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Pathology Pt 2
Ch 13 -
Terms in this set (83)
Increased thickness (hyperplasia) of the lower layers of the epidermis
What is acanthosis?
Increased thickness of the stratum corneum (superficial layer) of the epidermis
What is hyperkeratosis?
Loss of cohesion (dissolution of intercellular birdges) btw adjacent epithelial cells
What is acantholysis?
Rare congenital tumor in which lens epithelium is formed within the inferonasal portion of the lower eyelid.
Cells enlarge and become "bladder" cells similar to PSC
What is a Phakomatous Choriostoma?
Verruca Vulgaris (wart):
A) Lesion has papillary growth pattern with fingerlike projections
B) Occasional koliocytes - cells with cytoplasmic clearing (arrow)
Cup-shaped, thickened, epidermis with central crater. Note eosinophillic inclusion bodies (arrows) becoming basophilic as they migrate to the surface
Can also cause a follicular conjunctivitis
- eosinophilic inclusion bodies
Granulomatous inflammation (epithelioid histiocytes and multinucleated giant cells) surrounds clear spaces formerly occupied by lipid (lipogranuloma)
Foam cells (filled with lipid) surrounding a venule (asterisk)
Note waxy elevation and associated purpura which is due to amyloid deposition in vessel walls causing fragility
Epidermal Inclusion Cyst (epidermoid):
Cyst lining resembles epidermis (stratified squamous keratinizing epithelium) and the lumen contains keratin
Apocrine hidrocystoma: Moll's gland cyst
Typically lines with a double layer of cuboidal epithelium
Epithelium is acanthotic with a papillary configuration and keratin-filled cysts (asterisk).
(arrow) within epidermis are seen to represent infoldings of epidermis
Sudden onset of multiple seborrheic keratoses and is associated with malignancy, usually a GI carcinoma
What is the Leser-Trelat Sign?
Visceral carcinoma (usually colon) with keratoacanthoma and sebaceous neoplasms
What is Muir-Torre Syndrome?
Breast carcinoma, thyroid, GI tract, with multiple trichilemmomas
What is Cowden disease?
Medulloblastoma, fibrosarcoma, multiple basal cell carcinomas
What is Basal Cell Nevus Syndrome?
Keratoacanthoma and molluscum
What two lesions present with "cup-like" configuration on histology?
Note cuplike configuration with central crater that was originally filled with keratin.
A) Note epidermal thickening, disorginization within dermis, parakeratosis (asterisk), and inflammtation within the dermis
B) mitotic figures (arrows)
Basal Cell Carcinoma:
Originate from stratum basale or stratum germinativum and the outer root of the hair follicle.
Squamous Cell Carcinoma:
Keratin (asterisk) is produced
Note small capillary sized vessels and proliferation of benign endothelial cells
of the epidermis by individual tumor cells and small clusters of tumor cells (arrows)
Congenital nevus of eyelid may develop in utero prior to separate of the lids
Junctional -> compound -> Intradermal
What are the three stages of a nevus?
Nests of nevus cells are seen at the junction btw epidermis and dermis
Nests of nevus cells are present in the dermis (arrows) as well as the junction btw the epidermis and dermis (arrowheads)
Nests of nevus cells confined to the dermis
Superficial spreading - most common
Nodular - more prominent vertical growth
Acrla-lentiginous - not on eyelids
What are the different types of cutaneous melanoma?
B) Cyst lined by keratinized stratified squamous epithelium
C) The wall of the cyst contains sebaceous glands (arrows) and adnexal structures
Non-specific Orbital Inflammation:
Note the skeletal muscle fibers (arrows) surrounded by a dense infiltrate of chronic inflammatory cells
Thyroid Eye Disease:
B) Fusiform enlargement of EOM sparring the tendons
C) Muscle fibers separated by fluid and accompanies by infiltrate of mononuclear cells
Aspergillus: Silver stain
Note 45 degree angle branching and septae
Most common benign lacrimal tumor
C) Low mag shows well circumscribed tumor
D) Both epithelial (arrows) and mesenchymal (asterisk) elements
E) Well-differentiated glandular structures with lumina (asterisk)
Adenoid Cystic Carcinoma:
Most common malignant tumor of lacrimal gland. Can erode bone and causes significant pain.
Not characteristic cribiform (
) pattern of tumor cells.
pattern indicates worse prognosis
Rx usually requires exteneration + removal of adjacent bone
B-Cell: CD 19, 20
T Cell: CD 3, 4, 8
What CD markers on on B and T cells?
An unencapsulated, diffusely infiltrating tumor that feature lymphatic vascular spaces and lymphoid aggregates in a fibrotic interstitium.
Characterized by fluctuation in proptosis. No capsule.
Note vascular channels and lymphoid follicles (arrow) with fibrotic stroma
Well-circumscribed retrobulbar mass with large spaces of blood separated by thick septa.
Capsulated in adults (capillary hemangioma in children is not encapsulated)
A common mesenchymal tumor, most are benign. Positive for CD45 and CD68, have a mat like or whorly pattern (
Mainly occur in adults.
Staghorn branching of blood vessels
with densely packed spindle-shaped cells.
Most common primary malignant orbital tumor in children. Proptosis often sudden and radily progressive.
C) Cross-striations representing Z-bands (arrow) of actin-myosin complexes within the cytoplasm are seen
D) Poorly cohesive rhabdomyoblasts separated fibrous septa (arrows) into "alveoli"
Note thickened, tortuous nerves (arrows) with proliferation of endoneural fibroblasts and schwann cells
Antoni A Pattern
- spindle cells are arranged in interlacing cords, whorls, or palisades that may form
(asterisk) which are collections of fibrils resembling sensory corpuscles
Antoni B Pattern
- made up of stellate cells with mucoid stroma that represents degeneration within the tumor
Normal Optic Nerve
P - pia
A - arachnoid
D - dura
Arrowheads: lamina cribosa
Optic Nerve Coloboma:
Gliotic, disorganized retina (asterisk) prolapses into the defect which is lined by excavated sclera. Normal retina terminate at the edge of the defect (arrows)
Blue staining area indicates normal myelin.
Note absent myelin (asterisk)
Luxol fast blue stain for myelin
Giant Cell Arteritis:
Vascular lumen is narrowed by concentric intimal hyperplasia (arrow). Prominent transmural inflammatory infiltrate with numerous multinucleated giant cells (arrowheads).
Sarcoidosis of Optic Nerve:
Noncaseating granulomas (arrows) infiltrating optic nerve
Optic disc Edema:
Swollen prelaminar axons demonstrate vacuolar alteration (red arrows) and displace the retina laterally (red arrowhead) from its normal termination just above Bruch membrane (black arrowhead).
Cavernous Optic Atrophy of Schnabel:
A degeneration of the optic nerve by cystic spaces in elderly patients (initially observed in glaucomatous eyes).
Characterized by cystic atrophy posterior to the lamina cribosa (asterisk).
The cystic space becomes filled with alcian-blue staining material
Optic Nerve Drusen:
Appear as discrete basophilic zones of calcification (arrows) just anterior to the lamina cribosa (asterisk)
A benign, deepl pigmented melanocytic tumor situated eccentrically on the optic disc. Slow growth can occur, malignant transformation rare.
Histo shows a magnocellular nevus.
A) Dome-shaped, jet-black mass involving the prelaminar optic nerve
B) Darkly pigmented menalocytes with dense intracytoplasmic pigment obscuring nuclear detail
Optic Nerve Glioma:
Left side of picture shows low grade pilocytic astrocytoma, right side (astrisk) shows normal nerve.
on higher mag which are degenerated eosinophilic filaments (not pathognomonic)
Optic Nerve Meningioma:
Arise from arachnoid sheath.
Psammoma bodies present, can have calcification
1 in 400
What is the lifetime risk of a oculodermal melanocytosis turning into a melanoma?
What location of ocular melanoma has the best prognosis?
3-10% of all uveal melanomas
Most common in inferior iris
Rx with excision if documented growth, can consider brachytherapy in select cases
Prognosis excellent (1-4% mortality) and much lower than other melanomas
Describe Iris Melanoma:
Often hard to detect and quite large at presentation.
Diagnose with slit lamp and GONIO
Dilated episcleral vessel or sectoral cataract be be suggestivet
Describe a Ciliary Body Melanoma:
Typically a pigmented, elevated, dome shaped, subretinal mass. Characterized by high amplitude intial echoes and
low internal reflectivity on A-scan
After diagnosis rule out systemic metastasis or primary focus with liver imaging, LFTs, and CXR (if any abnormal then goto CT/MRI/PET)
Treat with enucleation, brachytherapy (most common), charged partical radiation, or exteneration (rare today)
Describe ultrasound characteristics, clinical evaluation, and treatment of a Choroidal Melanoma:
It delivers a more homogenous dose of radiation to the tumor than brachytherapy with less lateral spread to tissues. Does have high rate of complications to anterior segment structures.
What are advantages and disadvantages of charged particle radiation in the treatment of melanoma (as compared to brachytherapy)?
Where is the most common location for a choroidal melanoma to metastasize?
Iodine-125 (most common) and ruthenium-106
What are the most common isotypes used in brachytherapy?
Transscleral diatherpy as it can provide a route for extrascleral extension from induced scleral damage
What treatment is CONTRAINDICATED for choroidal melanoma?
A benign bony tumor that typically arises from the juxtapapillary choroid in adolescent to young adults.
Appears yellow-orange and has well-defined margins. Ultrasound shows high-amplitude echo corresponding to the plate of bone, shows calcification on CT.
Typically enlarge slowly over the years and can impair vision if macular involved
Describe a choroidal osteoma:
To Find Small Ocular Melanoma
T - thickness > 2mm
F - fluid (subretinal)
S - symptoms (photopsias/floaters)
O - Orange pigment
M - margin < 3mm from optic disc
What is the mnemonic for assessing risk of a choroidal nevus for a melanoma?
Large Melanoma Trial:
-That enucleation alone was equal to external-beam radiotherapy followed by enucleation
Medium Melanoma Trial:
-Enucleation and brachytherapy were equal
Small Melanoma Trial:
-melanoma specific mortality of 1% for small tumors 1-2.4mm in height
What were the findings in the COMS (Collaborative Ocular Melanoma Study)?
A benign proliferation of the nonpigmented ciliary epithelium
Usually an incidental finding at autopsy and rarely becomes apparent clinically.
Appears as a glistening, white, irregular tumor from a ciliary crest.
What is a Fuchs adenoma?
: No systemic association, appears as red or orange tumor often in macula. Commonly produce a secondary RD causing decreased vision.
: Associated with Sturge-Weber. Produces diffuse reddish-orange fundus (tomato ketchup fundus) and can cause RD and glaucoma.
On Ultrasound has high internal reflectivitiy and FA shows large choroidal vessels in the early phases and late staining of the tumor.
Observe if not symptomatic. Otherwise treat with PDT (treatment of choice), laser, brachytherapy/charged-particle/external beam radiation.
Describe the two types of choroidal hemangioma?
Occurs in VHL
Can cause exudative detachment. FA shows rapid AV transit, immediate filling of the feeding vessel, and drainage by a large vein.
Rx with photocoagulation, cryo, scleral buckle + cryo for large RD, or penetrating diathermy if very extensive.
Consists of dilated, thin-walled vascular channels.
Rarely need treatment as exudation is not common.
FA shows slow filling, with fluorescein pooling in the upper part of the vascular space and erythrocytes pooling in the lower part. No leakage.
A retinal arterio-venous malformation
If associated with an AV malformation of the midbrain it is called Wyburn-Mason
No leakage on FA
Mutation in RB1 tumor supressor gene
A clinical diagnosis (best to avoid FNAB due to risk of seeding). Most common presenting signs are leukocoria, strabismus, and ocular inflammation
Ultrasound can show characteristic calcifications. MRI is best to assess orbits and brain as CT can give radiation.
: Definitive treatment if tumor is >50% of globe, anterior segment/orbital/optic nerve involvement, neovascular glaucoma, or limited vision potential.
: systemic or local, can shrink tumor for laser.
Photocoagulation with Hyperthermia
: 2-3 rows of laser destroy the blood supply
Cryotherapy, External beam radiation, or brachytherapy (mainly a salvage therapy if globe conserving therapy fails)
Survival over 95% in developed countries.
1) 45% (85% bilateral)
2) 7-15% (85% bilateral)
3) <<1% (33% bilateral)
What is the chance a child has retinoblastoma if:
1) Parent has bilateral retinoblastoma
2) Parent has unilateral retinoblastoma
3) Parent is unaffected
Persistent Fetal Vasculature
: Associated with microphthalmos, shallow/flat AC, hypoplastic iris with prominent vessels, and retrolenticular fibrovascular mass.
: More common in boys, characterized by unilateral retinal telangiectasia associated with intraretinal yellow exudation without a distinct mass
: typically in older children with a history of soil ingestion or exposure to puppies.
: Generally a small, smooth, white, glistening tumor located in the nerve fiber layer of the retina. Commonly arise from the optic disc and present in tuberous sclerosis.
: Both benign and malignant forms, typically in children but can also be in adults. Treatment usually enucleation or observation.
What is the DDX for retinoblastoma?
Though benign, retinocytoma carries the same genetic implications as retinoblastoma.
What is the risk of a child developing retinoblastoma if a parent had a retinocytoma?
Osteosarcoma (most common), pinealoma, brain tumors, cutaneous melanomas, and soft tissue sarcomas.
What are common malignancies that survivors of retinoblastoma develop?
Posterior choroid due to the rich vascular supply
Where to metastasis to the eye commonly occur?
What part of the eye is involved in primary intraocular lymphoma and in systemic lymphoma?
30% present unilaterally, up to 85% eventually have bilateral involvement.
Clinically has dense vitritis and yellow-white subretinal infiltrates.
Diagnose with undiluted vitreous biopsy. Associated with increased IL-10 to IL-6 ratio.
Rx with radiotherpy + systemic or intrathecal chemo.
Describe Primary Intraocular Lymphoma:
Formerly known as reactive lymphoid hyperplasia
Typically presents in 6th decade of life and can occur at any uveal site. Similar lymphoid proliferation can occur in orbit or conj.
Patients notice painless, progressive vision loss. A diffuse or rarely nodular amelanotic thickening of the choroid is noted.
Path shows diffuse infiltration of the uveal tract by lymphoid cells.
Rx with steroids or low dose radiotherapy.
What is Uveal Lymphoid Infiltration?
Ocular Findings in Leukemia:
Retina most commonly involved clinically, uvea histologically
Characterized by intraretinal and subhyaloidal hemorrhages, hard exudates, CWS, and white centered hemorrhages (Roth Spots)
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