(精品) 眼肿瘤学--概念与进展课件

challenge
▪ Early diagnosis ▪ Treatment for IIRC－D/E ▪ Lower recurrence ▪ Convenient model ▪ New drugs for Rb ▪ Mutation detection
rapid ,cheap and accurate
Rb
gnosis
▪ Hereditary: Norrie’s disease,congenital retinoschisis、FEVR、incontinential pigmenti
▪ Developmental: PHPV ▪ Inflammatory: toxocariasis(弓蛔虫)、
only choice
Mutations in Rb
▪ Mutation in one of 24 exons among total 27 exons ▪ types：nonsense mutations/frameshift
mutations/missense mutations/in-frame deletion/promotor mutations/intron mutations ▪ Detecting methods：碱基对分析，间接法 （RFLP/VNTR),直接法（SSCP/direct sequencing/FISH）
▪ Toronto ： Cyclosporine(CSA) 33mg/kg ,第1和第2天，注射3小时。 Vincristine 0.05mg/kg, 第2天，注射5分钟。 Etoposide 9mg/kg, 第2天，注射25分钟。 Carboplatin 22mg/kg, 第1天，注射30分钟。
Retinoblastoma(after enucleation)
▪ A scan：血管搏动、1/3旗 型
Infiltrating beyond eyeball
FFA
▪ Not characteristic ▪ Double circulation、多个针尖渗漏（hot spots） ▪ c.f. Macroaneurysm、disciform lesion、HCD
▪ Subsequent conservative treatment cryotherapy thermotherapy plaque radiotherapy
▪ Avoid enucleation and EBRT , except IIRC- E
Phili and Toronto chemo. protocol
MRI
▪ T1W high signal
▪ T2W low signal
Treatment (uveal melanoma)
▪ Depends on size. ▪ Options include:
Observation Enucleation TTT Brachytherapy Local resection ▪ Enucleation vs brachytherapy - No difference in risk of Mets/Survival ▪ Diagnostic biopsy
Video of uveal tumor resection (by Dr.Jiang Qian,2005)
Follow-up
▪ Clinical Exam ▪ +/- Liver imaging ▪ Long-term f/u necessary (COMS)
5yr rate of distant mets = 25% 10yr rate of distant mets = 34%
＞50% globe NVI
opaque media
local treatment chemo. plus chemo. plus chemo. plus enucleation
Retinoblastoma(chemo.plus)
▪ Chemoreduction to reduce tumor volume VEC
Sebaceous gland carcinoma (SGC)
▪ Mostly origin from Meibomian gland （Meibomian gland carcinoma）
▪ Elderly women, ’chalazion’,subcutaeous nodule,white mass from tarsal side
Ocular surface squamous neoplasm
（OSSN）
▪ Squamous cell origin
▪ Degeneration/carcinoma in situ
/squamous cell carcinoma
▪ Sunshine/ultraviolet/hpv
SCC
▪ Fissure area,limbus especially,weeks to years
▪ Metastasize frequently ▪ Excision & reconstruction ▪ Chalazion in elderly need regular
pathological examination
Cutler-Beard pro. 80% upper lid defect after resection, 22ms later
▪ Most common eyelid malignancy ▪ Nevus-like nodule, solidly,
grow very slowly, ulcer, ‘mouse-bitten ‘ appearance ▪ Local extension,metastasis rarely ▪ Sensitve to radiation
International intraocular retinoblastoma classification (IIRC,simplified)
Group ▪ A small ≤3mm ▪ B bigger ＞3mm macula,srf ▪ C contained seeds ▪ D diffuse seeds ▪ E extensive
▪ No curative therapy for metastatic disease
quiz
▪ The most common eyelid malignancy is …… ▪ ……should ruled out pathologically in a chalazion in an
Conjunctival nevus
▪ Color
brown
65%
tan
19%
nonpigmented 16%
▪ Discrete
▪ Elevated
▪ Cysts
▪ Stationary
Special nevi
▪ Marginal nevus cilia
▪ Kissing nevus cosmesis
▪ Philadelphia： Vincristine sulfate 1.5mg/m2 (0.05mg/kg for ≤36月儿童，最大量≤2mg) Etoposide 150mg/m2 (5mg/kg for ≤36月儿童) Carboplatin 560mg/m2 (18.6mg/kg for ≤36月儿童) 全部0天给药，Etoposide 1天。 ×6 cycles 28天为一个周期。
Ocular Oncology ------concept and advances
Scope of ocular oncology
▪ Eyelids ▪ Conjunctiva ▪ Intraocular ▪ Orbital ▪ Phakomatosis
Basal cell carcinoma (BCC)
▪ Indirect ophthalmoscopy the most important ex. 95％
▪ Dome-shaped/buttonshaped/mushroom-shaped mass, colored,exudative RD
▪ Breakthrough Bruch membrane, rare in other tumors
Malignant changes of nevus
▪ A newly nevus in an elderly people ▪ Recorded growth (area & thickness) ▪ cyst or cilia diminished ▪ Corneal extension ▪ palpebral conjunctiva ▪ Obvious feeding vessel ▪ Satellite ▪ Recurrence after excision
Primary acquired melanosis（PAM）
▪ Elderly/middle age, caucasian A typical PAM ▪ Acquired yellow to brown patch
/Unilateral/discrete/irregular/obscure margin ▪ Granular appearance, no cyst ▪ Limbus, invade cornea/palpebral conj./fornix/skin
Primary acquired melanosis（PAM）
▪ 50% PAM with cellular atypia
→ melanoma
melanoma from PAM
▪ Horizontal growth→ vertical growth
Zimmerman
▪ PAM ≈ carcinoma in situ