Starting with the 1975-2018 SEER Research Data (November 2020 submission), the Rare Cancer classification variable is available. The list of rare cancers was developed by the Surveillance of Rare Cancer in Europe (RARECARE) (1) to include clinically relevant, histologically defined rare cancers with an annual crude incidence rate smaller than 6 per 100,000. The original list of rare cancers was recently revised and applied to the European data and the SEER data, and is now available in SEER*Stat (2). The rare cancer list is organized into 2 tiers. The bottom tier (tier 2) includes rare cancers that are considered similar from the point of view of clinical management and research. The tier 2 rare cancers are then further grouped into more general categories of tumors (tier 1), considered to involve the same clinical expertise and patient referral structure. Only a few tier 1 tumors are rare and some are not sub-classified into tier 2.

The information provided in this table is also available to download (XLSX, 39 KB).

Tumor Primary Site ICD-O-3 Morphology Code Behavior Recode
1. Epithelial Tumors of Nasal Cavity and Sinuses
1.1 Squamous cell carcinoma with variants of nasal cavity and sinuses C30.0, C31 8004, 8023, 8032, 8050-8052, 8070-8076, 8078, 8083-8084, 8120-8123, 8130, 8560, 8980 0-3 01
1.2 Lymphoepithelial carcinoma of nasal cavity and sinuses C30.0, C31 8082 0-3 02
1.3 Undifferentiated carcinoma of nasal cavity and sinuses C30.0, C31 8020-8022 0-3 03
1.4 Intestinal type adenocarcinoma of nasal cavity and sinuses C30.0, C31 8144 0-3 04
1.5 Other epithelial tumors of nasal cavity and sinuses* C30.0, C31 8000-8001, 8003, 8010-8012, 8030-8031, 8033-8034, 8046 0-3 05
2. Epithelial Tumors of Nasopharynx
2.1 Squamous cell carcinoma with variants of nasopharynx C11 8004, 8020-8022, 8032, 8051-8052, 8070-8076, 8078, 8082-8084, 8120-8123, 8560, 8980 0-3 06
2.2 Papillary adenocarcinoma of nasopharynx C11 8050, 8260 0-3 07
2.3 Other epithelial tumors of nasopharynx* C11 8000-8001, 8010-8012, 8030, 8033-8034, 8046, 8145 0-3 08
3. Epithelial Tumors of Major Salivary Glands and Salivary-gland Type Tumors
3.1 Epithelial tumor of major salivary glands C07, C08 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8030-8035, 8046, 8050-8052, 8070-8076, 8078, 8082-8084, 8120-8123, 8130, 8140-8141, 8143-8144, 8147, 8190, 8200-8201, 8211, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8401, 8410, 8430, 8440, 8450, 8480-8481, 8490, 8500, 8502, 8504, 8510, 8525, 8550-8551, 8560-8562, 8570, 8574-8575, 8940-8941, 8951, 8980-8982 0-3 09
3.2 Salivary gland type tumor of head and neck C00-C06, C09-C14, C30.0, C31-C32 8140-8141, 8143, 8147, 8190, 8200-8201, 8211, 8230-8231, 8261, 8263, 8290, 8310, 8320, 8323, 8401, 8430, 8440, 8450, 8480-8481, 8490, 8500, 8504, 8510, 8525, 8550-8551, 8562, 8570, 8574, 8675, 8941, 8982 0-3 10
C00-C06, C09-C14, C32 8144
4. Epithelial Tumors of Hypopharynx and Larynx
4.1 Squamous cell carcinoma with variants of hypopharynx C12, C13 8004, 8020-8022, 8031-8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8120, 8123, 8560, 8980 0-3 11
4.2 Squamous cell carcinoma with variants of larynx C32 8004, 8020-8022, 8031-8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8120, 8123, 8560, 8980 0-3 12
4.3 Other epithelial tumors of hypopharynx and larynx* C12, C13, C32 8000-8001, 8003, 8005, 8010-8012, 8030, 8033, 8035, 8951 0-3 13
5. Epithelial Tumors of Oropharynx
5.1 Squamous cell carcinoma with variants of oropharynx C01.9, C02.4, C02.8, C05.1-C05.2, C05.8-C05.9, C09.0-C10.4, C10.8-10.9, C14.2 8004, 8020-8022, 8032, 8050-8052, 8070-8076, 8078, 8082-8086, 8120-8121, 8123, 8560, 8980 0-3 14
5.2 Other epithelial tumors of oropharynx* C01.9, C02.4, C02.8, C05.1-C05.2, C05.8-C05.9, C09.0-C10.4, C10.8-10.9, C14.2 8000-8001, 8003, 8005, 8010-8012, 8030-8031, 8033, 8951 0-3 15
6. Epithelial Tumors of Oral Cavity and Lip
6.1 Squamous cell carcinoma with variants of oral cavity C02.0-C02.3, C02.9, C03.0-C05.0, C06.0-C06.9 8004, 8020-8022, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8123, 8560, 8980 0-3 16
6.2 Squamous cell carcinoma with variants of lip C00.0-C00.9 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8090-8095, 8097, 8110, 8390, 8400, 8407, 8410, 8560, 8980 0-3 17
6.3 Other epithelial tumors of oral cavity and lip* C02.0-C02.3, C02.9, C03.0-C05.0, C06.0-C06.9 8000-8001, 8003, 8010-8012, 8015, 8030-8031, 8033-8034, 8951, 8981 0-3 18
7. Epithelial Tumors of Esophagus
7.1 Squamous cell carcinoma with variants of esophagus C15 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8123, 8560, 8980 0-3 19
7.2 Adenocarcinoma with variants of esophagus C15 8140-8145, 8147, 8190, 8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8551, 8570-8576 0-3 20
7.3 Salivary gland type tumor of esophagus C15 8200, 8430, 8550, 8562 0-3 21
7.4 Undifferentiated carcinoma of esophagus C15 8020-8022 0-3 22
7.5 Other epithelial tumors of esophagus* C15 8000-8001, 8003, 8010-8012, 8030-8031, 8033, 8035 0-3 23
8. Epithelial Tumors of Stomach
8.1 Adenocarcinoma with variants of stomach C16 8050, 8140-8145, 8147, 8190, 8201, 8210-8211, 8214, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 24
8.2 Squamous cell carcinoma with variants of stomach C16 8004, 8032, 8051-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 25
8.3 Salivary gland-type tumor of stomach C16 8200, 8430 0-3 26
8.4 Undifferentiated carcinoma of stomach C16 8020-8022 0-3 27
8.5 Other epithelial tumors of stomach* C16 8000-8001, 8003, 8005, 8010-8012, 8030-8031, 8033, 8035, 8040, 8122-8123, 8130, 8951 0-3 28
9. Epithelial Tumors of Small Intestine
9.1 Adenocarcinoma with variants of small intestine C17 8020-8022, 8140-8141, 8143-8145, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 29
9.2 Squamous cell carcinoma with variants of small intestine C17 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 30
9.3 Other epithelial tumors of small intestine* C17 8000-8001, 8003, 8010-8012, 8030-8031, 8033-8034, 8220, 8951 0-3 31
10. Epithelial Tumors of Colon (including appendix)
10.1 Adenocarcinoma with variants of colon C18, C19 8020-8022, 8140-8141, 8143, 8147, 8190, 8201, 8210-8211, 8213, 8220-8221, 8230-8231, 8255, 8260-8263, 8265, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440-8441, 8450, 8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8571-8576 0-3 32
C18.0, C18.2-C18.9, C19 8480-8481
10.2 Squamous cell carcinoma with variants of colon C18, C19 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 33
10.3 Fibromixoma and low grade mucinous adenocarcinoma of the appendix C18.1 8480-8481 0-3 34
10.4 Other epithelial tumors of colon (including appendix)* C18, C19 8000-8001, 8003, 8005, 8010-8012, 8040, 8951 0-3 35
11. Epithelial Tumors of Rectum
11.1 Adenocarcinoma with variants of rectum C20 8020-8022, 8140-8141, 8143-8145, 8147, 8190, 8200-8201, 8210-8211, 8213, 8220-8221, 8230-8231, 8255, 8260-8263, 8265, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 36
11.2 Squamous cell carcinoma with variants of rectum C20 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8094, 8120-8124, 8130, 8980 0-3 37
11.3 Other epithelial tumors of rectum* C20 8000-8001, 8003, 8010-8012, 8030-8031, 8033, 8040, 8951 0-3 38
12. Epithelial Tumors of Anal Canal
12.1 Squamous cell carcinoma with variants of anal canal C21 8004, 8020-8022, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8095, 8120, 8122-8124, 8980 0-3 39
12.2 Adenocarcinoma with variants of anal canal C21 8140-8141, 8143-8144, 8147, 8190, 8200-8201, 8210-8211, 8213, 8215, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8550-8551, 8560, 8562, 8570-8576 0-3 40
12.3 Pagets disease of anal canal C21 8542 0-3 41
12.4 Other epithelial tumors of anal canal* C21 8000-8001, 8010-8012, 8014, 8033, 8081, 8090, 8094 0-3 42
13. Epithelial Tumors of Pancreas
13.1 Adenocarcinoma with variants of pancreas C25 8010, 8020-8022, 8140-8141, 8143-8145, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440, 8450, 8471, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8521, 8523, 8525, 8542, 8560, 8562, 8570-8576, 8941 0-3 43
13.2 Squamous cell carcinoma with variants ofpancreas C25 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 44
13.3 Acinar cell carcinoma of pancreas C25 8550-8551 0-3 45
13.4 Mucinous cystadenocarcinoma of pancreas (invasive) C25 8470 0-3 46
13.5 Intraductal papillary mucinous carcinoma invasive of pancreasl C25 8453 0-3 47
13.6 Solid pseudopapillary carcinoma of pancreas C25 8452 0-3 48
13.7 Serous cystadenocarcinoma of pancreas C25 8441 0-3 49
13.8 Carcinoma with osteoclast-like giant cells ofpancreas C25 8035 0-3 50
13.9 Other epithelial tumors ofpancreas* C25 8000-8001, 8003, 8005, 8011-8012, 8030-8031, 8033-8034, 8040, 8552 0-3 51
14. Epithelial Tumors of Liver and Intrahepatic Bile Tract (IBT)
14.1 Hepatocellular carcinoma of Liver and IBT C22 8170, 8172-8175 0-3 52
C22, C23.9, C24 8180
14.2 Hepatocellular carcinoma, fibrolamellar C22 8171 0-3 53
14.3 Cholangiocarcinoma of IBT C22 8160 0-3 54
14.4 Adenocarcinoma with variants of liver and IBT C22 8140-8141, 8143, 8147, 8162, 8190, 8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8571-8576 0-3 55
14.5 Undifferentiated carcinoma of liver and IBT C22 8020-8022 0-3 56
14.6 Squamous cell carcinoma with variants of liver and IBT C22 8004, 8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 57
14.7 Bile duct cystadenocarcinoma of IBT C22 8161 0-3 58
14.8 Other epithelial tumors of liver and intrahepatic bile tract (IBT)* C22 8000-8001, 8003, 8010-8012, 8030-8031, 8033-8034, 8981 0-3 59
15. Epithelial Tumors of Gallbladder and Extrahepatic Biliary Tract (EBT)
15.1 Adenocarcinoma with variants of gallbladder C23.9 8020-8022, 8050, 8140-8141, 8143-8144, 8147, 8160-8162, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8450, 8470, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 60
15.2 Adenocarcinoma with variants of EBT C24 8020-8022, 8050, 8140-8141, 8143-8144, 8147, 8160-8163, 8190, 8200-8201, 8210-8211, 8220-8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8450, 8470, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 61
15.3 Squamous cell carcinoma of gallbladder and EBT C23.9, C24 8004, 8032, 8051-8052, 8070-8076, 8078, 8082-8084, 8980 0-3 62
15.4 Other epithelial tumors of gallbladder and extrahepatic biliary tract (EBT) C23.9, C24 8000-8001, 8003, 8005, 8010-8012, 8014-8015, 8030-8031, 8033, 8035, 8053-8069, 8145 0-3 63
16. Epithelial Tumors of Trachea
16.1 Squamous cell carcinoma with variants of trachea C33 8004, 8020-8022, 8031-8032, 8050-8052, 8070-8076, 8078, 8082-8084, 8560, 8980 0-3 64
16.2 Adenocarcinoma with variants of trachea C33 8140-8141, 8143-8144, 8147, 8190, 8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8440-8441, 8470, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 8570-8576 0-3 65
16.3 Salivary gland type tumor of trachea C33 8200, 8430, 8982 0-3 66
16.4 Other epithelial tumors of trachea* C33 8000-8001, 8010-8012, 8033, 8046 0-3 67
17. Epithelial Tumors of Lung
17.1 Squamous cell carcinoma with variants of lung C34 8015, 8050-8052, 8070-8073, 8075-8076, 8078, 8082-8084, 8123 0-3 68
17.2 Adenocarcinoma with variants of lung C34 8140-8141, 8143-8144, 8147, 8190, 8201, 8210-8211, 8221, 8230-8231, 8250-8257, 8260-8263, 8265, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8440-8441, 8460, 8470, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8570-8576 0-3 69
17.3 Adenosquamous carcinoma of lung C34 8560 0-3 70
17.4 Large cell carcinoma of lung C34 8012, 8014, 8034 0-3 71
17.5 Poorly differentiated endocrine carcinoma of lung C34 8002, 8013, 8041-8045, 8246 0-3 72
17.6 Salivary gland type tumor of lung C34 8200, 8430, 8562, 8982 0-3 73
17.7 Sarcomatoid carcinoma of lung C34 8004, 8022, 8030-8033, 8074, 8972, 8980 0-3 74
17.8 Other epithelial tumors of lung* C34 8000-8001, 8003, 8005, 8010-8011, 8020-8021, 8023, 8035, 8040, 8046, 8120, 8146, 8951, 8981 0-3 75
18. Epithelial Tumors of Thymus
18.1 Malignant thymoma C37, C38.1, C38.3 8580-8586 0-3 76
18.2 Squamous cell carcinoma of thymus C37 8046, 8050-8052, 8070-8076, 8078, 8083-8084, 8123 0-3 77
18.3 Adenocarcinoma with variants of thymus C37 8020-8022, 8082, 8140-8141, 8143-8144, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 78
18.4 Other epithelial tumors of thymus* C37 8000-8001, 8003-8004, 8010-8012, 8032-8033, 8588-8589, 8980 0-3 79
19. Epithelial Tumors of Breast
19.1 Inv carcinoma of no special type-NST (obs Invasive ductal carc of breast) C50 8035, 8500-8501, 8507, 8514, 8521-8523 0-3 80
19.2 Invasive lobular carcinoma of breast C50 8022, 8520, 8524 0-3 81
19.3 Mammary Pagets disease of breast C50 8540-8541, 8543 0-3 82
19.4 Special types of adenocarcinoma of breast C50 8050, 8201, 8211, 8260, 8290, 8314-8315, 8401, 8410, 8480-8481, 8502-8503, 8510, 8513 0-3 83
19.5 Metaplastic carcinoma of breast C50 8015, 8032, 8070, 8560, 8570-8572, 8575 0-3 84
19.6 Salivary gland type tumor of breast C50 8200, 8430, 8550, 8982 0-3 85
19.7 Other epithelial tumors of breast* C50 8000-8001, 8003-8005, 8010-8012, 8020-8021, 8030-8031, 8033, 8140-8141, 8143, 8145, 8147, 8190, 8210, 8221, 8230-8231, 8255, 8261-8263, 8310, 8320, 8323, 8333, 8440, 8490, 8504-8505, 8508, 8512, 8525, 8530, 8551, 8562, 8573-8574, 8576, 8951, 8980, 8983 0-3 86
20. Epithelial Tumors of Corpus Uteri
20.1 Adenocarcinoma with variants of corpus uteri C54, C57.4 8050-8051, 8120, 8122-8123, 8130, 8140-8141, 8143-8144, 8147, 8190, 8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8313, 8315, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440, 8470-8471, 8480-8482, 8490, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576, 9000, 9110 0-3 87
20.2 Squamous cell carcinoma with variants of corpus uteri C54, C57.4 8052, 8070-8076, 8078, 8082-8084 0-3 88
20.3 Adenoid cystic carcinoma of corpus uteri C54, C57.4 8200 0-3 89
20.4 Clear cell adenocarcinoma, NOS C54, C57.4 8310 0-3 90
20.5 Serous (papillary) carcinoma C54, C57.4 8441, 8450, 8460 0-3 91
20.6 Mullerian mixed tumor C54, C57.4 8950-8951, 8980-8981 0-3 92
20.7 Other epithelial tumors of corpus uteri* C54, C57.4 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8030-8033 0-3 93
21. Epithelial Tumors of Cervix Uteri
21.1 Squamous cell carcinoma with variants of cervix uteri C53 8015, 8050-8052, 8070-8076, 8078, 8082-8084, 8120, 8123, 8130 0-3 94
21.2 Adenocarcinoma with variants of cervix uteri C53 8098, 8140-8141, 8143-8144, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8410, 8430, 8440-8441, 8460, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 8570-8576, 9110 0-3 95
21.3 Undifferentiated carcinoma of cervix uteri C53 8020-8022 0-3 96
21.4 Mullerian mixed tumor of cervix uteri C53 8950-8951, 8980 0-3 97
21.5 Other epithelial tumors of cervix uteri* C53 8000-8001, 8003-8005, 8010-8012, 8030-8033, 8560 0-3 98
22. Epithelial Tumors of Ovary and Falloppian Tubei
22.1 Adenocarcinoma with variants of ovary C56.9 8050, 8120, 8122-8123, 8130, 8140-8141, 8143, 8146-8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8313, 8315, 8320, 8323, 8330, 8333, 8380-8384, 8401, 8430, 8440-8441, 8450, 8452-8453, 8460-8461, 8463, 8500, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 8570-8576, 9000, 9014-9015, 9110 0-3 99
22.2 Mucinous adenocarcinoma of ovary C56.9 8470-8471, 8480-8482, 8490 0-3 100
22.3 Clear cell adenocarcinoma of ovary C56.9 8310 0-3 101
22.4 Primary peritoneal serous/papillary carcinoma C48 8050, 8260, 8441, 8450, 8460-8461 0-3 102
22.5 Mullerian mixed tumor of ovary and falloppian tube C56.9, C57.0-C57.3 8950-8951, 8980-8981 0-3 103
22.6 Adenocarcinoma with variants of fallopian tube C57.0-C57.3 8050, 8120, 8140-8141, 8143, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8460-8461, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576, 9014-9015, 9110 0-3 104
22.7 Other epithelial tumors of ovary and falloppian tube* C56.9 8000-8001, 8003-8005, 8010-8012, 8020-8022, 8030-8034, 8040, 8051-8052, 8070-8076, 8083-8084, 8340, 8410, 8474, 8560 0-3 105
23. Non Epithelial Tumors of Ovary
23.1 Sex cord tumor of ovary C56 8590, 8600, 8620, 8630-8631, 8634, 8640, 8650, 8670 0-3 106
23.2 Malignant/Immature teratoma of ovary C56 9080-9084, 9090-9091, 9102, 9391, 9440, 9473, 9501 0-3 107
23.3 Germ cell tumor of ovary C56 9060-9065, 9070-9073, 9085, 9100-9101 0-3 108
23.4 Other non epithelial tumors of ovary* C57.1 9391 0-3 109
24. Epithelial Tumors of Vulva and Vagina
24.1 Squamous cell carcinoma with variants of vulva and vagina C51, C52 8050-8052, 8070-8076, 8078, 8082-8084, 8090-8095, 8097, 8120, 8123 0-3 110
24.2 Adenocarcinoma with variants of vulva and vagina C51, C52 8098, 8102, 8140-8141, 8143, 8147, 8190, 8200-8201, 8210-8211, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8333, 8380-8384, 8390, 8400-8403, 8407-8410, 8413, 8420, 8430, 8440, 8460, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8550-8551, 8562, 8570-8576, 8982, 9110 0-3 111
24.3 Pagets disease of vulva and vagina C51, C52 8542-8543 0-3 112
24.4 Undifferentiated carcinoma of vulva and vagina C51, C52 8020-8022 0-3 113
24.5 Mullerian mixed tumor of vulva and vagina C51, C52 8950-8951, 8980 0-3 114
24.6 Other epithelial tumors of vulva and vagina* C51, C52 8000-8001, 8003-8004, 8010-8012, 8030, 8032-8033, 8144, 8560 0-3 115
25. Trophoblastic Tumors of Placenta
25.1 Choriocarcinoma of placenta C58.9 9100 0-3 116
25.2 Other trophoblastic tumors of placenta* C58.9 8000-8001, 9101-9102, 9104-9105 0-3 117
26. Epithelial Tumors of Prostate
26.1 Adenocarcinoma with variants of prostate C61 8030, 8032-8033, 8074, 8082, 8140-8141, 8143, 8145, 8190, 8210-8211, 8231, 8251, 8255, 8261-8263, 8290, 8310, 8320, 8323, 8333, 8380-8384, 8430, 8440-8441, 8450, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 8570-8576, 8941, 8980 0-3 118
26.2 Squamous cell carcinoma with variants of prostate C61 8051-8052, 8070-8073, 8075-8076, 8084, 8121, 8123, 8560 0-3 119
26.3 Infiltrating duct carcinoma of prostate C61 8050, 8201, 8230, 8260, 8500, 8503 0-3 120
26.4 Transitional cell carcinoma of prostate C61 8120, 8122, 8130-8131 0-3 121
26.5 Basal cell adenocarcinoma of prostate C61 8147, 8200 0-3 122
26.6 Other epithelial tumors of prostate* C61 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8031, 8034, 8040, 8951 0-3 123
27. Testicular and Paratesticular Cancers
27.1 Paratesticular adenocarcinoma with variants C63.0, C63.1, C63.8 8120, 8123, 8140-8141, 8147, 8190, 8200, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8333, 8380-8384, 8401, 8430, 8440-8441, 8460, 8470, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 124
27.2 Non seminomatous testicular cancer C62 9065, 9070-9072, 9080-9083, 9085, 9100-9102, 9105 0-3 125
27.3 Seminomatous testicular cancer C62 9060-9062 0-3 126
27.4 Spermatocytic seminoma C62 9063 0-3 127
27.5 Teratoma with malignant transformation C62 9084 0-3 128
27.6 Testicular sex cord cancer C62 8630-8631, 8634, 8640, 8650 0-3 129
27.7 Other testicular and paratesticular cancers* C62 8000-8001, 9064 0-3 130
28. Epithelial Tumors of Penis
28.1 Squamous cell carcinoma with variants of penis C60 8020-8022, 8050-8052, 8054, 8070-8076, 8078, 8082-8084, 8090, 8094, 8123, 8320, 8560, 8980 0-3 131
28.2 Adenocarcinoma with variants of penis C60 8140-8141, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8310, 8323, 8402, 8410, 8440, 8480-8482, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 8570-8576, 8940, 8982 0-3 132
28.3 Other epithelial tumors of penis* C60 8000-8001, 8003-8004, 8010-8012, 8031-8033, 8095, 8120, 8390, 8430 0-3 133
29. Epithelial Tumors of Kidney
29.1 Renal cell carcinoma with variants C64 8005, 8050, 8140-8141, 8143, 8147, 8190, 8200-8201, 8211, 8221, 8230-8231, 8251, 8255, 8260-8263, 8270, 8290, 8300, 8310-8312, 8315-8320, 8323, 8330, 8333, 8401, 8430, 8440, 8450, 8480-8482, 8490, 8500, 8503-8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8562, 9110 0-3 134
29.2 Squamous cell carcinoma spindle cell type of kidney C64 8004, 8032, 8074, 8980 0-3 135
29.3 Squamous cell carcinoma with variants of kidney C64 8020-8022, 8051-8052, 8070-8073, 8075-8076, 8078, 8082-8084, 8560 0-3 136
29.4 Other epithelial tumors of the kidney* C64 8000-8001, 8003, 8010-8012, 8014, 8030-8031, 8033-8035, 8123, 8210, 8280, 8380-8384, 8570-8576, 8981 0-3 137
30. Epithelial Tumors of Pelvis and Ureter
30.1 Transitional cell carcinoma of pelvis and ureter C65.9,C66.9 8020-8022, 8031, 8082, 8120-8122, 8130-8131 0-3 138
30.2 Squamous cell carcinoma with variants of pelvis and ureter C65.9,C66.9 8004, 8050-8052, 8070-8076, 8123 0-3 139
30.3 Adenocarcinoma with variants of pelvis and ureter C65.9,C66.9 8005, 8140-8141, 8143, 8147, 8190, 8200-8201, 8211, 8221, 8230-8231, 8251, 8255, 8260-8263, 8270, 8280, 8290, 8300, 8310, 8320, 8323, 8330, 8342, 8430, 8480-8482, 8490, 8500, 8510, 8512, 8514, 8525, 8542, 8560, 8570, 9110 0-3 140
30.4 Other epithelial tumors of pelvis and ureter* C65.9,C66.9 8000-8001, 8003, 8010-8012, 8014, 8030, 8032-8033, 8035, 8078, 8083-8084, 8145, 8210, 8440, 8504, 8550, 8562, 8571-8576, 8980 0-3 141
31. Epithelial Tumors of Urethra
31.1 Transitional cell carcinoma of urethra C68.0, C68.1 8020-8022, 8031, 8082, 8120-8122, 8130-8131 0-3 142
31.2 Squamous cell carcinoma with variants of urethra C68.0, C68.1 8050-8052, 8070-8076, 8094, 8123 0-3 143
31.3 Adenocarcinoma with variants of urethra C68.0, C68.1 8140-8141, 8143, 8147, 8190, 8200-8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8430, 8480-8482, 8490, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8570, 9110 0-3 144
31.4 Other epithelial tumors of urethra* C68.0, C68.1 8000-8001, 8004, 8010-8012, 8033, 8078, 8083-8084, 8380, 8440, 8504, 8562, 8571-8576, 8980 0-3 145
32. Epithelial Tumors of Bladder
32.1 Transitional cell carcinoma of bladder C67 8020-8022, 8030-8033, 8082, 8120-8122, 8130-8131, 8145 0-3 146
32.2 Squamous cell carcinoma with variants of bladder C67 8050-8052, 8070-8076, 8078, 8083-8084, 8094, 8123 0-3 147
32.3 Adenocarcinoma with variants of bladder C67 8140-8141, 8143-8144, 8147, 8190, 8201, 8210-8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8380, 8440, 8460, 8480-8482, 8490, 8510, 8512, 8514, 8525, 8542, 8560, 8570, 9110 0-3 148
32.4 Salivary gland type tumor of bladder C67 8200, 8430 0-3 149
32.5 Other epithelial tumors of bladder* C67 8000-8001, 8003-8004, 8010-8012, 8015,8034-8035, 8550-8551, 8562, 8571-8575, 8951, 8980 0-3 150
33. Epithelial Tumors of Eye and Adnexa
33.1 Squamous cell carcinoma with variants of eye and adnexa C69.0-C69.9 8020, 8050-8052, 8070-8076, 8078, 8082-8084, 8090 0-3 151
33.2 Adenocarcinoma with variants of eye and adnexa C69.0-C69.9 8140, 8147, 8190, 8200, 8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8410, 8430, 8440, 8480-8482, 8490, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576, 8941 0-3 152
33.3 Other epithelial tumors of eye and adnexa* C69.0-C69.9 8000-8001, 8004, 8010-8011, 8032, 8094, 8097, 8120-8121, 8123, 8141 0-3 153
34. Epithelial Tumors of Middle Ear
34.1 Squamous cell carcinoma with variants middle ear C30.1 8050-8052, 8070-8076, 8078, 8082-8084, 8090, 8094, 8120-8121, 8123, 8130 0-3 154
34.2 Adenocarcinoma with variants of middle ear C30.1 8140, 8147, 8190, 8200, 8211, 8221, 8230-8231, 8255, 8260-8263, 8290, 8310, 8320, 8323, 8410, 8430, 8440, 8480-8482, 8490, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8576 0-3 155
34.3 Other Adenocarcinoma with variants of middle ear* C30.1 8000-8001, 8003-8004, 8010-8012, 8020, 8022, 8032, 8141, 8201, 8401 0-3 156
35. Malignant Mesothelioma
35.1 Mesothelioma of pleura and pericardium C38 9050, 9051, 9052, 9053 0-3 157
35.2 Mesothelioma of peritoneum and tunica vaginalis C48, C63.7 9050, 9051, 9052, 9053 0-3 158
35.3 Other malignant mesothelioma* C00-C37, C39-C47, C49-C63.6, C63.8-C80 9050, 9051, 9052, 9053 0-3 159
36. Malignant Skin Melanoma
36.1 Superficial spreading melanoma C44, C63.2, C51, C60 8743 0-3 160
36.2 Nodular melanoma C44, C63.2, C51, C60 8721 0-3 161
36.3 Lentigo maligna melanoma C44, C63.2, C51, C60 8742 0-3 162
36.4 Acral lentiginous melanoma malignant C44, C63.2, C51, C60 8744 0-3 163
36.5 Other malignant skin melanoma* C44, C63.2, C51, C60 8720, 8722-8723, 8728, 8730, 8740-8741, 8745-8746, 8761, 8770-8774, 8780 0-3 164
37. Malignant Melanoma of Mucosa and Extracutaneous C00-C26, C30-C34, C52-C57, C61-C62, C64-C68, C70-C75 8720-8723, 8728, 8730, 8740-8746, 8761, 8770-8774, 8780 0-3 165
38. Malignant Melanoma of Eye
38.1 Malignant melanoma of conjunctiva C69.0 8720-8723, 8728, 8730, 8740-8746, 8761, 8770-8774, 8780 0-3 166
38.2 Malignant melanoma of uvea C69.3-C69.4 8720-8723, 8728, 8730, 8740-8746, 8761, 8770-8774, 8780 0-3 167
38.3 Other malignant melanoma of eye* C69.1-C69.2, C69.5-C69.9 8720-8723, 8728, 8730, 8740-8746, 8761, 8770-8774, 8780 0-3 168
39. Epithelial Tumors of Skin
39.1 Basal cell carcinoma of skin C44, C63.2 8090-8095, 8097-8098 0-3 169
39.2 Squamous cell carcinoma with variants of skin C44, C63.2 8011, 8032, 8050-8052, 8070-8076, 8078, 8560 0-3 170
39.3 Other epithelial tumors of skin* C44, C63.2 8980 0-3 171
40. Adnexal Carcinomas of Skin
40.1 Nodular hidradenoma, malignant C44, C63.2 8402 0-3 172
40.2 Sebaceous adenocarcinoma C44, C63.2 8410 0-3 173
40.3 Adenoid cystic carcinoma C44, C63.2 8200 0-3 174
40.4 Pagets disease extramammary C44, C63.2 8542 0-3 175
40.5 Apocrine adenocarcinoma C44, C63.2 8401 0-3 176
40.6 Mucinous adenocarcinoma C44, C63.2 8480-8481 0-3 177
40.7 Pilomatrix carcinoma C44, C63.2 8110 0-3 178
40.8 Eccrine poroma, malignant C44, C63.2 8409 0-3 179
40.9 Mixed tumor malignant, NOS C44, C63.2 8940 0-3 180
40.10 Sclerosing sweat duct carcinoma C44, C63.2 8407 0-3 181
40.11 Malignant eccrine spiradenoma C44, C63.2 8403 0-3 182
40.12 Tubular adenocarcinoma C44, C63.2 8211 0-3 183
40.13 Eccrine papillary adenocarcinoma C44, C63.2 8408 0-3 184
40.14 Other adnexal carcinomas of skin* C44, C63.2 8100, 8102, 8123, 8140-8141, 8143, 8147, 8190, 8201, 8230-8231, 8255, 8260, 8310, 8320, 8323, 8330, 8390, 8400, 8413, 8420, 8430, 8440-8441, 8450, 8470, 8490, 8500, 8504, 8510, 8514, 8520-8521, 8523, 8550, 8562, 8570-8571, 8574-8575, 8941, 8982 0-3 185
41. Neuroblastoma and Ganglioneuroblastoma C00-C69, C73-C80 9490, 9500 0-3 186
42. Nephroblastoma C64.9 8960 0-3 187
43. Embryonal Tumors of Eye
43.1 Retinoblastoma C69.2, C69.9 9510-9514 0-3 188
43.2 Medulloepithelioma C69.4 9501-9502 0-3 189
44. Hepatoblastoma C22.0 8970 0-3 190
45. Pleuropulmonary Blastoma C34, C38.4 8973 0-3 191
46. Pancreatoblastoma C25 8971 0-3 192
47. Olfactory Neuroblastoma C00-C80 9520-9523 0-3 193
48. Odontogenic Malignant Tumors
48.1 Odontogenic tumor, malignant C00-C80 9270 0-3 194
48.2 Clear cell odontogenic carcinoma C00-C80 9341 0-3 195
48.3 Ghost cell odontogenic carcinoma C00-C80 9302 0-3 196
48.4 Other odontogenic malignant tumors* C00-C80 9261, 9290, 9310, 9330, 9342 0-3 197
49. Extragonadal Germ Cell Tumors
49.1 Non seminomatous germ cell tumor C00-C55, C57, C59-C61, C63-C70, C73-C75.0, C75.2, C75.4-C80 9065, 9070-9071, 9080-9085, 9100-9102, 9105 0-3 198
49.2 Seminomatous germ cell tumor C00-C55, C57, C59-C61, C63-C70, C73-C75.0, C75.2, C75.4-C80 9060-9063 0-3 199
49.3 Germ cell tumor of Central Nervous System (CNS) C71-C72, C75.1, C75.3 9060-9065, 9070-9071, 9080-9085, 9100-9102, 9105 0-3 200
49.4 Other extragonadal germ cell tumors* C00-C55, C57, C59-C61, C63-C70, C73-C75.0, C75.2, C75.4-C80 9064 0-3 201
50. Soft Tissue Sarcoma C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C80 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8910, 8912, 8920-8921, 8930-8931, 8933-8935, 8959, 8963-8964, 8990-8991, 9020, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9251-9252, 9260, 9364-9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 202-220
C00-C06, C09-C29.9, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C43, C45-C59, C61-C63.1, C63.3-C80 8940
C49 8004
C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C55, C57-C70, C73-C80 9473
C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C70, C73-C80 9503
50.1 Soft tissue sarcoma of head and neck C00.0-C14.8, C31.0-C32.2, C32.4-32.9, C49.0, C73.9, C75.2, C75.4, C76.0 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 202
50.2 Soft tissue sarcoma of limbs C49.1-C49.2, C76.4-C76.5 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 203
50.3 Soft tissue sarcoma of superficial trunk C49.3-C49.4, C49.6, C76.1-C76.2, C76.7 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 204
50.4 Soft tissue sarcoma of mediastinum C38.1-C38.3, C38.8 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 205
50.5 Soft tissue sarcoma of heart C38.0 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 206
50.6 Soft tissue sarcoma of breast C50 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9020, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 207
50.7 Soft tissue sarcoma of uterus C53-C55 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8930-8931, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 208
50.8 Soft tissue sarcoma of paratestis C63.0-C63.7 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 209
C63.0-C63.1, C63.3-C63.7 8940
50.9 Soft tissue sarcomas of other genitourinary tract C51.0-C52.9, C56.9-C57.9, C60.0-C62.9, C63.8-C63.9, C64.9-C68.9 8710-8711, 8714, 8800-8806, 8810-8815, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8959, 8963-8964, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 210
C51.0-C52.9, C56.9-C57.9, C61.9-C62.9, C63.8-C63.9, C64.9-C68.9 8940
50.10 Soft tissue sarcoma of viscera C15-C26, C34.1-C37, C38.4, C39, C42.2 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 211
50.11 Soft tissue sarcoma of retroperitoneum and peritoneum C48.0-C48.8 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 212
50.12 Soft tissue sarcoma of pelvis C49.5, C76.3 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 213
50.13 Soft tissue sarcoma of skin C44 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 214
50.14 Soft tissue sarcoma of paraorbit C69 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 215
50.15 Soft tissue sarcoma of brain and other parts of the nervous system C47, C70-C72, C75.1, C75.3 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8933-8935, 8940, 8963, 8990-8991, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 216
50.16 Embryonal rhabdomyosarcoma of soft tissue C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C80 8910 0-3 217
50.17 Alveolar rhabdomyosarcoma of soft tissue C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C80 8920 0-3 218
50.18 Ewings sarcoma of soft tissue C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C80 9260, 9364 0-3 219
C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C55, C57-C70, C73-C80 9473
50.19 Other soft tissue sarcoma* C14.9, C27-C29, C30.2-C30.9, C33.0-C33.8, C38.5-C38.7, C38.9, C42.0-C42.1, C42.3-C43, C45-C46, C48.9, C49.7-C49.9, C56.0-C56.8, C58-C59, C64.0-C64.8, C73.0-C73.8, C74-C75.0, C75.5-C75.9, C76.6, C76.8-C80 8710-8711, 8714, 8800-8806, 8810-8815, 8825, 8830, 8832-8833, 8840, 8842, 8850-8855, 8857-8858, 8890-8891, 8894-8896, 8900-8902, 8912, 8921, 8930-8931, 8933-8935, 8959, 8963-8964, 8990-8991, 9020, 9040-9044, 9120, 9124, 9130, 9133, 9137, 9150, 9170, 9180-9183, 9185-9187, 9192-9195, 9220, 9231, 9240, 9251-9252, 9365, 9540, 9542, 9560-9561, 9571, 9580-9581 0-3 220
C00.0-C14.8, C31.0-C32.2, C32.4-32.9, C49.1-C49.2, C73.9, C75.2, C75.4, C76.0, C76.4-C76.5 8930-8931, 8959, 8964, 9020, 9251
C38.1-C38.3, C38.8, C47.0-C47.9, C49.3-C49.4, C49.6, C70.0-C72.9, C75.1, C75.3, C76.1-C76.2, C76.7 8930-8931, 8959, 8964, 9020, 9251
C15.0-C26.9, C34.1-C38.0, C38.4, C39.0-C39.9, C42.2, C48.0-C48.8, C49.5, C63.0-C63.7, C69.0-C69.9, C76.3 8930-8931, 8959, 8964, 9020, 9251
C50 8930-8931, 8959, 8964, 9181-9183, 9185-9187, 9192-9195, 9220, 9251
C53-C55 8959, 8964, 9020, 9251
C51-C52, C56.9-C57, C60-C62, C63.8-C63.9, C64.9-C68 8825, 8930-8931, 9020, 9251
C44 8930-8931, 8959, 8964, 9020, 9251
C00-C06, C09-C14.9, C27-C29.9, C30.2-C32.2, C32.4-C33.8, C38.5-C38.7, C38.9, C42.0-C42.1, C42.3-C43, C45-C46, C48.9-C49.0, C49.7-C49.9, C56.0-C56.8, C58-C59, C61.0-C61.8, C64.0-C64.8, C73-C75.0, C75.2, C75.4-C76.0, C76.6, C76.8-C80 8940
C49 8004
C00-C29, C30.2-C32.2, C32.4-C33.8, C34.1-C39, C42-C70, C73-C80 9503
51. Bone Sarcoma C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 8800-8806, 8810-8812, 8815, 8830, 8840, 8850-8855, 8890-8891, 8894-8896, 8900-8902, 8910, 8912, 8920, 9040-9044, 9120, 9124, 9130, 9133, 9150, 9170, 9180-9187, 9192-9195, 9220-9221, 9230-9231, 9240, 9242-9243, 9250, 9260, 9364, 9473, 9540, 9560-9561, 9571, 9580-9581 0-3 221-227
C40-C41 8004
C00-C80 9370-9372
51.1 Osteogenic sarcoma C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 9180-9183, 9185-9187, 9192-9195 0-3 221
51.2 Chondrogenic sarcoma C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 9220-9221, 9230-9231, 9240, 9242-9243 0-3 222
51.3 Notochordal sarcoma, chordoma C00-C80 9370-9372 0-3 223
51.4 Vascular sarcoma C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 9120, 9124, 9130, 9133, 9150, 9170 0-3 224
51.5 Ewings sarcoma C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 9260, 9364, 9473 0-3 225
51.6 Other high grade sarcomas (fibrosarcoma, malignant fibrous histiocytoma) C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 8810, 8830 0-3 226
51.7 Other bone sarcoma* C30.0-C30.1, C32.3, C33.9-C34.0, C40-C41 8800-8806, 8811-8812, 8815, 8840, 8850-8855, 8890-8891, 8894-8896, 8900-8902, 8910, 8912, 8920, 9040-9044, 9184, 9250, 9540, 9560-9561, 9571, 9580-9581 0-3 227
C40-C41 8004
52. Gastrointestinal Stromal Sarcoma All cancer sites 8936 0-3 228
53. Kaposis Sarcoma All cancer sites 9140 0-3 229
54. NET GEP
54.1 Well diff not functioning endocrine carc of pancreas and digestive tract C15-26, C48 8150, 8240-8245, 8248-8249 0-3 230
54.2 Well diff functioning endocrine carc of pancreas and digestive tract C15-26, C48 8151-8153, 8155-8157 0-3 231
54.3 Poorly differentiated endocrine carcinoma of pancreas and digestive tract C15-26, C48 8002, 8013, 8041-8045, 8246 0-3 232
54.4 Malignant mixed pancreatic endocrine and exocrine tumor C15-26 8154 0-3 233
54.5 Other NET GEP* C48 8154 0-3 234
55. NET Lung/typical and Atypical Carcinoid of the Lung C34 8240, 8241, 8242, 8243, 8244, 8245, 8249 0-3 235
56. NET Other Sites C74 8700 0-3 236-240
C00-C80 8247, 8680, 8693
C73.9 8002, 8013, 8041, 8240, 8244-8246, 8249, 8345-8347, 8510, 8512
C44.0-C44.9, C51.0-C51.9, C60.0, C60.9, C63.2 8002, 8013, 8041, 8240, 8244-8246, 8249
C00-C14, C27-C33, C35-C43, C45-C47, C49-C50, C52-C59, C61-C63.1, C63.3-C73.8, C74-C80 8002, 8013, 8041-8045, 8150-8153, 8155-8157, 8240-8246, 8248-8249
56.1 Pheochromocytoma, malignant C74 8700 0-3 236
56.2 Paraganglioma C00-C80 8680, 8693 0-3 237
56.3 Endocrine carcinoma of thyroid gland C73.9 8002, 8013, 8041, 8240, 8244-8246, 8249, 8345-8347, 8510, 8512 0-3 238
56.4 Neuroendocrine carcinoma of skin C44.0-C44.9, C51.0-C51.9, C60.0, C60.9, C63.21 8002, 8013, 8041, 8240, 8244-8246, 8249 0-3 239
C00-C80 8247
56.5 Neuroendocrine carcinoma of other sites C00-C14, C27-C33, C35-C43, C45-C47, C49-C50, C52-C59, C61-C63.1, C63.3-C73.8, C74-C80 8002, 8013, 8041-8045, 8150-8153, 8155-8157, 8240-8246, 8248-8249 0-3 240
57. Carcinomas of Pituitary Gland C75.1 8000-8001, 8010, 8012, 8020-8021, 8140-8145, 8180, 8190, 8200-8201, 8210-8211, 8214-8215, 8220-8221, 8230-8231, 8250-8255, 8260-8263, 8270, 8272, 8280-8281, 8290, 8300, 8310, 8312-8320, 8322-8323, 8330-8333, 8335, 8337, 8341-8347, 8350, 8370, 8380-8381 0-3 241-242
57.1 Pituitary carcinoma C75.1 8272 0-3 241
57.2 Other carcinomas of pituitary gland* C75.1 8000-8001, 8010, 8012, 8020-8021, 8140-8145, 8180, 8190, 8200-8201, 8210-8211, 8214-8215, 8220-8221, 8230-8231, 8250-8255, 8260-8263, 8270, 8280-8281, 8290, 8300, 8310, 8312-8320, 8322-8323, 8330-8333, 8335, 8337, 8341-8347, 8350, 8370, 8380-8381 0-3 242
58. Carcinomas of Thyroid Gland C73.9 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8030-8035, 8050-8052, 8070-8076, 8078, 8082-8084, 8140-8141, 8190, 8200-8201, 8211, 8230, 8255, 8260-8263, 8290, 8310, 8320, 8322-8323, 8330-8335, 8337, 8339-8344, 8350, 8360, 8380, 8430, 8440, 8450, 8480-8481, 8490, 8500, 8504, 8514, 8520, 8550, 8560, 8562, 8574, 8580, 8588-8589, 8941, 8980, 8982 0-3 243-250
58.1 Papillary adenocarcinoma, NOS C73.9 8050, 8260 0-3 243
58.2 Follicular carcinoma, NOS C73.9 8330, 8339 0-3 244
58.3 Undifferentiated/anaplastic carcinoma C73.9 8020-8022, 8030-8033 0-3 245
58.4 Mucoepidermoid carcinoma C73.9 8430 0-3 246
58.5 Mucinous carcinoma C73.9 8480-8481 0-3 247
58.6 Spindle cell tumor with thymus-like differentiation (SETTLE) C73.9 8588 0-3 248
58.7 Carcinoma showing thymus-like differentiation (CASTLE) C73.9 8589 0-3 249
58.8 Other carcinomas of thyroid gland* C73.9 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8034-8035, 8051-8052, 8070-8076, 8078, 8082-8084, 8140-8141, 8190, 8200-8201, 8211, 8230, 8255, 8261-8263, 8290, 8310, 8320, 8322-8323, 8331-8335, 8337, 8340-8344, 8350, 8360, 8380, 8440, 8450, 8490, 8500, 8504, 8514, 8520, 8550, 8560, 8562, 8574, 8580, 8941, 8980, 8982 0-3 250
59. Carcinomas of Parathyroid Gland C75.0 8000, 8001, 8010, 8011, 8012, 8014, 8015, 8020, 8021, 8022, 8030, 8031, 8032, 8033, 8034, 8035, 8050, 8051, 8052, 8070, 8071, 8072,8073, 8074, 8075, 8076, 8140, 8147, 8190, 8200, 8211, 8230, 8231, 8255, 8260, 8261, 8262, 8263, 8290, 8310, 8320, 8322, 8323, 8340, 8430, 8440, 8480, 8481, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550, 8551, 8560, 8562,8570, 8571, 8572, 8573, 8575, 8576, 8980 0-3 251
60. Carcinomas of Adrenal Cortex C74 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8030-8035, 8050-8052, 8070-8076, 8140, 8147, 8190, 8211, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8370, 8430, 8440, 8480-8481, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8573, 8575-8576, 8671, 8980 0-3 252-253
60.1 Adrenal cortical carcinoma C74 8370 0-3 252
60.2 Other carcinomas of adrenal cortex* C74 8000-8001, 8003-8005, 8010-8012, 8014-8015, 8020-8022, 8030-8035, 8050-8052, 8070-8076, 8140, 8147, 8190, 8211, 8230-8231, 8255, 8260-8263, 8290, 8310, 8315, 8320, 8323, 8430, 8440, 8480-8481, 8490, 8504, 8510, 8512, 8514, 8525, 8542, 8550-8551, 8560, 8562, 8570-8573, 8575-8576, 8671, 8980 0-3 253
61. Tumors of Central Nervous System (CNS) C71-C72 8000-8001, 9380-9382, 9385, 9391-9393, 9396, 9400-9401, 9410-9411, 9420, 9423-9425, 9430, 9440-9442, 9445, 9450-9451, 9460, 9505 0-3 254-261
C71-C72 9383-9384, 9394, 9412, 9421, 9506 1
C71-C72 9413, 9492-9493 0
C00-C80 9390 0-3
C70 8000-8001 0-3
C70-C72 9530, 9538-9539 0-3
C75.3 8000, 9362, 9395 0-3
61.1 Astrocytic tumors of CNS C71-C72 9380-9382, 9385, 9400-9401, 9410-9411, 9420, 9423-9425, 9430, 9440-9442, 9445 0-3 254
C71-C72 9384, 9421 1
61.2 Oligodendroglial tumors of CNS C71-C72 9450-9451, 9460 0-3 255
61.3 Ependymal tumors of CNS C71-C72 9391-9393, 9396 0-3 256
C71-C72 9383, 9394 1
61.4 Neuronal and mixed neuronal-glial tumors C71-C72 9413, 9492-9493 0 257
C71-C72 9412, 9505-9506 1
C71-C72 9505 3
61.5 Choroid plexus carcinoma of CNS C00-C80 9390 0-3 258
61.6 Malignant meningiomas C70 8000-8001 0-3 259
C70-C72 9530, 9538-9539 0-3
61.7 Tumors of the pineal gland C75.3 8000, 9362, 9395 0-3 260
61.8 Other tumors of central nervous system (CNS)* C71-C72 8000-8001 0-3 261
C71-C72 9505 0, 2
62. Embryonal Tumors of CNS C71-C72 9470-9478, 9480, 9490, 9500-9504, 9508 0-3 262-274
62.1 Medulloblastoma C71-C72 9470 0-3 262
62.2 Desmoplastic nodular medulloblastoma C71-C72 9471 0-3 263
62.3 Medulloblastoma, large cell/anaplastic C71-C72 9474 0-3 264
62.4 Medulloblastoma, WNT-activated C71-C72 9475 0-3 265
62.5 Medulloblastoma, SHH-activated and TP53-mutant C71-C72 9476 0-3 266
62.6 Medulloblastoma, non-WNT/non-SHH C71-C72 9477 0-3 267
62.7 CNS Embryonal tumor, NOSa C71-C72 9473 0-3 268
62.8 CNS ganglioneuroblastoma C71-C72 9490 0-3 269
62.9 CNS neuroblastoma C71-C72 9500 0-3 270
62.10 CNS embryonal tumor with rhabdoid features C71-C72 9508 0-3 271
62.11 Medulloepithelioma, NOS C71-C72 9501 0-3 272
62.12 Embryonal tumor with multilayered rosettes, C19MC-related/NOS C71-C72 9478 0-3 273
62.13 Other embryonal tumors of CNS* C71-C72 9472, 9480, 9502-9504 0-3 274
63 Lymphoid Diseases C00-C80 9590-9591, 9596-9597, 9650-9655, 9659, 9661-9665, 9667, 9670-9671, 9673, 9675, 9678-9680, 9684, 9687-9691, 9695, 9698-9702, 9705, 9708-9709, 9712, 9714, 9716-9719, 9724-9729, 9731-9735, 9737-9738, 9760-9762, 9764, 9811-9818, 9820, 9823, 9826-9827, 9831-9837, 9940, 9948, 9970-9971 0-3 275-287
63.1 Hodgkin lymphoma, classical C00-C80 9596, 9650-9655, 9661-9665, 9667 0-3 275
63.2 Hodgkin lymphoma nodular lymphocyte predominance C00-C80 9659 0-3 276
63.3 Precursor B/T lymphoblastic leukemia/lymphoblastic lymphoma (and Burkitt) C00-C80 9687, 9727-9729, 9811-9818, 9826, 9835-9837 0-3 277
63.4 T cutaneous lymphoma (Sezary syn, Mycosis fung) C00-C80 9700-9701, 9709, 9718 0-3 278
63.5 Other T cell lymphomas and NK cell neoplasms C00-C80 9702, 9705, 9708, 9714, 9716-9717, 9719, 9724-9726, 9827, 9831, 9834, 9948 0-3 279
63.6 Diffuse B lymphoma C00-C80 9675, 9678-9680, 9684, 9735, 9737-9738 0-3 280
63.7 Follicular B lymphoma C00-C80 9597, 9690-9691, 9695, 9698 0-3 281
63.8 Hairy cell leukemia C00-C80 9940 0-3 282
63.9 Plasmacytoma/Multiple Myeloma (and Heavy chain diseases) C00-C80 9731-9734, 9762 0-3 283
63.10 Other non Hodgkin, Mature B cell lymphoma C00-C80 9670-9671, 9689, 9699, 9712, 9761, 9764, 9823, 9971 0-3 284
63.11 Mantle cell lymphoma C00-C80 9673 0-3 285
63.12 Prolymphocytic leukemia, B cell C00-C80 9832-9833 0-3 286
63.13 Other lymphoid diseases* C00-C80 9590-9591, 9688, 9760, 9820, 9970 0-3 287
64. Acute Myeloid Leukemia and Related Precursor Neoplasms C00-C80 9801, 9805-9809, 9840, 9860-9861, 9865-9867, 9869-9874, 9891, 9895-9898, 9910-9911, 9920, 9930-9931, 9984, 9987 0-3 288-289
64.1 Acute promyelocytic leukemia (AML with t(15;17) with variants) C00-C80 9866 0-3 288
64.2 AML C00-C80 9801, 9805-9809, 9840, 9860-9861, 9865, 9867, 9869-9874, 9891, 9895-9898, 9910-9911, 9920, 9930-9931, 9984, 9987 0-3 289
65. Myeloid and Lymphoid Neoplasms C00-C80 9800, 9965, 9966,9967 0-3 290
66. Myeloproliferative Neoplasms C00-C80 9740-9742, 9863, 9875, 9950, 9960-9964, 9991-9992 0-3 291-293
66.1 Chronic myeloid leukemia C00-C80 9863, 9875 0-3 291
66.2 Other myeloproliferative neoplasms C00-C80 9950, 9960-9964, 9991-9992 0-3 292
66.3 Mast cell tumor C00-C80 9740-9742 0-3 293
67. Myelodysplastic Syndrome and Myelodysplastic/myeloproliferative Diseases C00-C80 9876, 9945-9946, 9975, 9980, 9982-9983, 9985-9986, 9989 0-3 294-298
67.1 Myelodysplastic syndrome with 5q syndrome C00-C80 9986 0-3 294
67.2 Other myelodysplastic syndrome C00-C80 9980, 9982-9983, 9985, 9989 0-3 295
67.3 Chronic Myelomonocytic leukemia C00-C80 9945-9946 0-3 296
67.4 Atypical chronic myeloid leukemia BCR/ABL negative C00-C80 9876 0-3 297
67.5 Other myelodysplastic syn and myelodysplastic/myeloproliferative diseases* C00-C80 9975 0-3 298
68. Histiocytic and Dendritic Cell Neoplasms C00-C80 9750-9751, 9753-9759 0-3 299-300
68.1 Histiocytic malignancies C00-C80 9750-9751, 9753-9754, 9756 0-3 299
68.2 Lymph node accessory cell tumors C00-C80 9755, 9757-9759 0-3 300
69. Not Classified       999

(1)Gatta G, Capocaccia R, Botta L, Mallone S, De Angelis R, Ardanaz E, et al. Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncology. 2017;18(8):1022-39.
(2)Botta L, Gatta G, Trama A, Bernasconi A, Sharon E, Capocaccia R, et al. Incidence and survival of rare cancers in the US and Europe. Cancer Medicine. 2020;9(15):5632-42.
* NOS category, not a clinical entity.

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