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

33 CRs from 14 countries

MesotheliomaMesothelioma

Issues for data quality

Diagnostic accuracy Quality of follow-up

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

% of leukaemia NOS by registry (average 6%)

0% 10% 20% 30% 40%

Leukemia NOS before vs after the revision

0100

200300400500

600700

CR

No c

ases

Before

After

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

Some conclusions (2)

MDET Can we accept the criteria for define the

invasion for carcinoids? Can we improve the diagnosis?

Sarcomi GIST

Leukaemia Underestimation of CML incidence Evolution of the lymphoma and leukaemia

classification Others?