OPT 221 Study Guide: Understanding Malignant Lesions of the Orbit

What is a lacrimal gland carcinoma?

malignant tumor of the lacrimal gland

What is the etiology of lacrimal gland carcinoma?

-adenoid cystic carcinoma: nest of basaloid cells

-pleomorphic adenocarcinoma; proliferation of epithelial cells

What demographics are most affected by lacrimal gland carcinoma?

-adenoid cystic carcinoma: typically occurs between age 30-40

-pleomorphic adenocarcinoma: typically occurs in the elderly

What is the laterality of lacrimal gland carcinoma?

unilateral

What are symptoms of lacrimal gland carcinoma?

-displaced eye

-double vision

-eye pain

What are signs of lacrimal gland carcinoma?

-globe displacement inferiorly and medially

-restricted EOM

-eyelid edema and erythema

-palpable lacrimal gland mass

What are complications of lacrimal gland carcinoma?

intracranial extension

How is lacrimal gland carcinoma managed?

-orbital CT or MRI

-refer out (complete surgical excision)

-radiotherapy and chemotherapy

What are pearls of lacrimal gland carcinoma?

-onset of pain is rapid (over 1-3 months)

-five year survival rate is 47% and at 15 years its 22%

-major cause of death is intracranial extension

What is orbital lymphoma?

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

What is the etiology of orbital lymphoma?

proliferation of lymphocyte

What demographics are affected by orbital lymphoma?

typically occurs between the ages of 50-70 years

What is the laterality of orbital lymphoma?

unilateral>bilateral

What are symptoms of orbital lymphoma?

-bulging/displaced eye

-double vision

-droopy eyelid

-eyelid swelling

-eye pain in a minority of cases

What are signs of orbital lymphoma?

-proptosis

-globe displacement

-restricted EOM with possible pain on eye movement

-ptosis

-palpable mass if anterior

-may have pink salmon patch area of sub conjunctival extension

What are complications associated with orbital lymphoma?

-compression on the globe and/or optic nerve

-metastasis

How is orbital lymphoma managed?

-orbital CT or MRI

-refer out (biopsy, radiotherapy, chemotherapy)

What are pearls associated with orbital lymphoma?

-60% chance of developing systemic lymphoma within 5 years

-lymphoma represents one end of the spectrum of lymphoproliferative lesions

What is orbital rhabdomyosarcoma?

malignant tumor of the orbit

What is the etiology of orbital rhabdomyosarcoma?

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

What demographics are most affected by orbital rhabdomyosarcoma?

infancy to adulthood (70% occurs between ages 8-10)

What is the laterality of orbital rhabdomyosarcoma?

unilateral

What are symptoms of orbital rhabdomyosarcoma?

-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

What are signs of orbital rhabdomyosarcoma?

-proptosis

-globe displacement

-restricted EOM with possible pain on movement

-ptosis

-eyelid edema and erythema

-conjunctival injection and chemosis

What are complications associated with orbital rhabdomyosarcoma?

-compression on the globe and/or optic nerve

-erosion of orbital bones

-metastasis

How is orbital rhabdomyosarcoma managed?

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

What is the most common malignant orbital tumor in children?

orbital rhabdomyosarcoma

What is the most common location of orbital rhabdomyosarcoma?

superior nasal orbit

What are the hallmarks of orbital rhabdomyosarcoma?

rapid onset and progression

What has similar presentations as orbital rhabdomyosarcoma?

metastatic neuroblastoma (second most common orbital malignancy)

What is the most common pediatric orbital metastatic tumor?

metastatic neuroblastoma

Where does metastatic neuroblastoma arise from?

primary tumor in abdomen, mediastinum, or neck

What do patients with metastatic neuroblastoma present with?

sudden proptosis with eyelid ecchymosis that may be bilateral (may develop ipsilateral horners syndrom)

prognosis is poor*