Mass Effect/Tumors Flashcards
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
common primary benign tumors of orbit in chidren
Infantile (Capillary) Hemangioma
Infantile (Capillary) Hemangioma
lesions may be present _____ or appear in ________ of life
at birth; first few weeks of life
enlarge dramatically over the first 6-12 months of life & begin to involute after first year of life.
Infantile (Capillary) Hemangioma
Main ocular complications of Infantile Hemangioma
Amblyopia
Strabismus
Anisometropia
True/False
Treatment may be deferred until it is clear that natural course of lesion will not lead to desired result
TRUE
women are affected more often than men
Cavernous Hemangioma
Infantile (Capillary) Hemangioma
First line of management:
Observation
Refractive correction
Amblyopia therapy
Cavernous hemangioma
Principal findings
(SRHOIS)
-Slowly progressive proptosis
-Retinal striae
-Hyperopia
-Optic nerve compression
-Increased intraocular pressure (IOP)
-Strabismus
most common benign neoplasm of orbit in adults
Cavernous Hemangioma
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
Diagnostic: ________ important in determining position of cavernous hemangioma relative to the optic nerve
Coronal imaging
uncommon, encapsulated, hypervascular, hypercellular lesions that appear in midlife (adult)
Hemangiopericytoma
resembles cavernous hemangiomas on both computed tomography (CT) & MRI, but appear __________ intraoperatively
bluish
composed of: ____ that surround a rich capillary network
Plump pericytes (fat cells)
Hemangiopericytome treatment
Complete excision
uncommon, usually benign tumors that occur predominantly in children in first decade of life
Optic Nerve Glioma
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)
Optic nerve glioma
Intracranial involvement may be associated with
IDD
-Intracranial hypertension
-Decreased function of hypothalamus
-Decreased function of pituitary gland
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)
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
Optic nerve glioma
Treatment: c. Chemotherapy
-Combination therapy
- Actinomycin D
- Vincristine
- Etoposide
Effective in patients with progressive chiasmal hypothalamic gliomas
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
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
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
- CT
- MRI scan
- PET scan (indication of whether tumor is benign)
- 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:
- Location of tumor
- Size of tumor
- 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
- Neurofibromas
- Cafe-Au LAit Spots
- Freckling
- 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
- Plexiform neurofibromas involving lateral aspect of upper eyelid
- S-shaped contour of eyelid margin
- Pulsating proptosis secondary to sphenoid bone dysplasia
- Optic nerve glioma
NF1 Management
ECRR
- Excision or resection of tumors
- Chemotherapy
- Radiotherapy
- 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
- Temporal fossa mass
- Proptosis
- Eyelid edema
- 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
Resection of tumor (through combined approach to intracranial & orbital component)
Meningiomas treatment
Observation: (in px w/ VA of 20/50 or better)
BVC
- Biannual neuroimaging & ophthalmologic examinations
- Visual field testing
- 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
- To relieve pressure in the orbit & around the optic nerves
- Improve pain management
- 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
- Firm, lobular mass (palpated near the superolateral orbital rim)
- 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
- Complete resection of tumor via surgery [recurrence rate is 75% (5-10 year post surgery)]
- Postoperative radiation therapy (for those with poor prognosis)
- Long-term follow-up monitoring
