2018 ICD-O-3 Updates in Table Format with Annotation for Reference

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1 Status Histology Description (this may be preferred term or a synonym) Report Comments New term Urachal carcinoma (C65.9, C66.9, C67._, C68._) New term Combined large cell neuroendocrine carcinoma (C34._, C37.9) Midline carcinoma of children and young adults with NUT rearrangement (C30.0, C31.9, C34._) NUT carcinoma (C30.0, C31.9, C34._) NUT midline carcinoma (C30.0, C31.9, C34._) New term High-grade neuroendocrine carcinoma (C54._, C55.9) New term Neuroendocrine carcinoma, poorly differentiated (C50._) New term Small cell carcinoma pulmonary type (C56.9) New term Small cell carcinoma, hypercalcemic type (C56.9) Condylomatous carcinoma (C60.0-C60.2, C60.9) 8051/3 All other sites use 8051/ forward Warty carcinoma (C60.0-C60.2, C60.9) 8051/3 All other sites use 8051/ forward /term /term Differentiated penile intraepithelial neoplasia (C60._) N Not reportable for 2018 cases Differentiated-type vulvar intraepithelial neoplasia (C51._) N Not reportable for 2018 cases Squamous cell carcinoma, HPV-positive (C01.9, C10.2, C10.3, C10.8, C10.9, C31.0 C31.3, C31.9) Squamous cell carcinoma, HPV-negative (C01.9, C10.2, C10.3, C10.8, C10.9, C31.0 C31.3, C31.9) Lipid-rich urothelial carcinoma (C65.9, C66.9, C67._, C68._) Microcystic urothelial carcinoma (C65.9, C66.9, C67._, C68._) Nested urothelial carcinoma (C65.9, C66.9, C67._, C68._) Squamotransitional cell carcinoma (C53._) Urothelial carcinoma with divergent differentiation (C65.9, C66.9, C67._, C68._) Urothelial carcinoma with squamous differentiation (C65.9, C66.9, C67._, C68._) Urothelial carcinoma with trophoblastic differentiation (C65.9, C66.9, C67._, C68._) Clear cell (glycogen-rich) urothelial carcinoma (C65.9, C66.9, C67._, C68._) New term Minimally invasive adenocarcinoma, NOS (C34._) New term Endocervical adenocarcinoma usual type (C53._) New term Acinar adenocarcinoma (C61.9 ONL) For prostate only, do not use 8550/3 New term Enteric adenocarcinoma (C34. 0, C65.9, C66.9, C67._, C68._) New term Intestinal-type adenocarcinoma (C30.0, C53._) New term Mucinous carcinoma, intestinal type (C53._)

2 Status Histology Description (this may be preferred term or a synonym) Report Comments ACTH-producing tumor N Endocrine tumor, functioning, NOS N Adenocarcinoma, pancreatobiliary-type (C24.1) 8255/ Pancreatobiliary-type carcinoma (C24.1) 8255/3 New term Thymic carcinoma with adenoid cystic carcinoma-like features (C37.9) /term Serrated adenocarcinoma (C18.0, C18.2, C18.9, C19.9, C20.9) New term Neuroendocrine tumor, well differentiated (C50._) /term Adenocarcinoma in situ, non-mucinous (C34._) New term Lepidic adenocarcinoma (C34._) New term Lepidic predominant adenocarcinoma (C34._) /term Adenocarcinoma in situ, mucinous (C34._) Important note: lung primaries ONL: For cases diagnosed 1/1/2018 forward do not use 8480 (mucinous adenocarcinoma) for in-situ adenocarcinoma, mucinous or invasive mucinous adenocarcinoma. New term Invasive mucinous adenocarcinoma (C34._) Important note: lung primaries ONL: For cases diagnosed 1/1/2018 forward do not use 8480 (mucinous adenocarcinoma) for in-situ adenocarcinoma, mucinous or invasive mucinous adenocarcinoma. New term Mixed invasive mucinous and non-mucinous adenocarcinoma (C34._) Minimally invasive adenocarcinoma, non-mucinous (C34._) Minimally invasive adenocarcinoma, mucinous (C34._) New term Endometrioid adenocarcinoma, villoglandular (C54._, C55.9) New term Villoglandular carcinoma (C53._) Micropapillary carcinoma, NOS (C18._, C19.9, C20.9, C34._) 8507/3. Code 8265 is not valid for C50._. Use 8507 for micropapillary adenocarcinoma in breast primaries Micropapillary adenocarcinoma (C34._) 8507/3. Code 8265 is not valid for C50._. Use 8507 for micropapillary adenocarcinoma in breast primaries /term /term Hereditary leiomyomatosis & RCC- associated renal cell carcinoma (C64.9) MiT family translocation renal cell carcinoma (C64.9)

3 Status Histology Description (this may be preferred term or a synonym) Report Comments New term Renal cell carcinoma, unclassified (C64.9) New term Acquired cystic disease-associated renal cell carcinoma (RCC) (C64.9) New term Tubulocystic renal cell carcinoma (C64.9) Follicular thyroid carcinoma (FTC), encapsulated angoinvasive (C73.9) New term Non-invasive EFVPTC (C73.9) New term Non-invasive encapsulated follicular variant of papillary thyroid carcinoma (non-invasive EFVPTC) (C73.9) New term Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) (C73.9) New term Non-invasive FTP (C73.9) New term Encapsulated follicular variant of papillary thyroid carcinoma, NOS (EFVPTC, NOS) (C73.9) New term Invasive encapsulated follicular variant of papillary thyroid carcinoma (invasive EFVPTC) (C73.9) /term Atypical hyperplasia/endometrioid intraepithelial neoplasm (C54._) N Not reportable for 2018 cases /term /term Serous endometrial intraepithelial carcinoma (C54._, C55.9) Serous tubal intraepithelial carcinoma (C57.0) New term Intraductal papillary mucinous neoplasm with high-grade dysplasia (C25._) New term /term /term New term Intraductal papillary mucinous neoplasm (IPMN) with an associated invasive carcinoma (C25._) Non-invasive low grade serous carcinoma (C56.9) Serous borderline tumor-micropapillary variant (C56.9) N Not reportable for 2018 cases Low-grade serous carcinoma (C56.9, C48._, C57.0, C57.1 C57.3) New term High-grade serous carcinoma (C48._, C56.9, C57.0, C57.1 C57.3) New term Mucinous cystic tumor with associated invasive carcinoma (C25._) Seromucinous carcinoma (C56.9) Low grade appendiceal mucinous neoplasm (C18.1) N New term Mucinous tubular and spindle cell carcinoma (C64.9) New term Mucinous carcinoma, gastric type (C53._) New term Low grade cribriform cystadenocarcinoma (LGCCC) (C06.9, C08.9) New term Mammary carcinoma, in situ (C50._) New term Non-invasive mammary carcinoma (C50._) New term Salivary duct carcinoma (C06.9, C08.9) New term Invasive carcinoma of no special type (C50._) New term Invasive carcinoma, NST (C50._)

4 Status Histology Description (this may be preferred term or a synonym) Report Comments New term Invasive mammary carcinoma (C50._) New term Intraductal papilloma with ductal carcinoma in situ (C50._) New term Intraductal tubulopapillary neoplasm (C25._) New term Intracystic papillary neoplasm with associated invasive carcinoma New term Encapsulated papillary carcinoma C50._) New term Encapsulated papillary carcinoma with invasion (C50._) /term Invasive micropapillary carcinoma (C50._) Solid papillary carcinoma in situ (C50._) Solid papillary carcinoma with invasion (C50._) New term Renal medullary carcinoma (C64.9) For all sites other than C50._, see 8265/ Pleomorphic lobular carcinoma in situ (C50._) ICD-O-3 rule F DOES NOT APPL to Invasive pleomorphic lobular carcinoma is d 8520/3 New term Intraductal papilloma with lobular carcinoma in situ (C50._) New term Invasive lobular carcinoma, alveolar type (C50._) New term Invasive lobular carcinoma, solid type (C50._) New term Invasive lobular carcinoma, tubulolobular variant (C50._) New term Pleomorphic lobular carcinoma (C50._) New term Invasive lobular carcinoma (C50._) New term Tubulolobular carcinoma (C50._) New term Acinar cell carcinoma Excludes C61.9-see 8140/3 New term Acinar adenocarcinoma (C34._) Lung primaries diagnosed prior to 1/1/2018 use 8550/3; for prostate (all years) see 8140/ Mixed acinar ductal carcinoma 8523/3 New term Endometrioid carcinoma with squamous differentiation (C54._, C55.9) New term Low grade adenosquamous carcinoma (C50._) New term Carcinoma with chondroid differentiation (C50._) New term Carcinoma with osseous differentiation (C50._) New term Metaplastic carcinoma with chondroid differentiation (C50._) New term Metaplastic carcinoma with osseous differentiation (C50._) New term Acinar adenocarcinoma, sarcomatoid (C61.9) New term Fibromatosis-like metaplastic carcinoma (C50._) New term New term Adenocarcinoma admixed with neuroendocrine carcinoma (C53._) Carcinoma with other types mesenchymal differentiation (C50._)

5 Status Histology Description (this may be preferred term or a synonym) Report Comments New term Metaplastic carcinoma with other types mesenchymal differentiation (C50._) New term Metaplastic carcinoma of no special type (C50._) /term Mixed germ cell sex cord-stromal tumor, unclassified (C48.2, C56.9, C57.9) Adult granulosa cell tumor (C56.9 ONL) N Not reportable for 2018 cases Malignant perivascular epithelial cell tumor PEComa, malignant Perivascular epithelioid cell tumor, malignant New term Meningeal melanoma (C70._, C71._) New term Undifferentiated spindle cell sarcoma New term Undifferentiated pleomorphic sarcoma New term Undifferentiated round cell sarcoma New term Undifferentiated epithelioid sarcoma New term Undifferentiated uterine sarcoma /term New term Myxoinflammatory fibroblastic sarcoma (MIFS) (C49._) N Solitary fibrous tumor/hemangiopericytoma Grade 1 (CNS) N Reportable for CNS New term/behavior Solitary fibrous tumor/hemangiopericytoma Grade 2 (CNS) Reportable for CNS ONL New term Solitary fibrous tumor/hemangiopericytoma Grade 3 (CNS) /term /term Low-grade myofibroblastic sarcoma ( C01.9, C02._, C06.9, C49._) Myofibroblastic sarcoma New term Malignant fibrous histiocytoma (MFH) of bone New term Undifferentiated high-grade pleomorphic sarcoma New term Fibrosarcomatous dermatofibrosarcoma protuberans New term Low-grade fibromyxoid sarcoma New term Sclerosing epithelioid fibrosarcoma /term /term Ossifying fibromyxoid tumor, malignant (C49._) Pulmonary myxoid sarcoma with EWSR1-CREB1 translocation (C34._) New term Sclerosing rhabdomyosarcoma New term Mullerian adenosarcoma (C54._, C55.9) /term Calcifying nested epithelial stromal tumor (C22.0) N Adenomyoepithelioma with carcinoma (C50._) New term Phosphaturic mesenchymal tumor, malignant New term Periductal stromal tumor, low grade (C50._) Biphenotypic sinonasal sarcoma (C30.0, C31.0-C31.3, C31.8, C31.9) Germ cell tumors with associated hematological malignancy (C37.9)

6 Status Histology Description (this may be preferred term or a synonym) Report Comments New term Adenocarcinoma of rete ovarii (C56.9) /term Epithelioid hemangioendothelioma Intimal sarcoma Pulmonary artery intimal sarcoma New term Low-grade central osteosarcoma (C40._, C41._) New term Low-grade intramedullary osteosarcoma (C40._, C41._) New term Chondrosarcoma grade 2/3 /term /term Ghost cell odontogenic carcinoma (C41.0, C41.1) Clear cell odontogenic carcinoma (C41.0, C41.1) New term Anaplastic oligoastrocytoma New term Oligoastrocytoma, NOS Diffuse midline glioma, H3 K27M-mutant Papillary tumor of pineal region (C75.3) Ependymoma, RELA fusion-positive New term Diffuse astrocytoma, IDH-mutant New term Diffuse astrocytoma, IDH-wildtype New term Anaplastic astrocytoma, IDH-mutant New term Anaplastic astrocytoma, IDH-wildtype New term Anaplastic pleomorphic xanthroastrocytoma 9361/ Pilomyxoid astrocytoma Angiocentric glioma Pituicytoma (C75.1) 9421/3 9380/1 9380/1 New term Epithelioid glioblastoma New term Glioblastoma, IDH wildtype New term Glioblastoma, IDH-mutant Oligodendroglioma, IDH-mutant and 1p/19q-leted New term Anaplastic oligodendroglioma, IDH-mutant and 1p/19q leted New term Medulloblastoma, classic New term Medulloblastoma, SHH-activated and TP53-wildtype Medulloblastoma, WNT-activated Medulloblastoma, SHH-activated and TP53 mutant Medulloblastoma, group Medulloblastoma, group Medulloblastoma, non-wnt/non-shh

7 Status Histology Description (this may be preferred term or a synonym) Report Comments Embryonal tumor with multilayered rosettes C19MC-altered Embryonal tumor with multilayered rosettes, NOS New term CNS Embryonal tumor with rhabdoid features New term Embryonal tumor with rhabdoid features /term Diffuse leptomeningeal glioneuronal tumor Papillary glioneuronal tumor Rosette-forming glioneuronal tumor Epithelioid malignant peripheral nerve sheath tumor (C47.0- C47.6, C47.8, C47.9) Melanotic schwannoma (C72.4, C72.5) Indolent systemic mastocytosis N 9505/1 9505/1 9505/1

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