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TORIC > Oncologie > Flashcards

Oncologie Flashcards

1
Q

Comment fonctionne la chimiothx?

A

Disruption of DNA synthesis at S phase (carboplatin, etoposide, 5-FU)
Inhibition of Mitosis at M phase (vincrisitine)

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2
Q

Mécanismes et types de radiothérapie

A

Mécanismes
- Direct damage of tumour DNA
- Indirect damage of DNA through ionization of H2O –> free radicals

Types :
- Teletherapy
- Brachytherapy

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3
Q

Ddx des eyelid epidermal tumours

A

Benign epidermal tumours
- Squamous Papilloma
- Seborrheic Keratosis
- Keratacanthoma

Malignant Epidermal Tumours
- Basal Cell Carcinoma
- Squamous Cell Carcinoma

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4
Q

Caractéristiques du BCC (types de morphologie, FdR, localisation)

A

Morphological types :
- Nodular
- Ulcerative
- Pigmented
- Morpheaform
- Rarement : Cystic or Multicenteric

FdR : high solar exposure

Localisation :
- Lower eyelid majoritairement
- Medial canthus (25%) : deep invasion, lacrimal obstruction, recurrence

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5
Q

2 types de Eyelid Sebaceous Tumors

A

Sebaceous adenoma
Sebaceous gland carcinoma

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6
Q

Caractéristiques du Sebaceous Adenoma

A

Lobulated mass
Commonly of Meibomiam origin
Carries NO malignant potential

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7
Q

Caractéristiques d’un Sebaceous Gland Carcinoma

A

Meibomian, Zeis or carancle origin
Circumscribed mass (mime un chalazion) or Diffuse
Intraepithelial spread (chronic conjunctivitis unilatérale)

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8
Q

3 types de eyelid neural tumors

A

Neurofibroma
Schwannoma
Merkel cell tumor

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9
Q

Caractéristiques du neurofibrome (¢ prolifératives, types)

A

Neurofibroma : Proliferation of axons, Schwann cells & endoneural fibroblasts

Types :
- Plexiform Neufibroma : Pathognomonic NF1, trends to recur
- Multiple Neurofibromata : Mostly associated with NF1
- Solitary Neurofibroma : Not associated with NF1

Signes NF1 : Lisch nodules, café au lat Patches

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10
Q

Ddx Eyelid Vascular Tumors

A

Congenital capillary hemangioma
Acquired capillary hemangioma
Cavernous hemangioma
Lymphangioma
Diffuse Angioma (Nevus Fammeus)
Vascular malformations
Hemangioendothelioma

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11
Q

Congenital Capillary Hemangioma : types, évolution et Tx

A

Types :
- Cutaneous (le plus fréquent)
- Subcutaneous
- Diffuse (rare)
- Orbital

Évolution :
- Involution 50% by fifth year, 70% by seventh year

Tx :
- PROPANOLOL
- Steroids
- Surgical
- Interferon

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12
Q

À quelle malformation vasculaire est associée le Sturge-Weber?

A

Diffuse Angioma (Nevus Fammeus)

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13
Q

Types de Bx pour une lésion diffuse eyelid (x2)

A

Incision Bx
Punch Bx
(Include adjacent normal tissu in the Bx)

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14
Q

Types de Bx pour une lésion circonscrite eyelid (x2)

A

Excision Bx
Shave Bx

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15
Q

Non-surgical Tx of Eyelid Tumours

A

Radiothérapie
Cryothérapie (for small lesion, may cause skin depigmentation)
Steroids (intra-lesional, systemic for capillary hemangioma)
Interferon alpha-2
Propanolol
Sclerosing agents
Chemotherapy
Immunotherapy (Imiquod 5% cream for BCC, BRAF inhibitors in melanoma)

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16
Q

Cible de l’immunothérapie utilisé pour certains mélanomes

A

BRAF inhibitors

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17
Q

Définition, indication et complications de l’orthovoltage X-Ray Radiotherapy

A

Orthovoltage : Type of radiation therapy
- X-rays used are strong enough to kill cancer cells but do not penetrate more than few mm beyond the surface of the skin
- Effective treatment for very superficial, small tumours (ex. skin cancer)

Indication : Malignant eyelid tumours ≤ 10 mm depth

Provides 95% tumour control

Complications :
- Loss of lashes
- Skin telangiectasia
- Dry eye/Epiphora

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18
Q

Most common ocular surface neoplasia

A

Epithelial tumors :
- Squamous Papilloma
- Conjunctival Intraepithelial Neoplasia (CIN)
- Squamous cell carcinoma

Melanocytic tumors :
- Nevus
- Melanosis (racial, PAM, subepithelial)
- Melanoma

Lymphoproliferative tumors

Vascular : lymphangioma, hemangioma

Others : pyogenic granuloma, dermoid…

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19
Q

Caractéristiques et Tx Conjunctival Intraepithelial Neoplasia (CIN)

A

Flat/Sessile mass
Translucent/gray/fleshy
Starts at the limbus → empiète sur la cornée
NO basement membrane invasion → NO metastasis
Tx :
- Surgical excision
- Interferon
- MMC (topical mitomycin C)
- 5-FU eyedrops

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20
Q

Caractéristique et Tx Squamous Cell Carcinoma

A

THE MOST common conjunctival malignancy
CIN → invades basement membrane
Arise de novo, at the limbus or fornix
Examine for local lymph nodes (if suspicious → MRI)
Tx :
- Excision + margin cryotherapy +/- PO local chemo
- If scleral invasion on UDM → Brachytherapy

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21
Q

Quel est the most common benign conjunctival tumour?

A

Nevus

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22
Q

Quel est la caractéristique typique des Nevus conjonctivaux?

A

Clear cyst are characteristic

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23
Q

Caractéristiques d’un Nevus a/n conjonctive

A

The most common benign conjunctival tumour
Early adulthood
Bulbar conjunctiva, Plica or Caruncle, very rarely Palpebral
Color : melanotic to amelanotic
Clear cyst are characteristic
Tx : periodic observation with photographs, or excision

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24
Q

2 types de melanosis

A

Complexion (racial)
Primary Acquired Melanosis (PAM)

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25
Q

Caractéristiques d’un complexion (racial) melanosis

A

Dark-skinned races
Inter-palpebral on bulbar conductive and around limbus
Malignant transformation is exceedingly RARE
NO Tx is needed

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26
Q

Caractéristiques d’un Primary Acquired Melanosis (PAM)

A

Fair-skinned
Involved any area of the conjunctiva
PAM with severe atypia → significant nuclear change → 35% develop melanoma

Tx :
- Clinically mild : observation
- Suspicious : Bx + cryothx
- Extensive : topical anti-mitotic + cryothx + excise residue

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27
Q

Mélanome de la conjonctive (origine, localisation, caractéristiques à l’E/P, sites de métastases, Tx)

A

Arise de novo, from PAM or very rarely a nevus (<7%)

Any area of the conjunctiva

Fleshy elevated, with feeder vessels

Early metastasis : lymph nodes, brain, other sites

Tx :
- Localized : excision + brachytherapy
- Extensive : exenteration

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28
Q

Signe caractéristique d’un tumeur lymphoproliférative (ocular surface neoplasia)

A

Salmon patch (all grades)

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29
Q

Type de ¢ le plus souvent impliqué dans tumeur lymphoproliférative (ocular surface neoplasia)

A

Lymphoma is mostly non-Hodgkin’s SMALL B-cell type

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30
Q

Caractéristiques de lymphome (ocular surface neoplasia)

A

Spectrum : low grade (ex. MALT) ad high grade (ex. Mantle cell)

Clinically : Salmon patch (all grades)

Lymphoma is mostly non-Hodgkin’s SMALL B-cell type

Treatment :
- Localized : excision bx + systemic staging
- Diffuse : incision bx + systemic stating

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31
Q

Ix pour le staging du lymphoma (ocular surface neoplasia) et CAT

A

Lymphoproliferative Systemic Staging
- MRI of head and neck
- Bx and pathological grading
- Whole body CT scans
- Referral to radiation/medical oncologist

Tx :
- Radiotherapy (20-40Gy)
- Chemotherapy
- Interferon
- Rituximab (Anti-CD 20 antibodies)

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32
Q

Caractéristiques d’un lymphangiome

A

Vascular Tumor

Multiple small cysts filled with clear fluid and/or blood

Eyelid involvement is mostly associated with conjunctival lymphangioma

Episodic swelling due to bouts of hemorrhage (crises d’hémorragie) and/or local infections

Associated with buccal or labial lymphangioma

NO involution with age

CAT :
- Poor response to steroids
- Observation
- CO2 laser-aided debulking
- Complete excision in selected cases
- For deeper, extensive lymphangiomas : intralesional sclerosing agents

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33
Q

2 type of Treatment for Conjunctival Tumors

A

SURGICAL excision

TOPICAL treatment for Conjunctival

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34
Q

SURGICAL excision of Conjunctival Tumour

A

SURGICAL excision
- Alcool (absolute 70%) corneal epitheliectomy
- Total tumour excision from cornea and conjunctiva
- Cryotherapy of the conjunctival margins

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35
Q

TOPICAL treatment of Conjunctival Tumour (Rx, indications et complications)

A

TOPICAL treatment for Conjunctival Tumors
- Mitomycin-C (MMC) 0,02-0,004%
- 5-Flourouracil 1% (5-FU)
- Interferon alpha 2a

Indications :
- CIN; superficial SCC = Interferon
- PAM = MMC
- Intra-epithelial spread of Sebaceous Cell carcinoma

Complications :
- MMC = Severe epithelial toxicity
- Lacrimal drainage scarring
- Resistance/Recurrence

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36
Q

Clinical Manifestations of Orbital Tumors (Les Ps)

A

Proptosis : direction, constancy, positional change
Pain : with inflammation, malignancy
Progression
Palpation : orbital rim, lacrima fossa
Pulsations : vascular fistula, NF1, meningocele
Peri-orbital changes : salmon patches, ecchymosis

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37
Q

Clinical classification of Orbital Tumours

A

Classification à partir du CT scan
- CT scan = gold standard

Lacrimal gland mass
Circumscribed orbital mass
Diffuse orbital mass
Orbital cysts
Optic nerve mass
Paediatric orbital mass

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38
Q

Ddx d’une lacrimal gland mass

A

Lymphoproliferative/chronic inflammation
Acute dacryoadenitis
Pleomorphic adenoma (le plus fréquent)
Adenoid cystic carnoma

39
Q

Caractéristiques du lymphoproliferative/chronic inflammation (lacrimal gland mass)

A

+/- bilateral
Painless
Moulds to orbital structures

40
Q

Caractéristiques de l’Acute dacryoadenitis (lacrimal gland mass)

A

Acute dacryoadenitis
+/- bilateral
Rapid course
Painful
Oblong swelling

41
Q

Caractéristiques du Pleomorphic adenoma (lacrimal gland mass)

A

Pleomorphic adenoma = le plus fréquent
- Unilateral
- Slow growth
- Spherical mass
- Indents bone (ne cause pas d’érosion des os)

42
Q

Caractéristiques de l’Adenoid cystic carnoma (lacrimal gland mass)

A

Unilateral
Rapid growth
Painful
Irregular mass
+/- bone erosion

43
Q

Ddx circumscribed orbital mass

A

Cavernous hemangioma
Haemangiopericytoma
Neurofibrom
Schwannoma/Neurilemmona
Fibrous histiocytoma
Rare : solitary fibrous tumor, thromboses varix, ectopic meningioma

44
Q

Caractéristiques Cavernous hemangioma

A

Most commun
Very slow growth
Progression per-grossesse

45
Q

Caractéristiques Haemangiopericytoma

A

Injection conjonctive
Récurrence
Malignancy

46
Q

Caractéristiques Neurofibrom

A

Solitary
Can be multiple or extensive in NF1

47
Q

Caractéristiques du Schwannoma/Neurilemmona

A

Along a peripheral nerve
Antoni A&B
1% malignant

48
Q

Caractéristiques Fibrous histiocytoma

A

Superonasal quadrant
Middle age
Benign or malignant

49
Q

Ddx diffuse orbital mass in Adult

A

Lymphoproliferative/leukemia
Metastasis
Idiopathic/Granulomatous inflammation
Cellulitis/Subperiosteal abcess

50
Q

Caractéristiques Lymphoproliferative/leukemia

A

+/- Bilateral
Painless
Slow

51
Q

Caractéristiques Metastasis

A

+/- history of cancer
Painless
Rapid
Unilateral

52
Q

Caractéristiques Idiopathic/Granulomatous inflammation

A

Rapid
Painful
Recurrent
Responsive to steroids

53
Q

Caractéristiques Cellulitis/Subperiosteal abcess

A

Rapid
Painful
Fever
Motility limitation
Sinusitis

54
Q

Ddx des Orbital cysts

A

Dermoid cyst/Teratoma
Epithelial cyst
Parasitic cyst
Hemorrhagic cyst
Sinus Mucocele
Meningocele/Encephalocele
Colobomatous cyst
Cyst inside a solid tumour

(Fluide = très bright à l’IRM)

55
Q

Ddx des optic nerves tumours

A

Optic nerve sheath meningioma
Optic nerve glioma
Secondary (ex. RB)

Les 2 premiers sont les plus fréquents

56
Q

Caractéristiques du Optic nerve sheath meningioma

A

Middle aged female
Minimal proptosis
Gaze evoked amaurosis
Central scotoma
Disc swelling or atrophy
Retino-choroidal shunts on the disc
Tram track sign : calcification au CT

57
Q

Qu’est que le Tram track sign et à quelle pathologie est-il associé?

A

Tram track sign : calcification in CT scan
Optic nerve sheath meningioma

(Tram track sign: parallel thickening and enhancement around the optic nerve)

58
Q

Caractéristiques du Optic nerve glioma

A

Slow, progressive proptosis in the first 2 decades
+/- Vision loss
+/- Involuntary eye mvt
Chronic dis swelling
Retinal vascular turtuosity
50% have NF1

Tx :
- Observation
- Chemotherapy
- Radiothx
- Surgery (optic canal invasion)

59
Q

Ddx Paediatric orbital Mass

A

Dermoid/Teratoma
Capillary Haemangioma
Rhabdomyosarcoma (le plus fréquent)
Lymphangioma
Orbital Cellulitis
Optic nerve glioma
Metastatic (Neuroblastoma, Ewing’s, Wilms’…)
Leukemic metastasis
Fibrous dysplasia (Albright’s syndrome)
Histiocytosis (Rosai-Dorfman syndrome)

60
Q

Présentation typique et Tx du Rhabdomyosarcome

A

Typical presentation
- Rapid (orbital cellulitis-like) proptosis
- First 2 decades of life

Tx
- Bx then
- Radiothx + Chemothx (excellent response)

61
Q

Clinical signs suspicious of malignant transformation of an iris nevus?

A

Documented rapid growth
Increasing tumour vascularity
Satellite lesions +/- pigment dispersion
Secondary glaucoma

62
Q

Incidence et Tx du Iris Melanoma

A

Incidence : 5% of all uveal melanoma

Treatment modalities
- Iridocyclectomy
- Brachytherapy
- Proton beam radiotherapy
- Enucleation

63
Q

Caractéristiques d’un Ciliary Body Melanoma (incidence, clinical clues)

A

10% of all uveal melanoma
85% asx → Delayed Dx (because of their hidden position)

Clinical Clues :
- Sentinel vessels
- Iris bulge
- Sector cataract
- Pigmented nodule at iris root
- Episceral pigmentation

64
Q

Anterior Segment Ultrasonography (UBM versus B-scan)

A

Ultrasound Biomicroscopy (UBM)
- Measures iris and ciliary body tumour < 4 mm
- Ciliary body involvement in peripheral tumours
- Circumferential extension
- Internal echogenicity (cysts, necrosis…)

B-scan
- Anterior tumours > 4 mm thickness
- Less accurate, less resolution

65
Q

Incidence et risque de transformation maligne d’un choroidal nevus

A

Incidence : 5-10% of the population

The risk of malignant transformation : 1: 8000 of typical naevi

66
Q

Imaging of choroidal melanoma

A

A-scan :
- Low internal reflectivity
- Angle-kappa (Decrescendo pattern)

B-scan :
- Acoustic hollowness
- Choroidal Excavation
- +/- Orbital Shadowing

Posterior Segment Ultrasonography :
- Measures apical height
- Tumour profile (dome, mushroom, flat…)
- Presence of sub retinal fluid
- Differentiates melanoma from other choroidal masses (in case of opaque media, non-pigmented tumour…)

Fluorescein Angiography :
- Dual-circulation sign
- Subretinal haemorrhage
- Hot Spots sign
- Delineation fo tumour margins

67
Q

Caractéristique Choroidal Melanoma (demographics and most common sites for metastasis)

A

Demographics :
- Peaks 55-70 ans
- No sex predilection
- Fair-skinned individuals

Most common sites for metastasis :
- Liver > 90%
- Lung
- Subcutaneous, GIT, renal…

68
Q

Clinical Risk Factors for Growth Intraocular Melanotic Lesions (To Find Small Ocular Melanoma Ultimately without Delay!)

A

To Find Small Ocular Melanoma Ultimately without Delay!

Thickness > 2 mm
Subretinal Fluid
Symptoms
Orange pigments
Margin near optic disc
Ultrasound hollowness
Absence of drusens

69
Q

Treatment of Uveal Melanoma

A

Radiation therapy
- Brachytherapy
- Teletherapy
Thermotherapy/PDT
Surgical Resection
Enucleation

No effective : Chemotherapy, Cryotherapy, Immunotherapy

70
Q

Isotopes de la brachytherapy

A

Ruthenium 106
Iodine 125

Autres :
Palladium 103
Iridium 192
Strontium 90
Gold 198
Cobalt 60

71
Q

Complications/ES de la radiothérapie

A

Cataractes
Glaucome néovx
Radiation retinopathy
Radiation papillopathy

72
Q

Indications de la trans pupillary thermotherapy (TTT)

A

Mechanism is unknown

Indications :
Tumour edge recurrence post radiotherapy
Adjuvant treatment with decentred plaques
Sandwich Technique with Ruthenium plaques

73
Q

Caractéristiques d’un Optic Disc Melanocytoma

A

Melanotic lesion
Histology :
- « Magnocellular Nevus »
- Plump Polyhedral cells with numerous melanosomes

Px :
- Subtle growth in 10% of cases
- Enlarged blind spot
- Arcuate field defect
- CRVO/CRAO
- Melanoma transformation : 2% of cases

74
Q

Caractéristiques d’un CHRPE (Congenital Hypertrophy of The Retinal Pigment Epithelium)

A

Melanotic lesion
Anomalie congénitale qui arrive in early age
Pigment souvent darker
Unique ou multiple
Lésion habituellement bien délimitée

No risks
Slow growth in 50% of cases
Small scotoma

Gardner syndrome :
- AD adenomatous colonic polyposis
- CHRPE-like lesions : lésions qui ressemblent plus à un poisson, bear tracks (lorsque mutiple lesions)
- Extracolonic benign tumours (osteoma, fibroma…)

75
Q

Caractéristiques Choroidal Metastases

A

Amelanotic Lesion
25% of cases with NO history of primary
Most common site of origin : lung cancer, breast cancer, GU cancers
Most common presentation : unilateral, unifocal and amelanotic lesion
CAT :
- Chemotherapy of the primary cancer
- Low dose external radiotherapy
- Plaque radiotherapy for a recurrent unifocal metastasis

76
Q

Choroidal Metastasis versus Amelanotic Melanoma (surface, growth rate, echogenecity, profile, intrinsic vessels)

A

Choroidal METASTASIS
- Surface : Leopard skin
- Growth rate : Significantly faster
- Echogenecity : Medium-high
- Profile : Irregular, diffuse, dome
- Intrinsic vessels : Absent (avasculaire)

Amelanotic MELANOMA
- Surface : +/- subtle intrinsic pigmentation
- Growth rate : Relatively slower
- Echogenecity : Medium-low
- Profile : Dome, diffuse, collar-button
- Intrinsic vessels : +/- Present

77
Q

Caractéristiques intraocular lymphoma

A

Non-Hodgkin’s LARGE B-cell lymphoma
Corticosteroids-resistant vitritis
+/- patchy retinal infiltration

80% of PIOL will develop PCNSL
20% of PCNSL will develop PIOL
PIOL : Primary intraocular lymphoma
PCNSL : Primary CNS lymphoma

78
Q

Caractéristiques Uveal +/- Adnexal Lymphoma

A

May have intraocular and extra-ocular components
Non-Hodgkin’s SMALL B-cell lymphoma (Mostly MALT)
Choroidal unifocal or multifocal infiltrates
Ultrasound : uveal thickening +/- extra ocular thickening
OCT : sea sick appearance
Tx : ocular irradiation
Px depends on lymphoma type :
- MALT & Follicular lymphoma : ocular, good Px
- Mantle cell & DLBC lymphoma : systemic, poor Px

79
Q

Caractéristiques d’un Choroidal Granuloma

A

Irregular margins with clumps of cells
Satellites +/- vitiritis +/- anterior uveitis
Etiology : TB, sarcoïdose, idiopathique…
Management :
- Posterior uveitis work-up
- If negative : Bx (pour démontrer que c’est idiopathique, et non pas une néo)

80
Q

Caractéristique circumscribed choroidal haemangioma

A

DD from uveal melanoma
- Orange color
- US : solid, high internal reflectivity
- FA : intense fluorescence (mid and late phases)

CAT:
- Observation (peripheral, dormant, no fluid)
- PDT
- Radiotherapy
- External irradiation

81
Q

Pathologie associée à un diffuse choroidal haemangioma

A

Sturge-Weber Syndrome

Risks : glaucoma, neurological sx

CAT of diffuse choroidal haemagioma
- Low dose external irradiation (20-30 Gys)
- Photodynamic therapy

82
Q

Ddx lésion intraoculaire

A

Melanotic Lesions
a. Iris Nevus
b. Iris Melanoma
c. Ciliary Body Melanoma
d. Iris Epithelial Cyst
e. Choroidal Nevus
f. Choroidal Melanoma
g. Other Melanotic Lesions : Optic Disc Melanocytoma, Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE)

Amelanotic Lesions
a. Choroidal Metastases
b. Amelanotic choroidal melanoma
c. Intraocular lymphoma
d. Choroidal granuloma

Vascular Lesions
a. Circumscribed Choroidal Haemangioma
b. Diffuse Choroidal Haemangioma
c. Retinal Capillary Haemangioblastoma

Calcific Lesions
a. Choroidal Osteoma
b. Retinal Astrocytic Hamartoma
5. Retinoblastoma

83
Q

Complication of retinal hemangioblastoma

A

Exudative retinal detachment (grand risque de décollement rétine 2nd vx)

CAT:
- Observation
- Photocoagulation, PDT
- Cryothx
- Plaque Radiotherapy

84
Q

Caractéristiques Choroidal Ostéome (typical patient, complications, CAT, Px)

A

Typical patient :
- Young female
- Unilateral
- Asx or +/- metamorphopsia (si présent sur la macula)

Complications :
- SRNVM (subretinal neovascular membrane)
- Central vision loss or field defects

CAT of SRNVM in osteoma :
- PDT
- Anti-angiogenic injections
- Laser photocoagulation less efficient (RPE atrophy)

Px : gradual loss of vision from retinal atrophy or SRNVM

85
Q

Caractéristiques Astrocytic Hamartoma

A

Cell of origin : undifferentiated neuroglial cells

Morphologic Types :
1. Round semi translucent lesion
2. Calcified mulberry lesion
3. Mixed, translucent with calcific center

86
Q

Pathologie associée à Astrocytic Hamartoma

A

Sclérose tubéreuse
- Sporadic or AD chromosome 9q
- 40% Astrocytic Hamartoma
- Mental retardation, epilepsy, skin lesions

87
Q

Incidence et 2 sx les plus fréquents du Retinoblastoma

A

Incidence of 1 : 12 000-18 000 live births

Sx :
- Leukocoria (55%)
- Strabismus (25%)

Obstetric Ultrasonography : large RB at 33 weeks of gestation

88
Q

Mutation et chromosome impliqué dans le Retinoblastoma

A

Chromosome 13

Mutation of the 2 alleles (AD) of the tumour-suppressor gene RB1
- « Knudson two-hit hypothesis »

89
Q

Acquisition du Retinoblastoma

A

SPORADIC (No family Hx)
- Non-Heritable (60%) : two somatic mutations in a progenitor retina cell → lésion unilaterale, unifocale
- Heritable (30%) : Germline mutation started in early embryogenesis of the proband → bilateral/multifocal

FAMILIAL (10%) : germline mutation inherited an affected parent → bilateral/multifocal

90
Q

Grouping and staging of Retinoblastoma

A

Group A :
- Tumors < 3 mm
- >1 Disc Diameter (DD) from optic disc
- > 2 DD from fovea

Group B :
- Tumors < 3 mm
- Any location
- No vitreous seeds
- Subretinal fluid (SRF) within 5 mm

Group C :
- Vitreous/SRF seeds within 3 mm from tumour
- SRF within one quadrant

Group D :
- Diffuse large vitreous/subretinal seeds > 3 mm from tumor
- SRF > one quadrant

Group E :
- Anterior segment
- NVG
- Massive Hg
- Diffuse infiltrating

Vitreous seeds : tumor cells floating within the vitreous cavity

91
Q

Initial Assessment for Retinoblastoma

A

Fx Hx and Pedigree drawing
IRM > CT tête et orbites
Examination under anesthesia (EUA) with bilateral Retcam imaging
B-scan and UBM
Fluorescein angiography
Blood sample for gene mutation testing
Group E or orbital tumor : CSF cytology
Treatment planning according to IIRC and TNM staging

92
Q

Ddx d’une leukocoria

A

Cataract
Uveitis/Toxocariasis
PHPV (persistance système vasculaire hyaloïde)
Astrocytoma (from endophytic RB)
ROP
Retinal dysplasia/coloboma
Coat’s disease

Différencier exophytic RB from Coat’s disease :
- Yellowish fundus color
- Unilateral
- Male
- Course of retinal vessels

93
Q

Tx of Retinoblastoma

A

Systemic chemotherapy
- Chemoreduction followed by focal therapy for bilateral RB (group B-D)
- Vincristine + Etoposide + Carboplatin
- Toxicity : ototoxicity, nephrotoxicity, myelodysplasia, acute myeloid leukemia (AML)

Focal therapy
- Focal laser : Group A-B, posterior location, minimal SRF
- Cryothx : peripheral tumours = prior to chemo
- Brachythx : post chemo, unifocal, medium

External Beam Radiothx
- Failed to Chemothx + Focal Thx in an eye with vision potentials
- Risks : secondary cancer (38% at 50 ys), orbital bone deformities (avoid be4 age of 12 months)

Enucleation
Indications :
- Any Group E tumor
- Advanced Group D tumor (if other eye has vision potential)
- Recurrent : if other Tx failed
- Evidence of optic nerve, anterior segment or extraocular invasion

Intra-Arterial Chemothx (IAC)
- Femoral artery catheterization
- Melphalan, Topotecan, Carboplain…
- Variable control rates
- Advantages : fewer systemic side effects than IV chemo
- Disadvantages : specific serious complications (sectoral choroidal occlusive vasculopathy, retinal arteriolar embolization, cataractogenic radiation dose from fluoroscopy)

Intravitreal chemothx
- Intravitreal Melphalan
- Best current Tx for VITREOUS SEEDS
- Cryo at injection site be4 withdrawing
- Toxicity : salt and pepper fundus, reduced ERG response

TORIC (17 decks)

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