Orbital Neoplasms and Malformations

Question 1

Most common primary benign tumor of the orbit in children?

Answer 1

capillary hemangioma

Question 2

Typical clinical course of capillary hemangioma?

Answer 2

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

Question 3

Common orbital locations for capillary hemangiomas?

Answer 3

superonasal quadrant of orbit or medial upper eyelid

Question 4

Multiple large visceral capillary hemangiomas producing thrombocytopenia?

Answer 4

Kasabach-Merritt syndrome

Question 5

First line tx for capillary hemangioma?

Second line

Answer 5

1. Observation, amblyopia treatment, refractive correction

2. Oral propranolol or topical timolol gel

Question 6

Most common benign orbital neoplasm in adults?

Answer 6

Cavernous hemangioma

Question 7

Risk factors for capillary hemangioma?

Answer 7

female sex, preterm birth, maternal chorionic villus sampling,

Question 8

First line treatment for symptomatic cavernous hemangiomas?

Answer 8

complete surgical excision

Question 9

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?

Answer 9

hemangiopericytoma; complete excision (may recur or metastasize)

Question 10

Orbital lesion that may enlarge during URI?

Answer 10

lymphatic malformation (aka lymphangioma)

Question 11

Orbital mass with MRI appearance of multiple grape-like cystic lesions with fluid-filled layering of serum and blood?

Answer 11

lymphatic malformation (aka lymphangioma)

Question 12

Treatment of lymphatic malformation (aka lymphangioma)?

Answer 12

previously surgical excision, but more recently sclerosing agents have been used first line

Question 13

Diagnosis and treatment of proptosis that increases with valsalva or head down position?

Answer 13

orbital varix; conservative management, surgery only if vision threatening or very painful

Question 14

Blood in the superior ophthalmic vein during arterial phase of CT?

Answer 14

Carotid-cavernous fistula

Question 15

Proptosis, orbital bruit and corkscrew episcleral vessels?

Answer 15

Carotid-cavernous fistula

Question 16

Ocular complications of direct c-c fistula?

Answer 16

Ischemic ocular damage, elevated IOP, choroidal effusion, blood in Schlemm canal, nongranulomatous anterior uveitis, EOM palsies,

Question 17

Mechanisms of direct and indirect (dural) c-c fistulas?

Answer 17

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

Question 18

What structures pass through the cavernous sinus?

Answer 18

CN III, IV, VI; V1 and V2; ICA and sympathetic nerves

Question 19

Gradual, painless, unilateral axial proptosis with vision loss and APD?

Answer 19

optic nerve glioma or optic nerve sheath meningioma

Question 20

Percentage of optic nerve gliomas associated with neurofibromatosis?

Answer 20

50%

Question 21

Fusiform enlargement of optic nerve with stereotypical kinking?

Answer 21

optic nerve glioma

Question 22

S-shaped configuration of eyelid?

Answer 22

plexiform neurofibroma (in NF1, not NF2)

Question 23

Orbital features of NF1?

Answer 23

plexiform neurofibroma of eyelid, pulsating proptosis from sphenoid wing dysplasia, optic nerve glioma

Question 24

Tram-track enlargement of optic nerve sheath on MRI?

Answer 24

optic nerve sheath meningioma

Question 25

Biphasic pattern of solid areas with nuclear palisading(1) and myxoid areas (2)?

Answer 25

Schwannoma (1) Antoni A (2) Antoni B

Question 26

Most common primary orbital malignancy of childhood?

Answer 26

rhabdomyosarcoma

Question 27

Sudden onset of rapidly progressive unilateral proptosis in a 6 year old?

Answer 27

rhabdomyosarcoma

Question 28

Unilateral eyelid edema and discoloration in a child?

Answer 28

rhabdomyosarcoma

Question 29

Initial management for suspected rhabdomyosarcoma?

Answer 29

biopsy (usually through anterior orbitotomy)

Question 30

Treatment of rhabdomyosarcoma?

Answer 30

chemo and radiation

Question 31

Four types of rhabdomyosarcoma?

Answer 31

embryonal, botryoid, alveolar, pleomorphic

Question 32

Most common subtype of rhabdomyosarcoma? Least common? Worst prognosis? Best prognosis?

Answer 32

embryonal pleomorphic alveolar pleomorphic

Question 33

Hyperostotic orbital bone with lack of dural enhancement, associated with cutaneous pigmentation and endocrine disorders?

Answer 33

fibrous dysplasia as part of Albright syndrome

Question 34

Most common type of orbital lymphoproliferative disease?

Answer 34

non-Hodgkin B-cell lymphoma

Question 35

salmon patch conjunctival lesion?

Answer 35

orbital lymphoproliferative lesion

Question 36

Treatment of choice for localized ocular adnexal lymphoproliferative disease?

Answer 36

radiation

Question 37

Lytic bone lesion of superotemporal orbit with relapsing episodes of orbital inflammation in 5-10 year old misdiagnosed as orbital cellulitis?

Answer 37

Langerhans cell histiocytosis

Question 38

Periocular xanthogranuloma, asthma, lymphadenopathy, increased IgG?

Answer 38

AAPOX (Adult-onset asthma with periocular xanthogranuloma)

Question 39

subcutaneous fatty lesions in eyelid and orbit that ulcerate and fibrose, associated with paraproteinemia?

Answer 39

NBX (necrobiotic xanthogranuloma)

Question 40

Dense, progressive, recalcitrant, fibrosclerosis of the orbit and internal organs leading to vision loss and frequently death?

Answer 40

ECD (Erdheim-Chester disease; form of adult xanthogranuloma)

Question 41

Non-Langerhans histiocytic disorder that occurs as a self-limited, steroid-sensitive, and usually focal subcutaneous disease of childhood?

Answer 41

JXG (Juvenile xanthogranuloma)

Question 42

Most common lacrimal gland mass?

Answer 42

nonspecific inflammation (dacryoadenitis)

Question 43

Most common site of orbital lymphoma?

Answer 43

lacrimal gland fossa (50%)

Question 44

Most common non-inflammatory lacrimal gland mass?

Answer 44

lymphoproliferative disorders

Question 45

Two general subtypes and associated percentages of lacrimal gland epithelial tumors?

Answer 45

benign mixed (aka pleomorphic adenoma - 50%) and carcinoma (50%)

Question 46

Most common malignant tumor of lacrimal gland?

Answer 46

adenoid cystic carcinoma

Question 47

Most common epithelial tumor of lacrimal gland?

Answer 47

pleomorphic adenoma

Question 48

Diagnosis and management of progressive, painless downward and inward displacement of globe in 50-year old?

Answer 48

pleomorphic adenoma; complete excision of tumor with reservation of pseudocapsule and with margins (no biopsy, which leads to higher chance of malignancy)

Question 49

Swiss-cheese pattern of benign-appearing cells on lacrimal gland biopsy?

Answer 49

adenoid cystic carcinoma

Question 50

Painful lacrimal gland mass appearing over less than a year?

Answer 50

adenoid cystic carcinoma (pain from perineural invasion and bony destruction)

Question 51

Chronic subclinical sinusitis causing orbital floor collapse and enophthalmos?

Answer 51

silent sinus syndrome

Question 52

Most common location of metastatic tumors in children? | In adults?

Answer 52

to orbit | to choroid

Question 53

Abrupt ecchymotic proptosis that may be bilateral?

Answer 53

metastatic neuroblastoma

Question 54

Ipsilateral Horner syndrome with heterochromia?

Answer 54

congenital neuroblastoma of cervical ganglion

Question 55

Treatment and prognosis of neuroblastoma?

Answer 55

chemotherapy first line; 90% survival if diagnosed before 1 year of age, 10% if diagnosed later

Question 56

Most common orbital met in men?

Answer 56

bronchogenic carcinoma

Question 57

Most common site of orbital met in adults? | Second most common?

Answer 57

EOMs (because of rich blood supply) | marrow of sphenoid bone

Question 58

Lytic destruction of lateral orbital wall?

Answer 58

metastasis

Question 59

Most common source of orbital met in women?

Answer 59

breast cancer

Question 60

Clinical presentation of breast cancer met to orbit?

Answer 60

enopthalmos (generally not proptosis), with possible restriction of EOM