Orbit: Benign and Malignant Lesions

Question 1

orbital cavernous hemangioma

Answer 1

• a collection of small blood vessels surrounded by a fibrous pseudocapsule
• large vascular spaces are within the mass, thus the term cavernous

Question 2

orbital cavernous hemangioma etiology/associations

Answer 2

thought to be a congenital venous malformation

Question 3

orbital cavernous hemangioma demographics

Answer 3

• typically diagnosed between the ages of 30-50 years

- women > men

Question 4

orbital cavernous hemangioma laterality

Answer 4

unilateral

Question 5

orbital cavernous hemangioma symptoms

Answer 5

• bulging eye/displaced eye

- double vision

Question 6

orbital cavernous hemangioma signs

Answer 6

• proptosis
• globe displacement
• restricted EOM

Question 7

orbital cavernous hemangioma complications

Answer 7

• compression on the globe and/or optic nerve

- thrombosis

Question 8

orbital cavernous hemangioma management

Answer 8

• orbital CT or MRI
• if symptomatic or complications, refer out
• complete surgical excision

Question 9

orbital cavernous hemangioma pearls:

• most common ____
• most common location is ____
• growth may accelerate during _____

Answer 9

benign orbital tumor in adults;
within the muscle cone of the orbit;
pregnancy

Question 10

orbital venous varix (orbital varices)

Answer 10

abnormally enlarged vein that becomes dilated with increased venous pressure (e.g., during Valsalva maneuvers, bending forward, or breath holding)

Question 11

orbital venous varix (orbital varices) etiology/associations

Answer 11

thought to be a congenital venous malformation

Question 12

orbital venous varix (orbital varices) demographics

Answer 12

typically diagnosed between the ages of 10-20 years (but can occur later)

Question 13

orbital venous varix (orbital varices) laterality

Answer 13

unilateral

Question 14

orbital venous varix (orbital varices) symptoms

Answer 14

• intermittent bulging eye/displaced eye

- intermittent double vision

Question 15

orbital venous varix (orbital varices) signs

Answer 15

• intermittent proptosis
• intermittent globe displacement
• intermittent restricted EOM

Question 16

orbital venous varix (orbital varices) complications

Answer 16

• compression on the globe and/or optic nerve
• orbital hemorrhage; rapid and painful proptosis
• thrombosis; rapid and painful proptosis

Question 17

orbital venous varix (orbital varices) management

Answer 17

• orbital CT or MRI
• if complications refer out
• surgery or embolization

Question 18

orbital venous varix (orbital varices) pearls:

-may also have ____

Answer 18

eyelid and conjunctival varices

Question 19

lacrimal gland pleomorphic adenoma (benign mixed cell tumor)

Answer 19

benign tumor of the lacrimal gland

Question 20

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) etiology

Answer 20

proliferation of epithelial cells

Question 21

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) demographics

Answer 21

typically occurs between the ages of 30-40 years

Question 22

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) symptoms

Answer 22

• displaced eye
• double vision
• eyelid swelling and redness

Question 23

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) signs

Answer 23

• globe displacement inferiorly and medially
• restricted EOM
• eyelid edema and erythema
• palpable lacrimal gland mass

Question 24

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) complications

Answer 24

malignant transformation

Question 25

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) management

Answer 25

- orbital CT or MRI - refer out - complete surgical excision

Question 26

lacrimal gland pleomorphic adenoma (benign mixed cell tumor) pearls: - _____ progression occurs over the course of 12 months - _____ progression and pain may indicate malignancy

Answer 26

slow, painless; | rapid

Question 27

lacrimal gland carcinoma

Answer 27

malignant tumor of the lacrimal gland

Question 28

lacrimal gland carcinoma etiology

Answer 28

- adenoid cystic carcinoma: nest of basaloid cells - pleomorphic adenocarcinoma: proliferation of epithelial cells; usually develops within a long-standing benign mixed epithelial tumor or as a recurrence of a previously resected benign mixed tumor

Question 29

lacrimal gland carcinoma demographics

Answer 29

- adenoid cystic carcinoma: typically occurs between the ages of 30-40 - pleomorphic adenocarcinoma: typically occurs in the elderly

Question 30

lacrimal gland carcinoma laterality

Answer 30

unilateral

Question 31

lacrimal gland carcinoma symptoms

Answer 31

- displaced eye - double vision - eye pain

Question 32

lacrimal gland carcinoma signs

Answer 32

- globe displacement inferiorly and medially - restricted EOM - eyelid edema and erythema - palpable lacrimal gland mass

Question 33

lacrimal gland carcinoma complications

Answer 33

intracranial extension

Question 34

lacrimal gland carcinoma management

Answer 34

- orbital CT or MRI - refer out - complete surgical excision - exenteration if extensive - radiotherapy and chemotherapy

Question 35

lacrimal gland carcinoma pearls: - onset of pain is ____ - five-year survival rate is ____, and at 15 years is _____ - major cause of death is ____

Answer 35

rapid (over 1-3 months); 47%; only 22%; intracranial extension

Question 36

orbital lymphoma

Answer 36

malignant tumor of the orbit; any part of the orbit may be affected with ~50% of cases affecting the lacrimal gland

Question 37

orbital lymphoma etiology/associations

Answer 37

proliferation of lymphocytes

Question 38

orbital lymphoma demographics

Answer 38

typically occurs between the ages of 50-70 years

Question 39

orbital lymphoma laterality

Answer 39

unilateral > bilateral

Question 40

orbital lymphoma symptoms

Answer 40

- bulging/displaced eye - double vision - droopy eyelid - eyelid swelling - eye pain in a minority of cases

Question 41

orbital lymphoma signs

Answer 41

- proptosis - globe displacement - restricted EOM with possible pain on eye movement - ptosis - palpable mass if anterior - may have pink salmon-patch area of subconjunctival extension

Question 42

orbital lymphoma complications

Answer 42

- compression on the globe and/or optic nerve | - metastasis

Question 43

orbital lymphoma management

Answer 43

- orbital CT or MRI - refer out - biopsy - radiotherapy (external beam) with or without chemotherapy (chemotherapy is considered if there is metastasis)

Question 44

orbital lymphoma pearls: - ____% chance of developing systemic lymphoma within 5 years - lymphoma represents one end of the spectrum of ____; at the other end of the spectrum is benign reactive lymphoid hyperplasia (demographics, laterality, signs and symptoms are similar, transformation to malignancy can occur)

Answer 44

60; | lymphoproliferative lesions;

Question 45

orbital rhabdomyosarcoma

Answer 45

malignant tumor of the orbit

Question 46

orbital rhabdomyosarcoma etiology/associations

Answer 46

proliferation of primitive mesenchymal stem cells that normally develop into skeletal muscle

Question 47

orbital rhabdomyosarcoma demographics

Answer 47

- infancy to adulthood | - 70% occur between ages of 8-10 years

Question 48

orbital rhabdomyosarcoma laterality

Answer 48

unilateral

Question 49

orbital rhabdomyosarcoma symptoms

Answer 49

- bulging/displaced eye - double vision - droopy eyelid - eyelid swelling and redness in a minority of cases - ocular pain and redness in a minority of cases

Question 50

orbital rhabdomyosarcoma signs

Answer 50

- proptosis - globe displacement - restricted EOM with possible pain on eye movement - ptosis - eyelid edema and erythema - conjunctival injection and chemosis

Question 51

orbital rhabdomyosarcoma complications

Answer 51

- compression on the globe and/or optic nerve - erosion of orbital bones - metastasis

Question 52

orbital rhabdomyosarcoma management

Answer 52

- refer to ED - orbital CT or MRI - biopsy - radiotherapy (external beam) and chemotherapy

Question 53

orbital rhabdomyosarcoma pearls: - most common _____ - ____ new cases per year - most common location is the _____ - can also present, less commonly, in _____ - hallmarks are _____ - _____ 5-year survival rate

Answer 53

``` malignant orbital tumor in children; ~35; superior nasal orbit; conjunctiva, eyelid, iris, or ciliary body; rapid onset and progression; 75-95% ```

Question 54

orbital rhabdomyosarcoma: - ____ has a similar presentation to rhabdomyosarcoma - 2nd most common orbital malignancy after rhabdomyosarcoma - most common _____ - usually arises from ____ - patients typically have sudden _____ - may develop ____ - prognosis is _____

Answer 54

metastatic neuroblastoma; pediatric orbital metastatic tumor; a primary tumor in the abdomen, mediastinum, or neck; proptosis with eyelid ecchymosis that may be bilateral; ipsilateral Horner's syndrome; poor