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Ocular Disease (Orbit) > Mass Effect/Tumors > Flashcards

Mass Effect/Tumors Flashcards

1
Q

11 Mass Effect/Tumors

(ICHONNMREPA)

A
  1. Infantie (Capillary) Hemangioma
  2. Cavernous Hemangioma
  3. Hemangiopericytoma
  4. Optic Nerve Glioma
  5. Neurofibroma
  6. Neurofibromatosis 1 (NF1)
  7. Meningiomas
  8. Rhabdomyosarcoma
  9. Eirdheim-Chester Disease (ECD)
  10. Pleomorphic Adenoma
  11. Adenoid Cystic Carcinoma
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2
Q

common primary benign tumors of orbit in chidren

A

Infantile (Capillary) Hemangioma

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3
Q

Infantile (Capillary) Hemangioma

lesions may be present _____ or appear in ________ of life

A

at birth; first few weeks of life

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4
Q

enlarge dramatically over the first 6-12 months of life & begin to involute after first year of life.

A

Infantile (Capillary) Hemangioma

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5
Q

Main ocular complications of Infantile Hemangioma

A

Amblyopia
Strabismus
Anisometropia

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6
Q

True/False

Treatment may be deferred until it is clear that natural course of lesion will not lead to desired result

A

TRUE

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7
Q

women are affected more often than men

A

Cavernous Hemangioma

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7
Q

Infantile (Capillary) Hemangioma

First line of management:

A

Observation
Refractive correction
Amblyopia therapy

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8
Q

Cavernous hemangioma

Principal findings

(SRHOIS)

A

-Slowly progressive proptosis
-Retinal striae
-Hyperopia
-Optic nerve compression
-Increased intraocular pressure (IOP)
-Strabismus

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8
Q

most common benign neoplasm of orbit in adults

A

Cavernous Hemangioma

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8
Q

Cavernous hemangioma

Treatment

(IPS)

A

Surgical excision
- If lesion compromises ocular function
- Proptosis
- Significant growth

Note: The surgical approach is dictated by location of the lesion

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8
Q

Diagnostic: ________ important in determining position of cavernous hemangioma relative to the optic nerve

A

Coronal imaging

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9
Q

uncommon, encapsulated, hypervascular, hypercellular lesions that appear in midlife (adult)

A

Hemangiopericytoma

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10
Q

resembles cavernous hemangiomas on both computed tomography (CT) & MRI, but appear __________ intraoperatively

A

bluish

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11
Q

composed of: ____ that surround a rich capillary network

A

Plump pericytes (fat cells)

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12
Q

Hemangiopericytome treatment

A

Complete excision

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13
Q

uncommon, usually benign tumors that occur predominantly in children in first decade of life

A

Optic Nerve Glioma

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14
Q

Optic Nerve Glioma

Chief clinical features:

(GVOONSR)

A

-Gradual, painless, unilateral axial proptosis
-Vision loss
-Optic atrophy
-Optic disc swelling
-Nystagmus
-Strabismus
-Relative Afferent Pupillary Defect (RAPD)

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15
Q

Optic nerve glioma

Intracranial involvement may be associated with

IDD

A

-Intracranial hypertension
-Decreased function of hypothalamus
-Decreased function of pituitary gland

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16
Q

Optic nerve glioma

Treatment: a. Observation only

(WIF)

A

-With good vision on the involved side
-If glioma is confined to the orbit
-Follow-up examinations & appropriate radiographic studies (MRI)

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17
Q

Optic nerve glioma

Treatment: b. Surgical excision

(IPPC)

A

-In an effort to isolate the tumor from optic chiasm & thus prevent chiasmal invasion
-Prevent proptosis
-Prevent corneal exposure
-Cosmetic purposes

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17
Q

Optic nerve glioma

Treatment: c. Chemotherapy

A

-Combination therapy
- Actinomycin D
- Vincristine
- Etoposide

Effective in patients with progressive chiasmal hypothalamic gliomas

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17
Q

Optic Nerve Glioma

Chemotherapy: Advantage

(MEP)

A
  1. May delay the need for radiation therapy
  2. Enhance long-term intellectual development in children
  3. Preservation of endocrine function in children
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17
Q

Optic Nerve Glioma

Treatment: Radiation therapy considered if:

TSC

A
  1. If tumor cannot be resected (usually chiasmal or optic tract lesions)
  2. If symptoms progress after chemotherapy
  3. If chiasmal & optic tract involvement is extensive
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17
Q

tumors composed chiefly of proliferating Schwan cells within the nerve sheaths

A

Nerurofibroma

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17
Q

benign nerve tumor that tends to form ____ within the nerve

A

centrally

Neurofibroma

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18
Q

happens when arises from several nerve bundles

A

Plexiform neurfibroma

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18
Q

Neurofibroma symptoms

A

Pain
Numbness

If the tumor presses against nerves

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18
Q

Neurofibroma may appear in people with a genetic condition called

A

Neurofibromatosis type 1

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19
Q

Neurofibroma is often often found in people ages ______

A

20 to 40 years old

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20
Q

Neurofibroma

Diagnosis: Imaging studies can help ____ where tumor is, find ________, & identify what ____

A

help pinpoint;
find very small tumors;
identify what tissues are affected or nearby

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21
Q

Imaging studies in diagnosis of Neurofibroma

A
  1. CT
  2. MRI scan
  3. PET scan (indication of whether tumor is benign)
  4. Biopsy (diagnose mass as being a neurofibroma)
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21
Q

Neurofibroma

Treatment/Management:

A

Monitoring & surgery

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21
Q

Neurofibroma

Treatment: Monitoring

A
  • Observation of tumor **if its in a place that makes removal difficult ** or if it is small & causes no problems
  • Observation includes regular checkups & imaging tests to see if tumor is growing
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22
Q

Neurofibroma

Treatment: Surgery to remove tumor

A
  • to relieve symptoms pressing on nearby tissue or damaging organs

Surgery may be considered if:
1. Symptoms
2. Cosmetic reasons

23
Q

Neurofibroma Treatment

Type of surgery depends on:

A
  1. Location of tumor
  2. Size of tumor
  3. Whether its intertwined with more than one nerve
24
Q

inherited through an autosomal dominant gene with incomplete penetrance

A

Neurofibromatosis 1 (NF1)

25
Q

Neurofibromatosis 1 is also known as

A

von Recklinghausen disease

26
Q

symptom of NF1: hyperpigmented macules or patches seen in various regions in the body

A

Cafe-Au Lait Spots

26
Q

group of congenital disorders that impact organs which arise from the ectoderm

A

Neurofribromatosis 1 (NF1)

26
Q

NF1

Signs & Symptoms

A
  1. Neurofibromas
  2. Cafe-Au LAit Spots
  3. Freckling
  4. Lisch Nodules
26
Q

a symptom of NF1 where in benign peripheral nerve sheath tumors that are soft, painless nodules that arise on or underneath the skin

A

Neurofibromas

26
Q

Areas affected by NF1

A

Central Nervous System
Skin
Eyes

26
Q

symptom of NF1: hyperpigmented areas smaller in size than Cafe-Au Lait spots most prominent in axillary & inguinal regions

A

Freckling

26
Q

symptom of NF1: pigmented iris hamartomas

A

Lisch nodules

26
Q

Neurofibromatosis 1

Significant orbital features

PSPO

A
  1. Plexiform neurofibromas involving lateral aspect of upper eyelid
  2. S-shaped contour of eyelid margin
  3. Pulsating proptosis secondary to sphenoid bone dysplasia
  4. Optic nerve glioma
27
Q

NF1 Management

ECRR

A
  1. Excision or resection of tumors
  2. Chemotherapy
  3. Radiotherapy
  4. Removal of cataracts
27
Q

neoplasms that arise from arachnoid villi

A

Meningiomas

28
Q

originate intracranially along sphenoid wing

A

Meningiomas

29
Q

arise primarily in the orbital portion of optic nerve sheath

A

Meningiomas

30
Q

Meningiomas

Secondary extension

ISO

A
  • Into the orbit through the bone
  • Superior orbital fissure
  • Optic canal
31
Q

Meningiomas

Ophthalmic manifestations related to location of primary tumor

MTSMP

A

a. Meningiomas arising near optic nerve
b. Tumors arising near the “pterion”
c. Sphenoid wing meningiomas
d. Malignant meningioma
e. Primary orbital meningioma

31
Q

Meningiomas ophthalmic manifestations

cause early visual field defects & papilledema or optic atrophy

A

Meningiomas arising near optic nerve

31
Q

Meningiomas ophthalic manifestations

posterior end of parieto sphenoid fissure, at the lateral portion of sphenoid bone

A

Tumors arising near “pterion”

32
Q

Meningiomas

Tumors arising near pterion causes

TPEC

A
  1. Temporal fossa mass
  2. Proptosis
  3. Eyelid edema
  4. Chemosis
33
Q

Meningioma ophthalmic manifestation

rare & results in rapid tumor growth that is not responsive to surgical resection, radiotherapy, or chemotherapy

A

Malignant meningioma

33
Q

produce hyperotosis of the involved bone & hyperplasia of associated soft tissues

A

Sphenoid wing meningiomas

33
Q

Meningiomas ophthalmic manifestations

originate in the arachnoid of the optic nerve sheath

A

Primary orbital meningiomas

33
Q

Meningiomas treatment/management

A

Observation
Surgery
Radiation therapy

33
Q

Primary orbital meningiomas symptoms

GAEO

A

-Gradual, painless, unilateral loss of vision
-Afferent pupillary defect
-Efferent pupillary defect
-Optic nerve head may appear normal, atrophic, or swollen

34
Q

Meningiomas treatment

Surgery: goals of surgery in orbital meningioma includes:

A

Long term tumor control,
Recovery of optic nerve & cranial nerve function, &
Cosmesis

Resection of tumor (through combined approach to intracranial & orbital component)

34
Q

Meningiomas treatment

Observation: (in px w/ VA of 20/50 or better)

BVC

A
  1. Biannual neuroimaging & ophthalmologic examinations
  2. Visual field testing
  3. Color plates
34
Q

Meningioma treatment

should only be considered prior to surgery

A

Radiation therapy

35
Q

most common primary orbital malignancy of childhood

A

Rhabdomyosarcoma

36
Q

Rhabdomyosarcoma

the average age of onset is ____

A

5-7 years

37
Q

Signs & symptoms of Rhabdomyosarcoma

ADPS

A
  • Adnexal response with edema
  • Discoloration of eyelids
  • Ptosis
  • Strabismus may be present
38
Q

Primary treatment for Rhabdomyosarcoma

A

Radiation therapy & Systemic chemotherapy

38
Q

most devastating of the adult xanthogranulomas

A

Erdheim-Chester Disease (ECD)

38
Q

ECD

____ of cases demonstrating ocular involvement

A

22%

39
Q

53 years old with male predominance

A

Eirdheim-Chester Disease (ECD)

40
Q

characterized by dense, progressive, recalcitrant fibrosclerosis of the orbit

A

Eirdheim-Chester Disease (ECD)

40
Q

True/False

There is no cure for ECD. Complete surgical excision is not possible due to the infiltrative nature of the disease

A

TRUE

40
Q

ECD Medical Management

CCI

A

Corticosteroids
Chemotherapeutic agents
Immunomodulators

40
Q

ECD Surgical Management

A

-Mechanical debulking of intraorbital masses
-Orbital decompression

40
Q

Goal: Not curative

TIP

A
  1. To relieve pressure in the orbit & around the optic nerves
  2. Improve pain management
  3. Prevent progressive vision loss from compressive optic neuropathy
41
Q

most common epithelial tumor of the lacrimal gland

A

Pleomorphic Adenona

42
Q

this tumor usually occurs in adults during 4th or 5th decade of life, affects slightly more men than women

A

Pleomorphic Adenoma

42
Q

Patients present with a progressive, painless downward & inward displacement of the glove with axial proptosis

A

Pleomorphic Adenoma

42
Q

Pleomorphic Adenoma is also known as

A

Benign Mixed Tumor

42
Q

Symptoms (usuaslly present for more than 12 months) of Pleomorphic Adenoma

A
  1. Firm, lobular mass (palpated near the superolateral orbital rim)
  2. Enlargement or expansion of lacrimal fossa (via orbital imaging)
43
Q

Treatment for Pleomorphic Adenoma

A

Compelete removal of tumor with its pseudocapsule & a surrounding margin of orbital tissue

44
Q

most comon malignant tumor of the lacrimal gland

A

Adenoid Cystic Carcinoma

44
Q

Adenoid Cystic Carcinoma is also known as

A

Cylindroma

45
Q

may cause pain because of perineural invasion & bone destruction

A

Adenoid cystic carcinoma

46
Q

Adenoid Cystic Carcinoma

Differential diagnosis from “Pleomorphic Adenoma/BMT”

A
  • Relatively rapid course (generally less than 1 year)
  • Early onset of pain
47
Q

Adenoid Cystic Carcinoma

Treatment/Management

A
  1. Complete resection of tumor via surgery [recurrence rate is 75% (5-10 year post surgery)]
  2. Postoperative radiation therapy (for those with poor prognosis)
  3. Long-term follow-up monitoring

Ocular Disease (Orbit) (2 decks)

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