Orbital Neoplasms and Malformations Flashcards
Most common primary benign tumor of the orbit in children?
capillary hemangioma
Typical clinical course of capillary hemangioma?
present at birth or first few weeks of life, enlarge dramatically over first 6-12 months then begin to involute after first year of life. 75% resolve during first 3-7 years of life
Common orbital locations for capillary hemangiomas?
superonasal quadrant of orbit or medial upper eyelid
Multiple large visceral capillary hemangiomas producing thrombocytopenia?
Kasabach-Merritt syndrome
First line tx for capillary hemangioma?
Second line
- Observation, amblyopia treatment, refractive correction
2. Oral propranolol or topical timolol gel
Most common benign orbital neoplasm in adults?
Cavernous hemangioma
Risk factors for capillary hemangioma?
female sex, preterm birth, maternal chorionic villus sampling,
First line treatment for symptomatic cavernous hemangiomas?
complete surgical excision
Diagnosis and treatment for well-encapsulate vascular lesion in orbit that appears blue in gross specimen and whose pathology shows plump pericytes that surround a rich capillary network?
hemangiopericytoma; complete excision (may recur or metastasize)
Orbital lesion that may enlarge during URI?
lymphatic malformation (aka lymphangioma)
Orbital mass with MRI appearance of multiple grape-like cystic lesions with fluid-filled layering of serum and blood?
lymphatic malformation (aka lymphangioma)
Treatment of lymphatic malformation (aka lymphangioma)?
previously surgical excision, but more recently sclerosing agents have been used first line
Diagnosis and treatment of proptosis that increases with valsalva or head down position?
orbital varix; conservative management, surgery only if vision threatening or very painful
Blood in the superior ophthalmic vein during arterial phase of CT?
Carotid-cavernous fistula
Proptosis, orbital bruit and corkscrew episcleral vessels?
Carotid-cavernous fistula
Ocular complications of direct c-c fistula?
Ischemic ocular damage, elevated IOP, choroidal effusion, blood in Schlemm canal, nongranulomatous anterior uveitis, EOM palsies,
Mechanisms of direct and indirect (dural) c-c fistulas?
Direct: traumatic tear in ICA where it passes through cavernous sinus
Indirect: insidious, degenerative fistula formation between meningeal branches of ICA/ECA/or both and cavernous sinus in patients with HTN, vascular dz, atherosclerosis
What structures pass through the cavernous sinus?
CN III, IV, VI; V1 and V2; ICA and sympathetic nerves
Gradual, painless, unilateral axial proptosis with vision loss and APD?
optic nerve glioma or optic nerve sheath meningioma
Percentage of optic nerve gliomas associated with neurofibromatosis?
50%
Fusiform enlargement of optic nerve with stereotypical kinking?
optic nerve glioma
S-shaped configuration of eyelid?
plexiform neurofibroma (in NF1, not NF2)
Orbital features of NF1?
plexiform neurofibroma of eyelid, pulsating proptosis from sphenoid wing dysplasia, optic nerve glioma
Tram-track enlargement of optic nerve sheath on MRI?
optic nerve sheath meningioma
Biphasic pattern of solid areas with nuclear palisading(1) and myxoid areas (2)?
Schwannoma
(1) Antoni A
(2) Antoni B
Most common primary orbital malignancy of childhood?
rhabdomyosarcoma
Sudden onset of rapidly progressive unilateral proptosis in a 6 year old?
rhabdomyosarcoma
Unilateral eyelid edema and discoloration in a child?
rhabdomyosarcoma
Initial management for suspected rhabdomyosarcoma?
biopsy (usually through anterior orbitotomy)
Treatment of rhabdomyosarcoma?
chemo and radiation
Four types of rhabdomyosarcoma?
embryonal, botryoid, alveolar, pleomorphic
Most common subtype of rhabdomyosarcoma?
Least common?
Worst prognosis?
Best prognosis?
embryonal
pleomorphic
alveolar
pleomorphic
Hyperostotic orbital bone with lack of dural enhancement, associated with cutaneous pigmentation and endocrine disorders?
fibrous dysplasia as part of Albright syndrome
Most common type of orbital lymphoproliferative disease?
non-Hodgkin B-cell lymphoma
salmon patch conjunctival lesion?
orbital lymphoproliferative lesion
Treatment of choice for localized ocular adnexal lymphoproliferative disease?
radiation
Lytic bone lesion of superotemporal orbit with relapsing episodes of orbital inflammation in 5-10 year old misdiagnosed as orbital cellulitis?
Langerhans cell histiocytosis
Periocular xanthogranuloma, asthma, lymphadenopathy, increased IgG?
AAPOX (Adult-onset asthma with periocular xanthogranuloma)
subcutaneous fatty lesions in eyelid and orbit that ulcerate and fibrose, associated with paraproteinemia?
NBX (necrobiotic xanthogranuloma)
Dense, progressive, recalcitrant, fibrosclerosis of the orbit and internal organs leading to vision loss and frequently death?
ECD (Erdheim-Chester disease; form of adult xanthogranuloma)
Non-Langerhans histiocytic disorder that occurs as a self-limited, steroid-sensitive, and usually focal subcutaneous disease of childhood?
JXG (Juvenile xanthogranuloma)
Most common lacrimal gland mass?
nonspecific inflammation (dacryoadenitis)
Most common site of orbital lymphoma?
lacrimal gland fossa (50%)
Most common non-inflammatory lacrimal gland mass?
lymphoproliferative disorders
Two general subtypes and associated percentages of lacrimal gland epithelial tumors?
benign mixed (aka pleomorphic adenoma - 50%) and carcinoma (50%)
Most common malignant tumor of lacrimal gland?
adenoid cystic carcinoma
Most common epithelial tumor of lacrimal gland?
pleomorphic adenoma
Diagnosis and management of progressive, painless downward and inward displacement of globe in 50-year old?
pleomorphic adenoma; complete excision of tumor with reservation of pseudocapsule and with margins (no biopsy, which leads to higher chance of malignancy)
Swiss-cheese pattern of benign-appearing cells on lacrimal gland biopsy?
adenoid cystic carcinoma
Painful lacrimal gland mass appearing over less than a year?
adenoid cystic carcinoma (pain from perineural invasion and bony destruction)
Chronic subclinical sinusitis causing orbital floor collapse and enophthalmos?
silent sinus syndrome
Most common location of metastatic tumors in children?
In adults?
to orbit
to choroid
Abrupt ecchymotic proptosis that may be bilateral?
metastatic neuroblastoma
Ipsilateral Horner syndrome with heterochromia?
congenital neuroblastoma of cervical ganglion
Treatment and prognosis of neuroblastoma?
chemotherapy first line; 90% survival if diagnosed before 1 year of age, 10% if diagnosed later
Most common orbital met in men?
bronchogenic carcinoma
Most common site of orbital met in adults?
Second most common?
EOMs (because of rich blood supply)
marrow of sphenoid bone
Lytic destruction of lateral orbital wall?
metastasis
Most common source of orbital met in women?
breast cancer
Clinical presentation of breast cancer met to orbit?
enopthalmos (generally not proptosis), with possible restriction of EOM
