Masses/tumors

Question 1

most common orbital mass in childhood

Answer 1

dermoid cyst (choristoma)

Question 2

dermoid cyst

Answer 2

neural crest cell origin
Keratinizing epithelium with dermal appendages
Superior temporal quadrant near brow (usually)
rupture causes intense inflammation (remove en bloc)

Question 3

lipodermoid

Answer 3

solid tumor beneath conj over lateral surface of globe

Question 4

capillary hemangioma

Answer 4

enlarges over first 6-12 months then regresses by 5-8 yrs in 80% cases
superior nasal quadrant of orbit and medial upper eyelid
F > M (3:2)

Question 5

Most common benign orbital tumor in children

Answer 5

capillary hemangioma

Question 6

location of tumors:
Dermoid
capillary hemangioma
lymphangioma
Rhabdomyosarcoma

Answer 6

Dermoid - superior temporal quadrant near brow
Capillary hemangioma - superior nasal orbit or medial upper lid
Lymphangioma - superior nasal
Rhabdomyosarcoma - superior nasal

Question 7

Lymphangioma

Answer 7

lymphatic filled choristoma
superior nasal
waxes and wanes but does not involute (increases with URI)
chocolate cyst - hemorrhage into channels
rarely surgical excision because complete excision difficulty and recurrence common.

Question 8

Varix

Answer 8

dilation of preexisting venous channel
proptosis increases with crying or straining or head down.
orbital hemorrhage after trauma
phlebolith present in 30% of cases

Question 9

Fibrous Dysplasia

Answer 9

tumor of connective tissue, cartilage and bone
monostotic/polyostotic
Frontal bone most common
cause of unilateral proptosis in first 2 decades

Question 10

Albright’s Syndrome

Answer 10

polyostotic fibrous dysplasia (facial assymetry, decreased VA and hearing)
short stature
premature closure of epiphysis
precocious puberty
hyperpigmented macules

Question 11

Neurofibroma

Answer 11

Hamartoma
20% with NF 1
path: well circumscribed, nonencapsulated proliferatino of Schwann cells, perineural cells and axons
Stains with S-100 (for neural crest cells

Question 12

Optic Nerve gloma

Answer 12

pilocytic astrocytoma (slow growing hamartoma)
25-50% associated with NF1
Fusiform enlargement of ON (RR track)
malignant in adults, better prognosis in children

Question 13

Rhabdomyosarcoma:
Age, gender?
common location in orbit?
A scan?
metastasis?

Answer 13

avg age 8 yrs
unilateral, often superonasal orbit
M > F 5:3
A-scan - medium internal reflectivity
Lungs/lymph nodes in chest

Question 14

most common primary orbital malignancy of children

most common metastatic orbital tumor in children

Answer 14

rhabdomyosarcoma

Neuroblastoma

Question 15

most common soft tissue malignancy in children

Answer 15

rhabdomyosarcoma

Question 16

Types of rhabdomyosarcoma
most common?
best prognosis?
worst prognosis?

Answer 16

embyronal: most common, elongated spindle cells with central hyperchromatic nucleus in peripheral palisading bands
Botryoid: (subtype of embryonal) in anterior orbit
Pleomorphic: less common, adults, best prognosis, rare in orbit
Alveolar: worst prognosis, inferior orbit (extremities in adolescents)

Question 17

Rhabdomyosarcoma

prognosis/survival rate

Answer 17

3 yr survival = 90%, 100% cure if localized to orbit, 60% if invasion of adj structures
Intrathoracic lesions - worst, 24% disease free at 2 yrs

Question 18

neuroblastoma
originates?
findings?

Answer 18

usually originates in adrenal/sympathetic ganglion
40% mets to orbit
proptosis, periorbital echhymosis, ipsi horners, opsoclonus (saccadomania, not during sleep)

Question 19

Ewing’s Sarcoma

Answer 19

originates in long bones of extremities/axial skeleton
Mets to bones/lungs
2nd-3rd decades

Question 20

Histiocytosis X
type of cells involved?
poor prognosis in which group?

Answer 20

abnormal proliferation of histiocytes (langerhans’ cells)
children under 2 with multifocal disease have poor prognosis
Lytic defect of orbital roof = progressive proptosis

Question 21

Hand-Schuller-Christian Disease

Triad?

Answer 21

proptosis
lytic skull defects
diabetes insipidus

Question 22

Burkitt’s Lymphoma

Answer 22

primarily affects maxilla in black children, can have secondary invasion of orbit
Related to EBV
Path - malignant B cells with ‘starry sky’ appearance

Question 23

Epibulbar osseous choroistoma

Answer 23

mass composed of mature bone
bulbar conj (superior temporal fornix)
no malignant potential
observe or excise

Question 24

Medullopeithelioma (diktyoma)

Answer 24

primary neoplasm of ciliary body (nonpigmented epithelium)
benign and malignant forms
nonteratoid (simple) - proliferation of embyonic nonpigmented ciliary epithelium
Teratoid - herotropic eleemnts - cartilage, brain, rhabdomyoblasts
Sx: pain, leukocoria, rubeosis, hyphema, glaucoma

Tx: resection or enucleation