how are cleft lip/ palate formed
failure of the maxillary and nasal/palatal bones to fuse
when does the cleft lip form
weeks 4-6 in utero
when does cleft palate form
weeks 6-12 in utero
associations of cleft lip/ palate
Trisomy 13
Trisomy 18
smoking
EtOH
Phenytoin
Folate antagonists
hypervitaminosis A
distinguish oral leukoplakia vs erythroplakia
which is more likely to become malignant transformation:
which cancer
leukoplakia: white plaque that cant be scraped off
erythroplakia: red velvety plaque (may be leveled or slightly depressed in the oral cavity that bleeds easily on contact
Erythroplakia is much more likely to become squamous cell carcinoma of the oral cavity
intermediate variant of leukoplakia and erythroplakia
speckled leukoerythroplakia
clinical and histological appearance of leukoplakia
clinical: relatively thin and smooth with well-demarcated borders
histology: severe dysplasia characterized by nuclear and cellular pleomorphism, numerous mitotic figures and loss of normal maturation
clinical and histological appearance of erythroplakia
clinical: red velvety plaque
histology: severe dysplasia, carcinoma in situ, minimal invasion
Name the molecular pathogenesis associated with squamous cell carcinoma of the oral cavity
inactivation/ mutation of p53 tumor suppressor genes
p63 and NOTCH1 are also associated
associations of oral cavity squamous cell carcinoma
HPV-16
Tobacco
Alcohol
Actinic sunlight
common sites of oral cavity squamous cell carcinoma
tonsils
base of tongue
pharynx
microscopy of oral cavity squamous cell carcinoma
keratin pearls
numerous sets of islands of malignant keratinocytes invading the underlying connective tissue stoma and skeletal muscle
how does squamous cell carcinoma of the oral cavity usually spread and where does it tend to metastasize
via lymphatics to the regional lymph node
usually spreads to lungs, liver and bones
prognosis of oral squamous cell carcinoma
5 year survival rate for early stage ~80%.
late stage ~20 %
another name for canker sore
aphthous ulcer
what ar canker sores
common, recurrent painful, superficial mucosal ulcer
associations of aphthous ulcers
stress
trauma
immunologic disorders
immune suppression
What is a pleomorphic adenoma and where are they most commonly found
benign tumors of mixed ductal origin and myoepithelial cells
parotid>submandibular>minor salivary glands
Pathogenesis of Pleomorphic adenoma
chromosomal rearrangements involving PLAG1 gene causes upregulated expression of genes that increase growth
Most common benign salivary gland tumor
pleomorphic adenoma
how do pleomorphic adenomas present
painless
slow-growing
mobile mass
parotid or submandibular
Macro-morphology of pleomorphic adenoma
well rounded, well-demarcated mass with partially developed capsule
grey-white myxoid stroma with blue translucent areas of chondroid
Micromorphology of pleomorphic adenoma
mixed cellular constitution with myoepithelial cells and chondroid tissue; typically dispersed within a mesenchyme-like background of loose myxoid tissue containing islands of cartilage and rarely foci of bone
cytokeratin
another name for a Warthin tumor
papillary cystadenoma lymphomatosum
where are Warthin tumors found
Parotid Gland only
Describe a Warthin tumor
benign cystic tumor comprised of admixed epithelial and lymphoid tissue
fluid of cyst resembles motor oil
clinical presentation of a warthin tumor
gradual, painless unilateral swelling of the parotid
morphology of Warthin Tumor
Macro: pale grey surface punctuated by narrow cystic or cleft like spaces filled with mucinous/ serous secretions
Micro: double layer of neoplastic epithelial cells resting in a dense lymphoid stroma, sometimes bearing germinal centers
- upper layer: palisading columnar cells with abundant finely granular eosinophilic cytoplasm*
- lower layer: cuboidal and polygonal cells*
what is a mucoepidermoid carcinoma
malignant tumor consisting of mucosal cells and squamous epithelium
Most common malignant salivary gland in children
where are mucoepidermoid carcinomas usually found
minor salivary glands
Morphology of mucoepidermoid carcinomas
capsule not really well defined
often infiltrative at the margins
pale grey-white transections containing mucin filled cysts
cords, sheets, or cystic configurations of squamous, mucous or intermediate cells
Associated pathogenesis of mucoepidermoid carcinoma
t( 11q21 ; 19p13 )
creates a fusion gene of MECT1 and MAML2
what is an adenoid cystic carcinoma
slow but infiltrative growth along nerve sheaths, with a cribriform tubular growth pattern
where do most adenoid cystic carcinomas occur
and where do the metastasize
minor salivary glands (palatine)
invade perineural spaces
mets to: bone, brain, liver
Morphology of adenoid cystic carcinoma
generally small, poorly encapsulated infiltrative, grey-pink lesions
small cells have dark compact nuclei and scant cytoplasm
spaces between tumor cells are often filled with hyaline material, thought to represent excess basement membrane
what causes Sjogren Syndrome
autoinflammation of lacrimal and salivary glands
Presents as: xerostomia and xerophthalmia, occasionally raynaud phenomenon