skull-based tumors
TRANSCRIPT
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P t R di ti ThProton Radiation Therapy of Tumors of the Cranial Base
Norbert J. Liebsch, M.D., Ph.D.
of Tumors of the Cranial Base
Department of Radiation Oncology Massachusetts General Hospital, Boston
H d M di l S h lHarvard Medical School
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Uncommon primary malignant bone tumorsp y garising from cranial base
Chordoma: Arises from remnants ofembryonal notochord
Chondrosarcoma: Arises from remnants of embryonal chondrocraniume b yo a c o d oc a u
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Anatomic LocationAnatomic Location
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Natural HistoryNatural History
• Indolent tumor growthIndolent tumor growth
L t t ti t ti l• Low metastatic potential
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3/1997 6/1997
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3/1998 3/1999
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19991999
2005 2005
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Clinical Symptoms & Signs: ChordomaClinical Symptoms & Signs: Chordoma
Headaches 52 % CN II 3 %Headaches 52 % CN II 3 %Neck pain 24 % III 11 %Airway obstruction 17 % IV 1 %N / iti 8 % V 1 %Nausea/vomiting 8 % V 1 %Dysphagia 6 % VI 42 % Dysphonia 3 % VII 3 %y pEust. tube dysfct. 6 % VIII 3 %Torticollis 10 % IX 9 %Ataxia 24 % X 7 %Ataxia 24 % X 7 %
XI 3 %XII 20 %
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Clinical Symptoms & Signs: Chondrosarcoma
• Headaches 40 %• Nasal obstruction 5 %• Eustachian tube dysfunction 5 %• Pituitary dysfunction 3 %• Ataxia 3 %• CN Deficits 81 %
CN II 4 %CN III 9 %CN IV 2 %CN V 10 %CN VI 50 %CNs VII, VIII 7 %CNs IX, X, XI 9 %CN XII 6 %
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Histology of ChordomaHistology of Chordoma
conventional chondroid de-differentiated
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PathologyHistology of ChondrosarcomaHistology of Chondrosarcoma
hyaline myxoid
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Cranial Base SurgeryCranial Base Surgery
• Gross total tumor resection uncommon
• Propensity of cranial base tumors to recur locally
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1992 Preop 1992 Postop
1994 2003
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Proton Radiation TherapyProton Radiation Therapy
High-dose fractionated precision
conformal radiation therapy of
targets in close proximity to critical,targets in close proximity to critical,
dose-limiting anatomical structures.
Effective dose Gy(RBE) = RBE x proton dose [pGy]
RBE = 1.1 (rel. radiobiological effectiveness)
pGy = proton-Gyp y p y
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Definition: Local Tumor Control (LC)
Freedom from tumor progression:
= Absence of radiographic features
of tumor progression
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Since 1975
600 pts. with chordoma of the cranial base– 500 adults– 100 children
400 pts. with chondrosarcoma of the cranial base
have received high-dose postoperative precisionconformal fractionated photon-proton RTconformal fractionated photon proton RTat the Massachusetts General Hospital
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Tumor DoseChordoma: Adults TD = 70 - 83 Gy(RBE)/37 - 44 fxs
Children TD = 79 Gy(RBE)/42 fxs
Chondrosarcoma: TD 70 Gy(RBE)/35 fxsChondrosarcoma: TD = 70 Gy(RBE)/35 fxs
Normal Tissue Dose Constraints
Optic Pathway: D ≤ 62 - 66 CGE
Normal Tissue Dose Constraints
Optic Pathway: D ≤ 62 66 CGE
Brain Stem:Surface D ≤ 67 - 70 CGE
Center D ≤ 55 - 58 CGE
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Normal Tissue Dose ConstraintsOptic Pathway: D < 62 Gy(RBE)Brain stem: surface: D < 67 Gy(RBE)Brain stem: surface: D < 67 Gy(RBE)
center: D < 55 Gy(RBE)
7976
chiasm
76726762555540 brainstem
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Local Tumor Control
Chordoma (adults) ChondrosarcomaLC = 75 % at 5 yrs. LC = 98 % at 5 yrs.
= 55 % at 10 yrs. = 95 % at 10, 15 yrs.
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Local Tumor Control: ChordomaAdultsAdultsLC = 75 % at 5 yrs.
= 55 % at 10 yrs
100
= 55 % at 10 yrs.
MalesLC = 80 % at 5 yrs 60
80
rol MM
LC = 80 % at 5 yrs.
= 65 % at 10 yrs.
Females40Lo
cal C
ontr
Adults
MalesFemalesLC = 65 % at 5 yrs.
= 45 % at 10 yrs
20
Males
Females
Children
45 % at 10 yrs.
ChildrenLC = 80 % at 5 10 yrs
00 5 10
Years
LC = 80 % at 5, 10 yrs.
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Local Tumor Control100 children with chordoma of the cranial basemedian F/U = 9.5 years
LC = 81 % 5 yrs.= 78 % 10 yrs.y= 72 % 15 yrs.
20 yrs20 yrs.
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LC: 100 Children with Chordoma of Cranial Base
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ConclusionChordoma and low-grade chondrosarcoma of the cranial base
are locally invasive tumors with low metastatic potential.y p
They can be successfully treated by an interdisciplinary
combined-modality strategy integrating:
(1) Cranial Base Surgery( ) g y
(2) Proton Radiation Therapy
with the aim of maximizing tumor control and minimizing
treatment-related morbidity.
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Treatment Strategy: Chordoma
Cranial Base Surgery:
Gross total resection of chordoma if feasible with acceptable surgical morbiditywith acceptable surgical morbidity.
M i d b lki f h d iMaximum debulking of chordoma to improve target geometry for postoperative proton RT.
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Treatment Strategy: Chondrosarcoma
Cranial Base Surgery:
Biopsy to establish diagnosis
Li it d t ti t iLimited tumor resection to improve tumor-related functional deficits
Partial tumor resection to improve target geometry for postoperative proton RT.g y p p p
N d f l i f l dNo need for complete tumor resection of low-grade chondrosarcoma with potentially higher surgical morbidity
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RT - ToxicityRT Toxicity• Neuro-cognitive• Neuro-endocrine• Visual• Audiologic• CNs
C b l• Cerebro-vascular• Cranio-facial
Osteoradionecrosis• Osteoradionecrosis• RT- induced tumor
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Chordoma of Cranio-cervical Junction11 y/o F, progr. rt.-sided weakness
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Chordoma of Cranio-cervical Junction
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Chordoma of Cranio-cervical Junction
12/22/06: Transoral-transpharyngeal biopsy: chordoma
01/27/07: Dorsal occipito-cervical (C3) stabilization
02/07/07: Part transoral transpalatal tumor removal02/07/07: Part. transoral-transpalatal tumor removal,res. ant. arch C1, odontoidectomy
05/02/07: Part. tumor removal via rt. submental appr.
06/07/07: Part. tumor removal via lt. submental appr.
06 12/07 Gl (I itib l t ) 400 / 2 d06-12/07: Gleevec (Imanitib mesylate) 400mg/m2 q.d.
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3333
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Chordoma of Cranio-cervical Junction
Near total removal of intracranial-intraspinal tumorin 2 stages:
01/14/08: Lt. far lat. transcondylar appr.
01/29/08: Rt. far lat. transcondylar appr.
Postop: part. lt. CN XII palsy
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Thank youy