing sialometaplasia causes
local ischemia and infarction
factors: trauma, surgery, infection, dental injection
sclerosing polycystic adenosis where and why
pseudoneoplastic reactive inflammatory process, usually parotid
sclerosing polycystic adenosis histo
multiple densely sclerotic lobules composed of hyalinized collagen around multiple ducts with prominent cystic change
cells: apocrine, sebaceous-like, and paneth-like
has intraluminal rounded projections
salivary gland tumors = where most common, what’s most common
66-75% of all tumors in parotid
66-75% are benign
salivary gland tumor occurrence by location
parotid (66) > minor (palate>upper lip> cheek) > submandibular > sublingual
what percentage of tumors are benign in parotid submandibular minor salivary (overall) sublingual
parotid 66 benign
submandibular 60 benign
minor salivary 50 benign
sublingual 10 benign
minor salivary glands, what percentage of tumors are benign:
upper lip
palate and buccal mucosa
retromolar pad, FOM, tongue, lower lip
upper lip 75 benign
palate and buccal mucosa 50 benign
retromolar pad, FOM, tongue, lower lip 10 benign
most common benign tumor in parotid
pleomorphic adenoma
most common malignant tumor in parotid
mucoep
most common benign tumor in submandibular
pleomorphic adenoma
most common malignant tumor in submandibular
adenoid cystic carcinoma
most common tumor of sublingual
mucoep
most common benign tumor of minor salivary glands overall
pleomorphic adenoma
most common malignant tumorS (list multiple) of minor salivary glands overall
PLGA/mucoep/ACC
most common salivary gland tumor
pleeomorphic adenoma
most common salivary gland tumor in children
pleomorphic adnoma
pleomorphic adenoma old aka and why; IHC?
myoepithelioma
composed of myoepithelial cells
calponin, p63+
which pleomorphic adenomas higher recurrence
myxoid
string of pearls on skin
recurrent pleomorphic adenoma
special histo finding in a small subset of pleomorphic adenomas and how common
tyrosinde crystals in 6%
tyrosine crystals which salivary gland tumor
pleomorphic adenoma, 6%
oncocytoma look of cells
oncocytes: swollen granular cytoplasm 2/2 increased mitochondria
oncocytoma location and origin
90% in parotid
poss from striated duct
oncocytoma special stains
PAS+ diastase sensitive (glycogen)
PTAH can show granules
oncocytosis aka (what kind of lesion?) histo
multinodular oncocytic hyperplasia (not neoplastic)
small multiple nodules without tumor stroma
nested zellballen-like oncocytes with vascular spaces and numerous connective tissue septa
small multiple nodules without tumor stroma
nested zellballen-like oncocytes with vascular spaces and numerous connective tissue septa
oncocytosis
aka multinodular oncocytic hyperplasia
warthin tumor location, demographic, other name
tail of parotid 8x more common in smokers 10:1 M:F 5-17% bilateral (metachronous) aka papillary cystadenoma lymphomatosum
papillary cystadenoma lymphomatosum what is?
warthin tumor
malignant warthin
carcinoma ex-papillary cystadenoma lymphomatosum
canalicular adenoma location
75% upper lip, then cheek
can be multifocal
basal cell adenoma location
75% parotid
then upper lip and cheek
basal cell adenoma histo types
solid, trabecular, tubular, membranous
membranous basal cell adenoma aka and histo findings
aka dermal anlage tumor
jigsaw puzzle and thick hyaline material
distinctive histo pattern of basal cell adenomas
basosquamous whorls
brooke-spiegler syndrome components
hereditary membranous basal cell adenoma, dermal cylindromas, trichoepitheliomas
hereditary membranous basal cell adenoma, dermal cylindromas, trichoepitheliomas
brooke-spiegler syndrome
basal cell adenoma with worst prognosis
membranous basal cell adenoma
30% recurrence
sialadenoma papilliferum look, location, histo
ductal papilloma on palate, histo like papilloma
intraductal papilloma look, location, histo
submucosal swelling, mostly minor salivary glands
unicystic structure with cuboidal/columnar epithelium
inverted ductal papilloma look, location, histo
nodule of lower lip and mandibular vestibule
unicystic with squamous, bulbous epithelium
sialadenoma papillefreum of skin
syringocystadenoma papilliferum
sialoblastoma aka and what is
lymphadenoma
congenital basaloid tumor
most common malignant ssalivary gland tumor in adults and children of the uS
mucoep
mucoep locations
parotid, then minor salivary glans
most common salivary gland tumor of lower lip, FOM, tongue, retromolar pad
mucoep
most common etiologic factor for mucoep
radiation
compare grade and prognosis for mucoeps in minor, submandibular, patorid
minor – lower grade, better prognosis
sub-MD worse prognosis than parotid
intraosseous mucoeps how?
ectopic salivary tissue, sinus lining, or odontogenic epithelium from cyst lining
acininc cell adenocarcinoma location, gender, prognosis
85% parotid
F>M
childhood, most commonly bilateral
prognosis good, better in minor glands
second most common malignant salivary tumor of childhood (what’s first)
2 - acinic cell adenocarcinoma
1 - mucoep
acinic cell adenocarcinoma special stain and histo
PAS, diastase resistant (not glycogen)
can be solid, cystic, microcystic, papillary-cystic
well-diff, dediff, follicular
has tumor associated lymphocytes
types of malignant mixed tumors of salivary glands and locations. which most common?
carcinoma ex-PA (95%; most parotid, then minor)
carcinosarcoma (parotid, sub-MD, minor)
metastasizing PA (lungs or bones)
malignant mixed tumors of salivary glands what’s the histo
usually poorly diff adenoca or salivary duct ca
grades of invasion and worse prognosis for malignant mixed tumors of salivary glands
non-invasive (in situ), minimally invasive (<1.5mm), invasive (>1.5mm – worse prognosis obvi)
salivary carcinosarcoma components usually
adenocarcinoma + chondrosarcoma
basal cell adenocarcinoma arises from; location
de novo (most) or basal cell adenoma – 90% parotid
types of basal cell adenocarcinoma
solid, membranous, tubular, trabecular
ddx basal cell adenoma from basal cell adenocarcinoma
invasive growth and/or perineural or vascular invasion
adenoid cystic carcinoma location
most in palate; then parotid in sub-MD
adenoid cystic carcinoma histo patterns, IHC, special stain
classic (cribiform swiss cheese), tubular, solid, and de-differentiated
IHC CD43+ and CD117+
Giemsa – metachromatic staining myxoid material
adenoid cystic pattern with best prognosis
tubular
adenoid cystic pattern and location with worst prognosis
solid
mx sinus and subMD gland
most common malignancy of subMD salivary gland
adenoid cystic carcinoma
polymorphous low grade adenocarcinoma location
almost exclusively minor salivary glands of palate
polymoprhous low grade adenocarcinoma IHC (helps to distinguish from?)
weak CD43, CD117 (strong in adenoid cystic) and GFAP (strong in PA)
epithelial-myoepithelial carcinoma histo, variants
biphasic:
inner layer epithelial cells; outer layer myoepithelial (clear cells)
variants: oncocytic, double clear
epithelial-myoepithelial carcinoma locations
60-80% parotid
remaining in sub-MD, sinonasal tract, minor
epithelial-myoepithelial carcinoma IHC
inner cells: AE1/3 and CAM5.2
outer: PAS, SMA, calponin, p63+ (p63 is best – participates in epidermal-mesenchymal interactions during embryonic development)
hyalinizing clear cell carcinoma histo and IHC
low-grade, monomorphic, glycogen rich (PAS+, diastase sensitive) carcinoma
dense fibrous stroma around chords/nests/sheets/trabeculae of tumor cells
myoepithelial markers negative
low-grade, monomorphic, glycogen rich (PAS+, diastase sensitive) carcinoma; negative myoepithelial markers
hyalinizing clear cell carcinoma
salivary duct carcinoma histo, location
aggressive adenocarcinoma ( similar to high grade breast ductal carcinoma) comedonecrosis in ducts; Roman bridge formation
most in parotid
90% are high grade
comedonecrosis in ducts; Roman bridge formation
salivary duct carcinoma
sebaceous carcinoma aka; age
lymphadenocarcinoma (malignant of sebaceous lymphadenoma – RAREST (6 cases))
30s and 80s
lymphadenocarcinoma aka; age
sebaceous carcinoma (malignant of sebaceous lymphadenoma – RAREST (6 cases))
30s and 80s
mammary analogue secretory carcinoma histo
macro and microcysts; secretion in cysts PAS+
prominent hobnailing
cells with granular pink vacuolated cytoplasm
macro and microcysts; secretion in cysts PAS+
prominent hobnailing
cells with granular pink vacuolated cytoplasm
in salivary
mammary analogue secretory carcinoma
mammary analogue secretory carcinoma IHC and genetics
IHC: vimentin, S100+
FISH: ETV6-NTRK3
ETV6-NTRK3
mammary analogue secretory carcinoma
mucinous adenocarcinoma aka and histo
colloid carcinoma
epithelial clusters with large pools of extracellular mucin
epithelial clusters with large pools of extracellular mucin in salivary
mucinous adenocarcinoma
mucinous adenocarcinoma IHC
CD20+
breast colloid carcinoma markers?
CK7 neg, CDX2 neg, TTF1 neg
salivary gland aplasia syndromes
mandibulofacial dysostosis (treacher collins); hemifacial microsomia; lacrimo-auriculo-dento-digital syndrome (LADD)
LADD syndrome genetics and clinical
Lacrimo-auriculo-dento-digital (LADD) syndrome
AD / FGF10 Aplasia or hypoplasia of SG, LG Cup shaped ears Hearing loss Dental and digital anomalies
plunging (cervical) ranula what happens, where
sublingual looks like?
mucin dissects through mylohyoid muscle
Sublingual space known as “tail sign”
Lateral to midline (vs dermoid cyst )
tail sign in ranulas
plunging ranula
Sublingual space known as “tail sign”
Most common inflammatory salivary gland disorder in children in the states; what happens to it
Juvenile recurrent parotitis; regresses at puberty
salivary gland inflammation in teenagers /young adults
where and what happens to it
Subacute necrotizing sialadenitis:
Resolves in 2 weeks
Hard and soft palate
inflammation of minor salivary glands of lower lip
cheilitis glandularis
types of cheilitis galndularis
- Simple
- Superficial suppurative (Baelz disease)
- Deep suppurative (Cheilitis glandularis apostematosa)
treatment of sialorrhea
Anti-emetic
Anti-cholinergic
sialorrhea underlying conditions
Rabies , metal poisoning, meds (cholinergic agonist / clozapine), acid from GERD, dentures
super salivation of unknown cause (2-5 min /short episodes)
Idiopathic paroxysmal sialorrhea:
Idiopathic paroxysmal sialorrhea:
super salivation of unknown cause (2-5 min /short episodes)
IgG4 disease IgG4 levels
Normal IgG4 levels = 8-40 mg/l / IgG4 disease 25 x > than normal levels of IgG
mikulicz aphthae
minor aphthous ulcers
mikulicz disease vs mikulicz syndrome
disease = SG + LG swelling syndrome = that but due to TB, sarcoid, lymphoma
locations for IgG4 disease
Pancreas > H&N ( submandibular)
Location for IgG-4 sialadenitis and special name
submandibular glands - Kuttner tumor
complications of IgG4 disease
Abdominal aortitis
Inflammatory pseudo tumors of the kidney
Thyroid inflammation ( Riedel thyroiditis )
lymphadenopathy
Kuttner tumor what is and histo
IgG4 sclerosing sialadenitis of submandibular gland
hyperplastic lymphoid follicle
acinar atrophy
clerosing sialadenitis of submandibular gland
hyperplastic lymphoid follicle
acinar atrophy
eval for what
Kuttner tumor, eval for IgG4 disease
malignancy in Sjogrens
Higher risk for MALT; Lymphoma extra nodal marginal zone B-cell lymphoma (20 X)
dry eyes in sjogrens special name
keratoconjunctivitis sicca
fruit laden branchless tree
Sjogren punctate sialectasia
what kind of dz is sjogren
autoimmune
special autoantibodies in Sjogrens and how often
SS-A (50-70%) SS-B ( 30-60%)
Sjogrens with other autoimmune, how often?
15% RA
30 % SLE
Sjogrens criteria
Patient must have at last 2 of the 3
1) + Anti-Ro ( SS-A) and /or Anti-La (SS-B) antigen or + RF and ANA titer ≥ 1:320
2) ≥ 1 focus / 4mm3
3) Keratoconjuctiva sicca with ocular score ≥ 3
pleomorphic adenoma genetics
PLGA1 gene
PLGA1 gene
pleomorphic adenoma
Warthin tumor aka
Papillary cystadenoma lymphomatosum
Papillary cystadenoma lymphomatosum aka
Warthin tumor
Niesse-Nicholson rests:
SG tissue trapped within LN seen in oncocytic change of Warthin tumor
SG tissue trapped within LN seen in oncocytic change of Warthin tumor
Niesse-Nicholson rests:
which basal cell adenoma is associated with hereditary disease
membranous basal cell adenoma:
eg Brooke Spiegler syndrome
Brooke Spiegler syndrome
Membranous BCAC
Dermal Cylindroma
Spiroadenoma
Trichoepithelioma
oncocytoma staining and what exactly
m/ch; PTAH
mucoepidermoid carcinoma genetics
t(11:19) CRTC1-MAML2
t(11:19)
CRTC1-MAML2 mucoepidermoid
CRTC1-MAML2
t(11:19) mucoepidermoid
special cells in acinic cell carcinoma
zymogen cells
zymogen cells which salivary tumor
acinic cell carcinoma
which mucoeps have better prognosis
with genetic mutation
MASC translocation
t(12:15) ETV6-NTRK3
t(12:15)
MASC ETV6-NTRK3
ETV6-NTRK3
t(12:15) MASC
causes of sialosis
non-inflammatory
1) Endocrine : DI, DM, pregnancy, acromegaly, hypothyroidism
2) Nutritional: Bulimia, anorexia, cirrhosis
3) Neurogenic : medication
sialosis medical treatment
pilocarpine
pleomorphic adenoma IHC
+ GFAP
adenoid cystic IHC
+ CD117, CD43
polymorphous low grade adenocarinoma IHC – ddx from what?
PLGA = - GFAP (vs PA), weak + CD117, CD43 (vs ACC)
von ebner glands where
in tongue beneath circumvalate and foliate papillae
minor glands in tongue beneath circumvalate and foliate papillae
von ebner
blandin-nuhn glands
ventral tongue
ventral tongue salivary glands
blandin-nuhn
superficial mucoceles seen in what
lichen planus, lichenoid drig reactions, GVHD
causes of sialadenitits
infections (viral-mumps, bacterial-staph aureus) and non-infections (Sjogrens, RT, sarcoid)
schirmer’s test to confirm dry eyes measurement
<5mm
test to confirm dry eyes
Schirmer’s, <5mm
dye to show defects on ocular surface epithelium and for what
sjogrens dry eyes, rose bengal
why is c/pl swollen in salivary oncocytoma
increased m/ch
increased m/ch in cells which tumor
oncocytoma
oncocytoma with stains
PAS+, diastase sensisitive glycogen
PTAH for m/ch
oncocytosis aka
neoplastic?
multinodular oncocytic hyperplasia
non-neoplastic
membranous basal cell adenoma, dermal cylindromas, trichoepitheliomas what is
Brooke-Spiegler syndrom
most common subtype of basal cell adenoma
solid
worst prognosis for basal cell adenoma which subtype
membranous, 30% recurrence
types of ductal papillomas
sialadenoma papilliferum (papilloma-like); intraductal papilloma (submucosal swelling); inverted ductal papilloma (nodule)
clinical look of intraductal papilloma
submucosal swelling
clinical look of inverted ductal papilloma
nodule
best prognosis location for mucoep
minor salivary glands
adenoid cystic carcinoma IHC
CD43+ and CD117+
worst histo type prognosis of adenoid cystic
solid
worst location prognosis for adenoid cystic
mx sinus and subMD gland