Disorders 1 Flashcards
(41 cards)
dermoid cyst (choristoma)
arises from dermal elements (neural crest)
MC orbital mass in childhood
lined by keratinizing dpi with dermal appendages
usually near SUPEROTEMPORAL quad
can lead to bony erosion and rupture can lead to severe inflammation
CT: well circumscribed with bony molding
TX: remove, en bloc
Epidermoid Cyst
ONLY epidermis, NO dermal appendages
filled with keratin
rupture = acute inflammatory process
Lipodermoid
solid tumor, beneath conj over lateral surface of glove
hard to excise; can look like prolapsed orbital fat, prolapsed lacrimal gland or lymphoma
Teratoma
rare, cystic tumor arises from >2 germinal layers (ectoderm + endoderm and/or mesoderm)
capilalry hemangioma
MC benign tumor
starts first few weeks of life, increases over first 6-12 months, regression by 5-8 yo
superior nasal quadrant of orbit and medial upper eyelid
F > M
diffuse unencapsulated irregular mass of plump endothelial cells/small vascular channels
s/s: strawberry nevus, when in orbit can look blue and with proptosis
CT: well circumscribed lesion
TX: obs, propanolol
Complications: high output CHF with multiple hemangiomas; respiratory compromise with subglottic ones
strabismus/amblyopia
Kassabach- Merrit syndrome
consumptive coagulopathy, platelet trapping, leads to thrombocytopenia and cardiac failure; 30% mortality
unencapsulated cysts
capillary hemangioma
lymphangioma
lymphangioma
rare, lymphatic filled choristoma
superonasal
eyelids, conj, deeper orbital tissues
waxes and wanes; does NOT involute
acute pain, proptosis, bluish hue to overlying lid, can hemorrhage into channels (chocolate cyst)
path: lymph filled vascular channels lined with endothelium
CT/MRI: infiltrative pattern
TX: obs, rarely surgical b/c hard to completely excise and can recur
Albright Syndrome
polyostotic fibrous dysplasia (multiple bones involved); short stature, premature closure of epiphyssis, precocious puberty, hyperpigmented macules
cavernous hemangioma
MC benign tumor in adults
slow porgressive proptosis
can induce hyperopia
CT: well demarcated, ENCAPSULATED intraconal mass
path: lesion filled with flood filled cavernous spaces, lined by endothelial cells
MRI: hypointense to fat on T1 weighted images, hyperintense to fat and equivalent to vitreous on T2 weighted images
hemangiopericytoma
rare tumor, abnl pericytes surround blood vessels
middle aged women
can METASTASIZE to lung, bone, and liver
slowly progressive proptosis, can be painful
CT: well circumscribed, encapsulated mass
Tx: COMPLETE removal
Schwannoma
encapulated
no malignant potential
can be painful
superior orbit
can grow along ANY peripheral or cranial nerve (MC = CN 8)
rarely associated with NF
path: S-100 stain (stains everything of neural crest origin)
Antoni A: spindle cells arranged in interlacing cords, whorls, or palisades, have verocay bodies
Antoni B: loose, myxoid, stellatTe cells with a mucoid stroma
CT: well circumscribed, fusiform mass
TX: complete surgical excision, can recur
optic nerve sheath meningioma
MC women
increased incidence in NF paitnets
from arachnoid cells in meninges
have decreased VA/color vision, visual field loss, proptosis, disc edema or pallor
path: sheets of cells or whorls, psammoma bodies (calcified) in the center
CT: tram track enhancement
TX: obs, surgery to prevent involvement of optic nerve/chiasm
spencer’s triad
optociliary shunts, optic atrophy, optic nerve mengioma
sphenoid wing meningioma
MC tumor to spread to orbit from intracranial spac
s/s: temporal fullness, proptosis, lid edema
CT: hyperostosis/calcification
Tx: obs/excise
Lymphoid tumor
abnl proliferation of lymphoid tissue make up 20% of all orbital tumors 90% are non Hodgkins B-cell are conjunctival/lacrimal gland lesions painLESS proptosis MUST have workup for systemic lymphoma
benign reactive lymphoid hyperplasia
bilateral painless enlargement of the lacrimal gland
path: mature lymphocytes with reactive germinal centers; T cells; 60-80% with scattered polyclonal B cells
atypical lymphoid hyperplasia
LOW grade lymphoma, no mitotic activity
path: follicles and polymorphous response = benign process
40% develop systemic disease in 5 years
lymphoma
superior orbit
adults
s/s: limitation of EOM, painless lacrimal gland swelling, conj salmon patch
path: ATYPICAL immature lymphocytes with mitoses; monoclonal B-cell proliferations 60-90% with scattered or reactive T cells
CT: putty like molding of tumor, orbital tissues NOT displaced
TX: radiotherapy, chemo
more like to be systemic with in eyelids > orbit > conj
Ossifying fibroma
well circumscribed slow growing monostotic (only in one bone) lesion
path: vascular stroma with lamellar bone with a rim of osteoid and osteoblasts
osteoma
dense bony lesions originating int eh frontal and ehmoid sinus
well circumscribed, slow growing mass
sxs 2/2 sinus obstruction/intracranial or intraorbital extension
path: lamellar bone with variable amounts of fibrous stroma
fibrous histiocytoma
firm orbital mass made up of fibroblasts and histiocytes
usually beign
MC mesenchymal orbital lesion of adults
path: storiform (cartwheel or spiral), nebular pattern of tumor cells (fibroblasts)
CT: well circumscribed mass anywhere in orbit, MC upper nasal
Pleomorphic adenoma (benign mixed tumor)
MC epithelial tumor of the lacrimal gland
4th-5th decade
MC male
slow onset
firm mass in lacrimal fossa with painless proptosis, globe will be medial and down
path: proliferation of epithelial cells into a double layer, makes lumina with ductal and secretory elements; ductal inner cells secrete mucus; outer stromal cells give rise to fibrous stroma/osteoid and cartilaginous metaplasia
TX: complete en bloc resection (incomplete can lead to malignant transformation into pleomorphic adenocarcinoma
pleomorphic adenocarcinoma (malignant mixed tumor)
mass in lacrimal fossa for a long time elderly individuals rapid progressive painful proptosis path: similar to benign mixed tumor but with foci of malignant change tx: radical orbitectomy and bone removal
