Lumps & Bumps: Iris, Orbit, Lymph & Neurogenic

Question 1

benign peripheral nerve sheath tumor composed of a bundle of enlarged peripheral nerves caused by proliferation of axons, fibroblasts & Schwann cells

Answer 1

neurofibroma (NF)

Question 2

characteristics:
- can affect skin in all parts of the body
- can occur as solitary lesions, or multifocal diffuse lesions (NF1)
- eyelid & orbit involvement
- plexiform neurofibroma → causes S-shaped curve of upper eyelid & feels like a “bag of worms: upon palpation
- typically stable, but can sometimes enlarge
- proptosis possible with orbital involvement

Answer 2

neurofibroma

Question 3

what is plexiform neurofibroma

Answer 3

a benign tumor of peripheral nerves

Question 4

management of neurofibroma

Answer 4

observation or resection if significant growth/pain develops

Question 5

tumor that is usually associated with systemic lymphoma

Answer 5

eyelid lymphoma

Question 6

characteristics:
- tumors can be benign, intermediate & malignant
- suspect immunocompromised state in younger patients (AIDS)

Answer 6

eyelid lymphoma

Question 7

management/treatment for eyelid lymphoma

Answer 7

biopsy for confirmation
- local → radiotherapy
systemic → chemotherapy

Question 8

iris lesion that consists of aggregation of connective tissue (can be normal finding in light iride invidividuals)

Answer 8

brushfield spots

Question 9

characteristics:
- small whitish peripheral iris speckles arranged in a concentric ring
- no malignant potential

Answer 9

brushfield spots

Question 10

frequently seen in patients with Down syndrome

Answer 10

brushfield spots

Question 11

iris lesion caused by pigment aggregation of melanocytes & spindle cells in the superficial iris stroma

Answer 11

lisch nodules

Question 12

characteristics:
- multiple nodules only found in patients with NF1 (present by age 6 in patients with NF1)
- no malignant potential

Answer 12

lisch nodules

Question 13

iris lesion caused by benign proliferation of melanocytes in superficial iris stroma

Answer 13

iris nevus

Question 14

characteristics:
- flat or variably elevated pigmented lesions

risk of malignant transformation:

• younger age (< 40)
• inferior location
• diffuse iris involvement
• bleeding from lesion

Answer 14

iris nevus

Question 15

how do you manage an iris nevus

Answer 15

lifelong observation & size documentation

Question 16

a benign iris lesion that arises from iris pigment epithelium

Answer 16

iris cyst

Question 17

characteristics:
- typically asymptomatic & non-progressive
- can cause elevated IOP due to angle obstruction
- best viewed with ultrasound biomicroscopy
- can be viewed with wide dilation

Answer 17

iris cyst

Question 18

management of iris cyst

Answer 18

must rule out iris melanoma (hollow center on B-scan & UBM)

Question 19

malignant iris lesion that is pigmented or amelanotic

Answer 19

iris melanoma

Question 20

characteristics:
- elevated with feeder vessels
- history of nevus undergoing growth

Answer 20

iris melanoma

Question 21

management of iris melanoma

Answer 21

URGENT referral to ocular oncology

Question 22

benign orbital lesion caused by protrusion of orbital fat through a defect in Tenon’s capsule into the conjunctival fornix

Answer 22

orbital fat prolapse

Question 23

characteristics:
- soft yellow mass in superotemporal conjunctival fornix
- predilection for older obese male patients

Answer 23

orbital fat prolapse

Question 24

benign congenital orbital cystic lesion due to entrapped ectoderm at side of embryologic bony structure

Answer 24

dermoid cyst

Question 25

characteristics:
- firm, non-movable subcutaneous mass
- filled with hair follicles, sebaceous glands, & sweat glands
- can cause significant inflammatory reaction if it ruptures

Answer 25

dermoid cyst