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Orbit, Eyelids, and Lacrimal System > Orbital Neoplasms and Malformations > Flashcards

Orbital Neoplasms and Malformations Flashcards

1
Q

Most common primary benign tumor of the orbit in children?

A

capillary hemangioma

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2
Q

Typical clinical course of capillary hemangioma?

A

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

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3
Q

Common orbital locations for capillary hemangiomas?

A

superonasal quadrant of orbit or medial upper eyelid

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4
Q

Multiple large visceral capillary hemangiomas producing thrombocytopenia?

A

Kasabach-Merritt syndrome

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5
Q

First line tx for capillary hemangioma?

Second line

A
  1. Observation, amblyopia treatment, refractive correction

2. Oral propranolol or topical timolol gel

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6
Q

Most common benign orbital neoplasm in adults?

A

Cavernous hemangioma

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7
Q

Risk factors for capillary hemangioma?

A

female sex, preterm birth, maternal chorionic villus sampling,

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8
Q

First line treatment for symptomatic cavernous hemangiomas?

A

complete surgical excision

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9
Q

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?

A

hemangiopericytoma; complete excision (may recur or metastasize)

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10
Q

Orbital lesion that may enlarge during URI?

A

lymphatic malformation (aka lymphangioma)

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11
Q

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

A

lymphatic malformation (aka lymphangioma)

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12
Q

Treatment of lymphatic malformation (aka lymphangioma)?

A

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

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13
Q

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

A

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

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14
Q

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

A

Carotid-cavernous fistula

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15
Q

Proptosis, orbital bruit and corkscrew episcleral vessels?

A

Carotid-cavernous fistula

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16
Q

Ocular complications of direct c-c fistula?

A

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

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17
Q

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

A

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

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18
Q

What structures pass through the cavernous sinus?

A

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

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19
Q

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

A

optic nerve glioma or optic nerve sheath meningioma

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20
Q

Percentage of optic nerve gliomas associated with neurofibromatosis?

A

50%

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21
Q

Fusiform enlargement of optic nerve with stereotypical kinking?

A

optic nerve glioma

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22
Q

S-shaped configuration of eyelid?

A

plexiform neurofibroma (in NF1, not NF2)

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23
Q

Orbital features of NF1?

A

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

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24
Q

Tram-track enlargement of optic nerve sheath on MRI?

A

optic nerve sheath meningioma

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25
Q

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

A

Schwannoma

(1) Antoni A
(2) Antoni B

26
Q

Most common primary orbital malignancy of childhood?

A

rhabdomyosarcoma

27
Q

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

A

rhabdomyosarcoma

28
Q

Unilateral eyelid edema and discoloration in a child?

A

rhabdomyosarcoma

29
Q

Initial management for suspected rhabdomyosarcoma?

A

biopsy (usually through anterior orbitotomy)

30
Q

Treatment of rhabdomyosarcoma?

A

chemo and radiation

31
Q

Four types of rhabdomyosarcoma?

A

embryonal, botryoid, alveolar, pleomorphic

32
Q

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

A

embryonal
pleomorphic
alveolar
pleomorphic

33
Q

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

A

fibrous dysplasia as part of Albright syndrome

34
Q

Most common type of orbital lymphoproliferative disease?

A

non-Hodgkin B-cell lymphoma

35
Q

salmon patch conjunctival lesion?

A

orbital lymphoproliferative lesion

36
Q

Treatment of choice for localized ocular adnexal lymphoproliferative disease?

A

radiation

37
Q

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

A

Langerhans cell histiocytosis

38
Q

Periocular xanthogranuloma, asthma, lymphadenopathy, increased IgG?

A

AAPOX (Adult-onset asthma with periocular xanthogranuloma)

39
Q

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

A

NBX (necrobiotic xanthogranuloma)

40
Q

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

A

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

41
Q

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

A

JXG (Juvenile xanthogranuloma)

42
Q

Most common lacrimal gland mass?

A

nonspecific inflammation (dacryoadenitis)

43
Q

Most common site of orbital lymphoma?

A

lacrimal gland fossa (50%)

44
Q

Most common non-inflammatory lacrimal gland mass?

A

lymphoproliferative disorders

45
Q

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

A

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

46
Q

Most common malignant tumor of lacrimal gland?

A

adenoid cystic carcinoma

47
Q

Most common epithelial tumor of lacrimal gland?

A

pleomorphic adenoma

48
Q

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

A

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

49
Q

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

A

adenoid cystic carcinoma

50
Q

Painful lacrimal gland mass appearing over less than a year?

A

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

51
Q

Chronic subclinical sinusitis causing orbital floor collapse and enophthalmos?

A

silent sinus syndrome

52
Q

Most common location of metastatic tumors in children?

In adults?

A

to orbit

to choroid

53
Q

Abrupt ecchymotic proptosis that may be bilateral?

A

metastatic neuroblastoma

54
Q

Ipsilateral Horner syndrome with heterochromia?

A

congenital neuroblastoma of cervical ganglion

55
Q

Treatment and prognosis of neuroblastoma?

A

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

56
Q

Most common orbital met in men?

A

bronchogenic carcinoma

57
Q

Most common site of orbital met in adults?

Second most common?

A

EOMs (because of rich blood supply)

marrow of sphenoid bone

58
Q

Lytic destruction of lateral orbital wall?

A

metastasis

59
Q

Most common source of orbital met in women?

A

breast cancer

60
Q

Clinical presentation of breast cancer met to orbit?

A

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

Orbit, Eyelids, and Lacrimal System (14 decks)

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