Mass Effect/Tumors Flashcards by Ashley Mendova

11 Mass Effect/Tumors

(ICHONNMREPA)

Infantie (Capillary) Hemangioma
Cavernous Hemangioma
Hemangiopericytoma
Optic Nerve Glioma
Neurofibroma
Neurofibromatosis 1 (NF1)
Meningiomas
Rhabdomyosarcoma
Eirdheim-Chester Disease (ECD)
Pleomorphic Adenoma
Adenoid Cystic Carcinoma

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common primary benign tumors of orbit in chidren

Infantile (Capillary) Hemangioma

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Infantile (Capillary) Hemangioma

lesions may be present _____ or appear in ________ of life

at birth; first few weeks of life

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

Infantile (Capillary) Hemangioma

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Main ocular complications of Infantile Hemangioma

Amblyopia
Strabismus
Anisometropia

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True/False

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

TRUE

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women are affected more often than men

Cavernous Hemangioma

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Infantile (Capillary) Hemangioma

First line of management:

Observation
Refractive correction
Amblyopia therapy

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Cavernous hemangioma

Principal findings

(SRHOIS)

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

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most common benign neoplasm of orbit in adults

Cavernous Hemangioma

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Cavernous hemangioma

Treatment

(IPS)

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

Coronal imaging

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

Hemangiopericytoma

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

bluish

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composed of: ____ that surround a rich capillary network

Plump pericytes (fat cells)

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Hemangiopericytome treatment

Complete excision

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

Optic Nerve Glioma

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Optic Nerve Glioma

Chief clinical features:

(GVOONSR)

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

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Optic nerve glioma

Intracranial involvement may be associated with

IDD

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

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Optic nerve glioma

Treatment: a. Observation only

(WIF)

-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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Optic nerve glioma

Treatment: b. Surgical excision

(IPPC)

-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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Optic nerve glioma

Treatment: c. Chemotherapy

-Combination therapy
- Actinomycin D
- Vincristine
- Etoposide

Effective in patients with progressive chiasmal hypothalamic gliomas

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Optic Nerve Glioma

Chemotherapy: Advantage

(MEP)

May delay the need for radiation therapy
Enhance long-term intellectual development in children
Preservation of endocrine function in children

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Optic Nerve Glioma

Treatment: Radiation therapy considered if:

TSC

If tumor cannot be resected (usually chiasmal or optic tract lesions)
If symptoms progress after chemotherapy
If chiasmal & optic tract involvement is extensive

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tumors composed chiefly of proliferating Schwan cells within the nerve sheaths

Nerurofibroma

benign nerve tumor that tends to form ____ within the nerve

centrally | Neurofibroma

happens when arises from several nerve bundles

Plexiform neurfibroma

Neurofibroma symptoms

Pain Numbness | If the tumor presses against nerves

Neurofibroma may appear in people with a genetic condition called

Neurofibromatosis type 1

Neurofibroma is often often found in people ages ______

20 to 40 years old

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

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

Imaging studies in diagnosis of Neurofibroma

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

# Neurofibroma Treatment/Management:

Monitoring & surgery

# Neurofibroma Treatment: Monitoring

- 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

# Neurofibroma Treatment: Surgery to remove tumor

- to relieve symptoms pressing on nearby tissue or damaging organs Surgery may be considered if: 1. Symptoms 2. Cosmetic reasons

# Neurofibroma Treatment Type of surgery depends on:

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

inherited through an autosomal dominant gene with incomplete penetrance

Neurofibromatosis 1 (NF1)

Neurofibromatosis 1 is also known as

von Recklinghausen disease

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

Cafe-Au Lait Spots

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

Neurofribromatosis 1 (NF1)

# NF1 Signs & Symptoms

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

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

Neurofibromas

Areas affected by NF1

Central Nervous System Skin Eyes

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

Freckling

symptom of NF1: pigmented iris hamartomas

Lisch nodules

# Neurofibromatosis 1 Significant orbital features | PSPO

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

NF1 Management | ECRR

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

neoplasms that arise from arachnoid villi

Meningiomas

originate intracranially along sphenoid wing

Meningiomas

arise primarily in the orbital portion of optic nerve sheath

Meningiomas

# Meningiomas Secondary extension | ISO

- Into the orbit through the bone - Superior orbital fissure - Optic canal

# Meningiomas Ophthalmic manifestations related to location of primary tumor | MTSMP

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

# Meningiomas ophthalmic manifestations cause early visual field defects & papilledema or optic atrophy

Meningiomas arising near optic nerve

# Meningiomas ophthalic manifestations posterior end of parieto sphenoid fissure, at the lateral portion of sphenoid bone

Tumors arising near "pterion"

# Meningiomas Tumors arising near pterion causes | TPEC

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

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

Malignant meningioma

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

Sphenoid wing meningiomas

# Meningiomas ophthalmic manifestations originate in the arachnoid of the optic nerve sheath

Primary orbital meningiomas

Meningiomas treatment/management

Observation Surgery Radiation therapy

Primary orbital meningiomas symptoms | GAEO

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

# Meningiomas treatment Surgery: goals of surgery in orbital meningioma includes:

Long term tumor control, Recovery of optic nerve & cranial nerve function, & Cosmesis ## Footnote Resection of tumor (through combined approach to intracranial & orbital component)

# Meningiomas treatment Observation: (in px w/ VA of 20/50 or better) | BVC

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

# Meningioma treatment should only be considered prior to surgery

Radiation therapy

most common primary orbital malignancy of childhood

Rhabdomyosarcoma

# Rhabdomyosarcoma the average age of onset is ____

5-7 years

Signs & symptoms of Rhabdomyosarcoma | ADPS

- Adnexal response with edema - Discoloration of eyelids - Ptosis - Strabismus may be present

Primary treatment for Rhabdomyosarcoma

Radiation therapy & Systemic chemotherapy

most devastating of the adult xanthogranulomas

Erdheim-Chester Disease (ECD)

# ECD ____ of cases demonstrating ocular involvement

22%

53 years old with male predominance

Eirdheim-Chester Disease (ECD)

characterized by dense, progressive, recalcitrant fibrosclerosis of the orbit

Eirdheim-Chester Disease (ECD)

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

TRUE

ECD Medical Management | CCI

Corticosteroids Chemotherapeutic agents Immunomodulators

ECD Surgical Management

-Mechanical debulking of intraorbital masses -Orbital decompression

Goal: Not curative | TIP

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

most common epithelial tumor of the lacrimal gland

Pleomorphic Adenona

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

Pleomorphic Adenoma

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

Pleomorphic Adenoma

Pleomorphic Adenoma is also known as

Benign Mixed Tumor

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

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

Treatment for Pleomorphic Adenoma

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

most comon malignant tumor of the lacrimal gland

Adenoid Cystic Carcinoma

Adenoid Cystic Carcinoma is also known as

Cylindroma

may cause pain because of perineural invasion & bone destruction

Adenoid cystic carcinoma

# Adenoid Cystic Carcinoma Differential diagnosis from "Pleomorphic Adenoma/BMT"

- Relatively rapid course (generally less than 1 year) - Early onset of pain

# Adenoid Cystic Carcinoma Treatment/Management

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

Mass Effect/Tumors Flashcards

(86 cards)