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67 Cards in this Set

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Skin: SqCCa
Invasion of epi BM by dysplastic Sq epi
anaplasia
severe atypia & abnormal polarity
abnormal polarity
loss of maturational order & normal layering of epi
Epidermidalization
keratinization & formation of skinlike feature by mucosal epi
Skin: Carcinoma in situ
Full thickness dysplasia
Nucleoli atypia
Individual cellular enlargement, pleomorphism, hyperchromasia, abnormal mitoses
retinoblastoma: Histo:
Flexner-Wintersteiner rosettes: cental clear lumen surrounded by parallel fibrils with a ring of nuclei
(also may see Homer Wright rosettes)
retinoblastoma: what indicates a Better prognosis:
presence of gliosis, Histo=presence of fleurettes (clusters of nearly mature photoreceptor elements) (amt of differentiation not important!!!!)
reese-Ellsworth
predicts visual prognosis after tx for retinoblastoma (not life prognosis);
retinoblastoma
Chr 13 (“DOUBLE HIT GENE”) may present as *leukocoria*, uveitis, angle closure, orb cellulitis, strab, hyphema; 2/3=unilateral & 6% have fam hx; all familial, all bilateral & 15% sporadic unilateralcan pass down via AR (but acts as AD w/ 80% penetrance); hereditary form=increased incidence of secondary malignancies later in life (sarcomas); if 1 child has hereditary form, 40% of subsequent children (1/2 of 80%) & 40% risk to their own children;
Met w/u includes, bone scan, bone marrow aspirate, LP
Increased incidence of coinciding primary pineal tumors
Leukemia: if infiltrates AC
spontaneous hyphema & heterochromia;
leukemia orbital infiltration
edema, proptosis, ecchymosis
leukemia cataract/glaucoma
glaucoma secondary to hyphema, tumor cells clogging TM, papillary block w/synechiae
cataract secondary to inflammation or radiation
SqCCa of limbus
gelatinous, leukoplakic or papilliform; Histo=epidermoid & spindle-shaped cells; Produce white keratin plaque; Tx=MMC, excision w/ local radiotherapy w/ plaque to scleral bed, excision w/ wide margins + Cryo; ***MOST common malignant epi tumor of the conj***
Conjunctival intraepithelial neoplasia
pre-invasive stage of SqCCa; NO invasion of BM; interpalpebral; leukoplakia & thickening of theepi w/ abnormal corkscrew BVs; Tx: excisional bx
Benign reactive lymphoid hyperplasia
prominent vascularity; heterogeneous cell population, numerous follicles
Conjunctival lymphoma
salmon colored lesion on bulbar conj; histo: monoclonal b-lymphocytes is associated w/ malignancy (no atypia in this malignancy); Tx=surgery, local chemo or radiation
Lymphoma
“leopard skin pattern”; >70y/o; vitritis (uni/bilateral)w/u: MRI, CT w/ contrast, LP? CNS lymphoma, PPV for cytology & staging; Tx=intrathecal MTX, IV cytotoxic agent, Whole brain & ocular XRT; poor prognosis (6mo following symptoms)
Mucoepidermoid carcinoma
aggressive form of SqCCa; suspect if recurrence; >60y/o & involves goblet cells (malignant mucus secreting); tends to invade globe
Conjunctival papilloma
DDx=seb K, actinic keratosis, verruca, SqCCa
Conjunctival papilloma;
kids & young adults
may be multiple in palpebral conj; viral; spontaneous resolution is common; histo=hyperkeratosis, parakeratosis, epidermidalization w/ lobular acanthotic epi over fibravascular core; HPV (also can cause SqCCa) pedunculated=HPV 6&8, sessile=HPV 16 & 18
Conjunctival papilloma;
Adults
single lesions, non-infectious, often at limbus; histo=atypical spindle cells w/ dark nuclei
Conjunctival papilloma
Neoplastic
older adults; unilateral; isolated; bulbar conj; will recur singley if not completely excised; fulminant conjunctivitis; rare spontaneous improvement
Conjunctival papilloma
Viral
kids/teens; occ bilateral; occ multiple; palpebral conj; incomplete excision=multiple recurrences; rarely assoc w/ conjunctivitis; may spontaneously resolve
Basal Cell Ca
typically lower lid; nuclei @ periphery of tumor cell nests retain polarity w/ palisading; fibrosing=worst prognosis; causes morbidity & mortality via direct extension into skull/CNS
SqCCa lid
typically upper lid;
SqCCa Cornea:
limited by Bowmans
Sebaceous Cell Ca
older people; needs prompt resection; typically misdiagnosed as chalazia & drained frequently before sample sent to path; “skip” lesions—therefore, Moh’s is not good choice; need map biopsies w/ frozen sections to preserve fat for Oil red O stain; features: epithelial xanthomatization, intraepithelial tumor & inflammatory cells, multiple tumor nests; possible origins: zeiss, caruncle glands, meibomian glands (NOT Moll)
Pilomatrixoma
“calcifying epithelioma of Malherbe”--benign; more common in kids; assoc w/ myotonic dystrophy
Syringoma
tumor derived from sweat gland; associated with Down syndrome, Marfan syndrome, and Ehlers-Danlos syndrome
Kaposi’s sarcoma
malignant counterpart in abnormal immune system (AIDS) of pyogenic granuloma; w/ normal immune system, affects lower extremities of older men; Tx=radiation
Multiple discrete eyelid nodules DDx=
JXG, Hand-Schuller Christian disease, lipoid proteinosis, syringoma
Pyogenic granuloma
small caliber vascular channels in a collagenase stroma
Epithelial inclusion cysts
lined nonkeratinizing stratified Sq Epi
PAM
unilateral; middle aged white people; multiple, superficial flat patches; 20-30% result in melanoma; if nodular, BX
Racial / ocular melanosis
perilimbal dusting; no malignant potential
Ocular
(“blue nevus”)
more common in whites; deep & flat; present @ birth
Cellular blue nevus
blue nevus as above w/ many dendritic nevus cells
Oculo-cutaneous (“nevus of Ota”)
more common in blacks/Asians but only malignant potential in whites; diffuse cong deep dermal nevus in periocular skin
Junctional nevus
nests of nevi cells in basal epi layer
Spindle cell nevus
compound nevus of childhood w/ bizarre cellular components but no malignant potential
Melanocytoma
elevated, deeply pigmented lesion near ON
Keratoacanthoma
form of pseudoepitheliomatous hyperplasia; rapidly growing (1-2 Mo) circular lesion w/ central ulceration filled w/ keratin, may have lash loss & appear malignant; left untreated, may resolve spontaneously; excisional bx is recommended to r/o malignancy; bx=inflammatory tissue (pseudoepitheliomatous hyperplasia), acantosis, hyperkerasosis, dyskeratosis
Hyperostosis / osteoblastic
(increase in bone) DDx:
prostate mets, sphenoid meningeoma, fibrous dysplasia (typically younger pts)
Lacrimal sac tumors
most primary malignant tumors are of the papillary carcinomas (SqCCa, Transitional cell, mixed cell); palpable subQ mass above medial canthal tendon Tx=local excision; good prognosis; next most common=lymphomas; Tx=radiation & systemic w/u
lacrimal gland tumor: Pleomorphic adenoma (benign mixed cell neoplasm):
most common lacrimal tumor; epithelial; typically painless, but may occasionally have bony erosion & expansion; >9 Mo duration of lacrimal enlargement ; spindle cells w/ ductal epi & mixed stromal pattern; pseudoencapsulated; Tx=excisional bx to confirm dx
lacrimal gland tumor: Primary lymphoma
epithelial; “molding/putty” to globe; acute or chronic; B & T cells w/ predominance of B cells
lacrimal gland tumor: Adenoid cystic carcinoma
most common malignant lacrimal tumor; epithelial; painful w/ bony destruction & rapid progression; histo=swiss cheese pattern; often w/ perineural invasion; TX=exenteration; +/-chemo & radiation
lacrimal gland tumor: Malignant Pleomorphic adenoma
lymphoproliferative; painful w/ bony destruction; ductal epi in tubular formation w/ malignant degeneration
Lymphoplasmacytoid proliferation w/ Dutcher bodies DDx:
Waldenstrom macroglobulinemia or systemic lymphoma w/ immunoglobulins
dutcher bodies
(intranuclear PAS + inclusions of immunoglobulins)
Orbital lymphangioma
kids; appears as bluish lesion; acute proptosis if crying, after URI, or hemorrhages (often after trauma)(endothelial lined thin wall interstitial capillary channels that are easily brokenecchymosis); MRI=layered hemorrhage in ocular & cystic mass “chocolate cysts” behind the eye; benign; observe for growth/compression then debulk/CT guided drainage & ?IV steroids if needed; typically starts superonasal
Rhabdomyosarcoma
most common primary malignancy in kids; rapid evolution of unilateral proptosis& reddish discoloration of eyelids mimicking orbital cellulitis; biopsy, radiation, chemo; DDx: myocele, mucopyocele, encephalocele, neurofiboroma
embryonal=most common
alveolar=worst prognosis
differentieated=least common; best prognosis
Neuroblastoma
most common metastatic tumor in kids (1/3 present as mets); abrupt eccymotic proptosis that may be bilateral (50%); in kids, mets to orbit (rapid proptosis, spontaneous ecchymosis, cellulitis like); in adults, mets to uveal tract from adrenal, mediastinum, neck; 20% have orbital involvement; mean age of presentation=2 y/o; poor prognosis if <1 y/o or bone mets; spontaneous regression rarely seen
metastatic==associated w/ opsoclonus (dancing eyes)
Neuroblastoma histo
Histo: Mets: sheets of round cells w/ scant cytoplasm; copious mitotic figures, areas of tumor necrosis, bony invasion
Primary tumor: Homer Wright rosettes: ring of nuclei w/ no central lumen surrounded by haphazardly arranged cell processes (see pic).
**pseudorosettes (which have a BV in the center)
Capillary hemangioma
high flow; spontaneous resolution; 70% resolve by age 7; most common benign tumor in kids (mostly girls); blanch with pressure; may also occur in adults; slow growth w/ progressive proptosis; Tx=corticosteroids or interferon; complications: Kassabach-Merritt syndrome= thrombocytopenia due to platelet sequestration;
--in retina: 20% risk for cerebellar hemangioblastomas
pathology: appears same as port wine stain
Cavernous hemangioma
low flow (phlebolith common)—does not cause proptosis with valsalva; slow growing lesions; does not acutely leak/bleed but in retinal cavernous hemangioma, VH is most common complication (most likely due to traction); most common, well=circumscribed orbital lesion in adults; increases in pregnancy; assoc findings=retinal striae, hyperopia, ON compression, increased IOP, strab
Orbital varix
irregular mass w/ phlebolith potential; increases w/ valsalva; Tx=conservative
Enophthalmos malignant differential
DDx=breast, GI, lung, prostate
Meningiomas histo
psammoma bodies
Schwannomas
(“neurilemmoma”) Present in 1% of NF pts; usually benign; perineural spread + compression=pain;
Antoni A: regularly arranged eosinophilic spindles w/ palisading nuclei
Antoni B: haphazardly arranged stellate cells in myxomatous matrix
Path=Schwann cells; true capsule present; S-100 +staining
Neurofibroma
Lesion of upper lid=strong correlation to congenital glaucoma
Plexiform=most specific for neurofibromatosis
Grows independent of peripheral nerves=painless; generally osteolytic
Path=axons + perineural cells + Schwann cells; S-100 + staining; no capsule
Optic nerve glioma
present w/ visual loss, strab, ptoptosis, hypothalamic abnormalities; 90% by age 20 (typically 2-6y/o); 15-60% have NF1; in adult, malignant gliomasurvival is 6-12 months; CT= “kinking” + fusiform enlargement of nerve; cannot remove w/out removing optic nerve as tumor is not encapsulated & involves nerve
if involves chiasm or hypothalamus—may appear as spasmus nutans
Optic nerve meningioma
occur primary in women; NF increases risk, but still only a few pts w/ tumor have NF; CT w/ contrast=”tram-track”, may show calcification (“psammoma bodies”); tumor=benign prolif of whorls of meningotheial cellsdamage via compression
Disc collaterals
(optociliary shunt vessels): DDx=CRVO, spheneoid wing meningioma, long standing POAG, optic gliomas, chronic papilledema, ON meningioma
Suprasellar craniopharyngiomas
remnants of Rathke’s pouch; near pituitary & chiasmgrowth & pituitary like compression on nerves; often calcified;
Parinaud’s dorsal midbrain syndrome
(ex. Pinealoma) skew deviation, lid retraction (Collier’s sign), papillary light-near dissociation, contraction-retraction nystagmus, upgaze paralysis