Cancer registries and rare cancers: quality of data, supplementary information
RARECARE WP6 3rd meetingNational Institute of Public Health
Warsaw 25th March 2010
RARECARE data quality study on high priority rare cancers (Gemma Gatta, Annalisa Trama)
ObjectivesObjectives
To verify the diagnostic accuracy
To assess the completeness of incidence
To verify the quality of follow-up
To verify the availability of information on stage, treatment and place of treatment
To improve the estimates of incidence and survival
Short listShort list
Mesothelioma Liver angiosarcoma Sarcomas Tumors of oral cavity SNC tumours Germ cell tumours Leukemia Endocrine tumours
Primary prevention
Diagnostic accuracy
Secondary prevention
Quality of care
Data quality
MethodsMethods
Revision of the clinical dossiers, pathologic reports filed at cancer registry offices
Revision of the follow-up only for mesothelioma, angiosarcoma of the liver and CNS tumours
Period of diagnosis: 1995-2002
0% 10% 20% 30% 40% 50%
Haut RhinIcelandNapoli
NavarraSomme
UK_West MidlandsMacerata
UK_East AngliaUK_Scotland
MaltaUK_Oxford
ValaisUK_South Western
BiellaAmsterdam
Reggio EmiliaNorway
UK_TrentSlovenia
UK_Northern&YorkNorth Netherlands
TorinoTarragonaEindhovenUK_WalesSt. Gallen
GenovaBas RhinMancheSwedenVenetoFerraraWarsawTrentoHeraultParmaUmbria
UK_NorthernBasque Country
FlemishMurciaIreland
SaarlandRomagna
VareseTicino
FirenzeFriuli V.G.
SassariTarnIsere
South PortugalAustria
ModenaBasel
SalernoAlto Adige
GironaGeneva
DoubsCracowRagusa
Kielce
Mesothelioma,
3-year relative survival by registry
EUROCARE-4
Registry that revised the data are marked with the red arrow
Mesothelioma revisionMesothelioma revision
The review focused on mesothelioma long term survivors (9050-9053) of any sites
(to verify the diagnostic accuracy and quality of follow-up)
all cases with pleural cancers ≠ mesothelioma (to ascertain the completeness of incidence mesothelioma of the pleura)
Mesotelioma long survivorsMesotelioma long survivors
Cancer registries participating = 29 No. of mesothelioma long survivors revised = 551 (32% F; 68% M)
Results of the morphology check
95% (524/551) were confirmed mesothelioma of (pleura, peritoneum, other male genital organs, unknown primary)
5% (27/551) were not mesothelioma. In details:• 8 adk (colon, lung, breast, ovary)• 6 neoplasm, NOS (lung, pleura, ovary)• 3 sarcomas (lung, bone, thyroid gland)• 1 lymphoma• 9 (benign, border line, error)
Mesothelioma long survivorsMesothelioma long survivors
Results of the life status check
455/551 (83%) confirmed as mesothelioma long survivors 318 (70%) M; 137 (30%) F
68/551 (12%) were mesothelioma not long survivors 22 lost to follow-up 46 death date changed
23/551 (4%) were not mesothelioma long survivors
Pleura cancers Pleura cancers ≠≠ mesotheliomamesothelioma
Morphology before Morphology before revisionrevision Freq. Percent Cum.
neoplasms, NOS 457 67.11 67.11
adk 141 20.7 90.01
squamous cell neoplasms 15 2.2 69.31
sarcomas 41 6.02 96.18
thymoma 1 0.15 90.16
hematopoietic 26 3.82 100
Total 681 100
Cancer registries participating = 29 No. of cases revised = 681 (58% M, 42% F)
Pleural cancers ≠Pleural cancers ≠ mesotheliomamesothelioma
465/681 (68%) pleura cancers• 323 = neoplasms, NOS• 47 = mesotheliomas• 34 = sarcomas• 56 = adk• 5 = squamous cell
216/681 (32%) not pleura cancers • 87 = adk (digestive, respiratory,
female genital, urinary, unknown primary)
• 67 = neoplasms, NOS (digestive tract, respiratory system, female gen organs, unknown primary)
• 26 = hematopoietic• 9 = squamous cell (respiratory, urinary
tract)• 9 = sarcomi (respiratory, skin, soft
tissue, unknown primary)• 55 = benign• 2 = records not reviewed
Results of the morphology check
Malignant digestive endocrine Malignant digestive endocrine
tumour (MDET)tumour (MDET)
Issues for data quality
Diagnostic accuracy (difficulties in distinguish tumors with different prognosis) Undifferentiated small-cell MDET
• (8041/3) and (8042/3) Well-differentiated MDET
• (8150/3), (8151/3), (8153/3), (8155/3), (8152/3), (8246/3), (8240/3, 8241/3, 8243/3, 8244/3)
Behaviour (carcinoids)
MDETMDET
The review focused on
undifferentiated (8020/3) and anaplastic (8021/3) carcinomas of the digestive tract (C15 to C25)
(to find small cell MDET)
all carcinoids (8240-8244) of the digestive tract (C15 to C25) (to verify the behaviours)
Criteria for defining the behaviour of carcinoids:
Invasion of the muscularis propria
Dimension of the tumour
Well differentiated benign and bordeline ET
Well differentiatedendocrine carcinoma
Undiff endocrine carcinoma
Differentiation Well differentiated Well differentiated Undifferentiated
Angioinvasion No Possible Possible
Size Stomach,
Small intestine: < 1cm
Appendix, colon, rectum: < 2 cm
Pancreas : < 2 cm
Stomach,
Small intestine: >1 cm
Appendix, colon, rectum: > 2 cm
Pancreas : >2 cm
Mitotic Index < 2 2 to 10 > 10
Prol index < 2 % 2 to 15 % > 15 %
Local invasion Digestive tumour:mucosae/submucosae Pancreas: intra-pancreatic
Digestive tumour:> Muscularis propria Appendix: invasion of the
visceral peritoneum
Pancreas: extra-pancreatic extension
Metastases no Possible Possible
Behavior: /1 /3 /3
Carcinoids (Carcinoids (behaviorbehavior))
Cancer registries participating = 21 No. of cases revised = 1672
Information for defining the behavior: available for ONLY 223 cases Behavior defined only if both dimension of the tumor and local invasion were available
TopographyBehavior
/1 /3NA deleted
Total
esophagus 0 0 5 0 5
stomach 13 17 199 2 231
small intestine 26 86 503 3 618
colon 2 17 177 2 198
appendix 18 1 269 1 289
recto-sigmoid junction 1 0 12 0 13
rectum 15 8 161 3 187
anus and anal canal 1 0 5 0 6
liver 0 0 1 0 1
gallbladder 0 0 7 0 7
other, unspec parts of biliar tract 3 0 5 0 8
pancreas 6 8 92 2 108
thymus 1 0 0 0 1
Total 86 137 1436 13 1,672
Carcinoids (behaviour): resultsCarcinoids (behaviour): results
Undifferentiated and anaplastic Undifferentiated and anaplastic carcinomascarcinomas
Cancer registries participating = 26 No. of cases revised = 844
Freq. %
undifferentiated carcinoma 719 85.19
small cell tumors 7 0.83
epithelial neoplasms, NOS 9 1.07
squamous cell 4 0.47
adk 74 8.77
sarcomas 2 0.24
lymphoma 1 0.12
info not avail 26 3.08
not malignant 2 0.24
Total 844 100
Results of the morphology check
Central Nervous System Central Nervous System tumourstumours
Issues for data quality
Diagnostic accuracy Quality of follow-up
from 27% to 16%
>20% (Finland, Iceland, Norway, Ireland, Wales,Austria, Belgium, Germany, Switzerland, Portugal)
source: Sant et al, EJC, 2008
CNS tumours CNS tumours 5-year relative survival5-year relative survival
CNS tumours revisionCNS tumours revisionThe review focused on
Long-term survivors with a diagnosis of unspecified morphology codes (8000, 8001, 8010)
(to verify the diagnostic accuracy and quality of follow-up)
Cases with diagnosis of Glioma NOS (9380) microscopically verified
(to verify the diagnostic accuracy for tumours with treatment options)
CNS tumoursCNS tumours long survivors long survivors Cancer registries participating = 22 No. of brain cancers long survivors revised = 705 (53% F; 47% M)
Results of the morphology check
93% (653/705) were confirmed brain tumours• 544 = neoplasms NOS• 44 = astrocitomi• 6 = oligodendroglial• 6 = non glial/embryonal tumurs • 2 = ependimal tumours• 1 = sarcoma• 47 = not malignant• 3 = epithelial neoplasms, NOS
5% (27/551) were not mesothelioma. In details:• 8 adk (colon, lung, breast, ovary)• 6 neoplasm, NOS (lung, pleura, ovary)• 3 sarcomas (lung, bone, thyroid gland)• 1 lymphoma• 9 (benign, border line, error)
CNS tumoursCNS tumours long survivors long survivorsResults of the morphology check
7% (52/705) were not brain tumours• 17 = meningiomas• 4 = sarcomas• 3 = neoplasms, NOS• 2 = lung tumours (1epithelial and 1 squamous cell neoplasm)• 2 = breast adk• 2 = lymphomas• 2 = ependimal tumours• 1 = skin melanoma• 1 = endocrine glands germinoma• 1= spinal cord astrocitoma• 11 = not malignant• 8 = information not available
CNS tumoursCNS tumours long survivors long survivors
Results of the life status check
343/705 (49%) confirmed as real long survivors Real brain tumors survivors 282/705 (40%) Not brain tumours long survivors 61/705 (9%)
337/705 (48%) were brain tumours not long survivors 124 lost to follow-up 213 death date changed
For 25 cases (3%) the information on the follow-up was missing. It has to be verfied with CRs
Glioma, NOSGlioma, NOS Cancer registries participating = 21 No. of cases revised = 472 (55% M, 45% F)
Morphology after the revision Freq. %
glioma malignant 362 76.69
astrocytic tumors 87 18.43
oligodendroglial 5 1.06
non glial/embryonal tumors 3 0.64
ependimal tumors 1 0.21
neoplasms, NOS 4 0.85
sarcoma 2 0.42
not malignant 8 1.69
Total 472 100
96 cases of brain tumours will contribute to modify incidence and 96 cases of brain tumours will contribute to modify incidence and survival of the second layer entitiessurvival of the second layer entities
Gonadal germ cell tumoursGonadal germ cell tumours
Issues for data quality
Diagnostic accuracy for tumors with treatment options(% of morphology codes, NOS)
Gonadal germ cell tumoursGonadal germ cell tumours
The review focused on: morphology NOS (8000-8010) cases of the testis
and of the ovary. ONLY microscopically verified cases
Gonadal germ cell tumours (1)Gonadal germ cell tumours (1) Cancer registries participating = 25 No. of cases revised = 1829
Results of the morphology check
89% (1629) were confirmed unspecified morphology of female and male genital organs
2.3% (41) = non epithelial tumors ovary/testis• 23 = germ cell tumours (19 testis; 4 ovary) • 12 = sex cord tumors of the ovary• 6 = malignant/immature teratomas (2 ovary, 4 testis)
Gonadal germ cell tumours (2)Gonadal germ cell tumours (2)Results of the morphology check (2)
5.96% (109) = adk (stomach, breast, 105 ovary, prostate, 1 testis) 1.53% (28) = other epithel neoplasms, NOS of the ovary and testis 0.16% (3) = squamous cell ca ovary 0.16% (3) = mixed epithel/mesench tumor of the ovary 0.11% (2) = sarcomas (soft tissue and ovary) 0.05% (1) = transitional cell carcinomas of the bladder 0.05% (1) = mesothelioma 0.05% (1) = ependimoma of the brain 0.05% (1) = lymphoma 0.44% (8) = benign tumours 0.11% (2) = deleted
The revision identified 23/1829 (1.2%) germ cell tumorsThe revision identified 23/1829 (1.2%) germ cell tumors
Ovary and TestisOvary and Testis
Malignant neoplasms, NOS - Before and after the reviosion
0
100
200
300
400
500
600
700
800
CR
N c
ases
before
after
Liver angiosarcomaLiver angiosarcoma
With morphology different from colangio/hepatocellularcarcinoma, hepatoblastoma.
Sarcoma NOS Angiosarcoma long survisors
(>1yr)
Angiosarcoma, Angiosarcoma, long survivorslong survivors
4 CRs identified long survivors
13 long survivors
6 long survivors (2m, 4f)
(range: 16 months-11years)
5 cases were lost to follow-up, 2 survived <1years
Liver, sarcoma NOSLiver, sarcoma NOS
8 CRs identified sarcoma NOS (18 cases checked)
malignant neoplasm, NOS 1epatocellular ca 6sarcoma nas 8800 3sarcoma, indifferentiated 3GIST 2angiosarcoma 2embrional sarcoma 8991 1
Epithelial tumor of theEpithelial tumor of the liver, liver, morphology not typicalmorphology not typical
1306 cases checked (29 CRs)
1134 cases of liver (87%)
None angiosarcoma
cholangiocarcinoma 9
hepatocellular carcinoma 57hepatocholangio ca 3
Epithelial tumor of theEpithelial tumor of the liver, liver, morphology not typicalmorphology not typical
1306 cases checked (29 CRs)
172 cases ≠ of liver (13%)
site unknown 55%digestive 38%lung 4%genital♀ 1%no tumour 1%
SarcomasSarcomas
Pathological diagnosis is not easy Delay in diagnosis and treatment
Checks of Sarcoma NOS or descriptive codes (8800, 8801-6)
0 5 10 15 20
CalvadosWarsaw
BaselRagusaSassari
AmsterdamNavarra
SloveniaFirenzeCracow
Haut RhinFerrara
IsereBas Rhin
IcelandGironaMurciaHerault
Basque CountryIreland
UK_East AngliaGenova
St. GallenEindhoven
MancheTarn
DoubsUK_Oxford
SaarlandTicino
AustriaUK_Northern&Yorks
North NetherlandsBiella
GranadaUK_West Midlands
MaltaParma
SommeFlemishGeneva
UK_South WesternUK_Scotland
NorwayTorino
ModenaVarese
UK_WalesCalvados digestive
VenetoTarragona
ValaisRomagnaUK_Trent
UK_Northern IrelandReggio Emilia
KielceFriuli V.G.Macerata
TrentoNapoli
Loire AtlantiqueSalernoUmbria
SwedenSouth Portugal
Alto Adige
% Sarcoma NOS% Sarcoma NOS by registryby registry
Registry that verified thedata are marked with the red arrow
SarcomasSarcomas
28 CRs checked
1725 sarcoma, NOS
No change, Sarcoma NOS
1271 cases (74%)1271 cases (74%)8 excluded from the incidence
No Sarcoma,38 cases
Sarcoma, different codes, 408 cases (24%)
SarcomasSarcomas
Sarcomas different codes No sarcomas
sarcoma NOS 77GIST 114sarcoma benign/borderline 9other sarcoma 217
neoplasms NOS 3epithelial NAS 5squamous cell ca 4adenocarcinoma 9melanoma 4non solid tumour 5glioma, mening and other CNS 8
Sarcoma, NOS
050
100150200250300350400450500
CR
N c
ases
Before
After
CRs
Sarcoma NOS before and Sarcoma NOS before and after the revisionafter the revision
LeukaemiaLeukaemia
Two major types: typical and atypical CML with different prognosisRevision of the unspecified codes of
leukaemia and CML, NOS (9800-1, 9820, 9860, 9863)
CML, NOSCML, NOS
17 CRs checked
2378 CML,NOS
No change, CML NOS
2009 cases (84%)2009 cases (84%)7 excluded from the incidence
Specified CML227 typical
24 atypical (11%)
Other leukaemia/lymphoma
55 cases (2%)
Other leukaemia/lymphoma (55 cases)
Lymphoid diseases 19Myeloproliferative neoplasms 23
Myelodisplastic/myeloproliferative diseases 12Leukaemia NOS 1
Leukaemia, NOSLeukaemia, NOS17 CRs checked
1449 leukaemia,NOS
No change, leukamia NOS
1214 cases (84%)1214 cases (84%)16 excluded from
the incidence
Specified CML3 typical
2 atypical
Other leukaemia/lymphoma
219 cases (15%)
Other leukaemia/lymphoma (219 cases, 15%)
Lymphoid diseases 73Acute myeloid leukaemia and related precursor 76Myeloproliferative neoplasms 24Myelodisplastic syndrome 24Myelodisplastic/myeloproliferative diseases 22
Tumours of oral cavityTumours of oral cavity
Issues for data quality
Diagnostic accuracy Morphology: neoplasms, NOS and carcinoma
NOS (ICD-O 8000, 8001, 8010, 8011) Topography: tongue overlapping lesion (C2.8)
and palate, NOS (C5.9)
Both subsites are in oropharynxMorphology NOS are in the layer 1 entity
Tumours of oral cavityTumours of oral cavity revisionrevision
The review focused on
morphology codes 8000, 8001, 8010, 8011 (carcinoma NOS) of the oral cavity (to verify the diagnostic accuracy) C02.0-C02.3, C02.9, C03.0-C05.0, C06.0-C06.9
dorsal and ventral surface of the tongue, border of the tongue,
anterior 2/3 of tongue,.gum, floor of mouth, …mouth NOS
C01.9, C02.4, C02.8, C05.1-C05.2, C05.9, C09.0-C10.3, C10.8-10.9, C14.2base of tongue, lingual tonsil, tongue overlapping, soft palate, uvula, palate,
NOS,…Waldeyer ring.
unspecific site codes C02.8 (overlapping lesion of the tongue) and C05.9 (palate, NOS) (to distinguish between oral cavity and oropharynx) 5-year survival: oropharynx = 37%
oral cavity = 59%
Oral cavity
Oropharynx
Carcinoma, NOSCarcinoma, NOS
Cancer registries participating = 26 No. of cases revised = 555 (68% M, 32% F)
Freq. %carcinoma, NOS 497 89.55
squamous cell 47 8.47
adenocarcinoma 5 0.9
sarcomas 1 0.2
Not in incidence any more 5 0.9
Total 555 100
Results of the morphology check
Unspecified sitesUnspecified sites
Cancer registries participating = 22 No. of cases revised = 388 (71% M, 29% F)
Results of the topography check
Freq. %
unspecified sites 272 70.1
oral cavity 69 17.78
oropharynx 43 11.08
nasopharynx 1 0.26
hypopharynx 1 0.26
erased 2 0.52
Total 388 100
Some conclusions (1)
Mesothelioma, angiosarcoma, CNS neoplasms NAS long survivorsCan we further describe these cases?Can we suggest recommendations to
registries? Pleura and CNS epithelial tumours
Can we suggest recommendations to registries
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