Epidemiological Study of the Incidence of Cancers Eligible for Proton or Carbon Ions Therapy: Methodology and Results of Recruitment Estimation
Table 1
Ranked list of hadrontherapy indications.
Type
Location
Clinical situations
ORPHANET number
European prevalence/100,000/year/2011*
Head and neck (H and N)
Salivary glands
Inoperable/refusal of surgery/R2 resections/local relapses/all histology
Facial sinuses
Inoperable/refusal of surgery/R2 resections/local relapses/all histology
Cystic adenoid carcinoma, base of skull expansion
Inoperable/refusal of surgery/R2 resections/local relapses
Malignant mucosal melanoma
Any location without immediately threatening metastases Tumour if possible nonoperated or R2 or nonirradiated local relapse
ORPHA168999
46.8
Squamous cell carcinoma
Relapses or second unresectable location in irradiated area and M0
ORPHA67037
<40
Skull brain spinal column
High-grade glioma (grade III or glioblastomas)
Relapse after initial radiotherapy treatment ± chemotherapy and progressing under chemotherapy. Initial treatment possibly after surgery and other EG inoperable in R2
ORPHA360
1
Benign meningioma
Base of skull, convexity
ORPHA2495
Malignant meningioma
Base of skull, convexity
ORPHA2495
Pituitary adenoma
Base of skull, to large for surgery or stereotactic rT
ORPHA99408
Ependymoma
Brain, posterior fossa, spinal column
ORPHA301
VIII neurinoma
Base of skull, to large for surgery or stereotactic rT
ORPHA637
Local and meta medulloblastoma
CNS
ORPHA616
Chordoma and sarcoma
Base of the skull, spinal column and sacrum: All clinical forms
ORPHA178
Digestive
Single hepatocarcinoma
> 4 to 5 cm, unresectable, M0, untreatable by conventional methods or photon therapy, with no life-threatening comorbidities
ORPHA88673 ORPHA210159 ORPHA33402
1
Single nodular cholangiocarcinoma
unresectable, M0, not previously irradiated, and nonprogressive under chemotherapy for 4 to 6 months
ORPHA70567
Pancreatic adenocarcinoma
unresectable, M0, not previously irradiated, and nonprogressive under chemotherapy for 4 to 6 months
ORPHA217074 ORPHA1333
11.9
Endocrine tumours of the pancreas (M0)
M0, progressive after multiple treatments: isotopic and/or chemotherapy and somatostatin
ORPHA97253
Pelvic relapse of rectal cancer
M0, previously irradiated or not
Sarcoma and chordoma
Nonretroperitoneal soft tissue
Low grade and M0, all histologies, all sites. Unresectable, surgery refused, or “definitive R2”: R2 without possible revision, R2 after revision surgery, or local R2 relapse. Nonthreatening M+ situation with debilitating T or rT
Retroperitoneal sarcoma
After local relapse and revision surgery: “R0” or R1 and M0 (for unresectable and R2 Ts, see above). Or R1 M0 situation
Head, neck, and limb soft tissues
“Definitive R1”: R1 resection without acceptable possibility of surgery
ORPHA97338
Osteo- and chondrosarcoma (excluding axial skeleton)
Nonoperated tumours or R2, M0 resections. M+ accepted for osteosarcomas only. Discussion according to grade
ORPHA223727
Chordoma and sarcoma
Base of the skull, spinal column, and sacrum: all clinical forms
ORPHA178
Paediatric tumours
Pelvic Ewing's sarcoma
inoperable, voluminous (more than 100 or 200 mL according to age)
ORPHA319
0.1
Aggressive chordoma in young children
<3-4 years
ORPHA178
Osteosarcoma
pelvic, unresectable
ORPHA668
Cranial and parameningeal sarcoma
Skull and base of skull
Primitive ectodermal tumour
M0 or inoperable
ORPHA251870
Medulloblastoma
Posterior fossa + medullary irradiation
ORPHA616
Craniopharyngioma
ORPHA54595
Ependymoma
ORPHA301
Optic pathway glioma
ORPHA2086
Neuroblastoma
M0 and inoperable
ORPHA635
11.3
Retinoblastoma
ORPHA790
5.4
Other
Miscellaneous, highly functional sites, meeting the study inclusion criteria
Criteria to declare in appendix 4 (study questionnaire)
Legend: R0: complete tumour resection without residues; R1: tumour resection with microscopic residues; R2: tumour resection with macroscopic residues; EG: conditions; CNS: central nervous system; : circumference; M0: medium-term metastatic risk (within 5 years) and considered to be sufficiently low to justify locoregional treatment; M+: several metastases at time of initial diagnosis, leading to discussion of locoregional treatment; T: tumour; rT: radiotherapy.
*Estimated incidences according to the ORPHANET 2011 survey [12].
