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Papillary thyroid carcinoma(TPC; PTC; PACT)

MedGen UID:
66773
Concept ID:
C0238463
Neoplastic Process
Synonyms: Familial Papillary Thyroid Carcinoma; NONMEDULLARY THYROID CARCINOMA, PAPILLARY; Thyroid carcinoma, papillary, somatic; Thyroid gland papillary carcinoma
SNOMED CT: PTC - papillary thyroid carcinoma (255029007); Papillary thyroid carcinoma (255029007)
 
HPO: HP:0002895
Monarch Initiative: MONDO:0005075
OMIM®: 188550

Definition

The presence of a papillary adenocarcinoma of the thyroid gland. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary thyroid carcinoma
Follow this link to review classifications for Papillary thyroid carcinoma in Orphanet.

Conditions with this feature

Euthyroid goiter
MedGen UID:
86230
Concept ID:
C0302859
Disease or Syndrome
DICER1 tumor predisposition (DICER1) is characterized by an increased risk for pleuropulmonary blastoma (PPB), pulmonary cysts, thyroid gland neoplasia (multinodular goiter, adenomas, and/or thyroid cancer), ovarian tumors (Sertoli-Leydig cell tumor, gynandroblastoma, and sarcoma), and cystic nephroma. Less commonly observed tumors include ciliary body medulloepithelioma, nasal chondromesenchymal hamartoma, embryonal rhabdomyosarcoma, pituitary blastoma, pineoblastoma, central nervous system (CNS) sarcoma, other CNS tumors, and presacral malignant teratoid tumor. The majority of tumors occur in individuals younger than age 40 years. PPB typically presents in infants and children younger than age six years. Ovarian sex cord-stromal tumors are most often diagnosed before age 40 years. Cystic nephroma generally presents in young children but has also been reported in adolescents. Additional clinical features that may be seen include macrocephaly, ocular abnormalities, structural anomalies of the kidney and collecting system, and dental anomalies (bulbous crowns).
Alagille syndrome due to a JAG1 point mutation
MedGen UID:
365434
Concept ID:
C1956125
Disease or Syndrome
Alagille syndrome (ALGS) is a multisystem disorder with a wide spectrum of clinical variability; this variability is seen even among individuals from the same family. The major clinical manifestations of ALGS are bile duct paucity on liver biopsy, cholestasis, congenital cardiac defects (primarily involving the pulmonary arteries), butterfly vertebrae, ophthalmologic abnormalities (most commonly posterior embryotoxon), and characteristic facial features. Renal abnormalities, growth failure, developmental delays, splenomegaly, and vascular abnormalities may also occur.
Familial adenomatous polyposis 1
MedGen UID:
398651
Concept ID:
C2713442
Disease or Syndrome
APC-associated polyposis conditions include (classic or attenuated) familial adenomatous polyposis (FAP) and gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). FAP is a colorectal cancer (CRC) predisposition syndrome that can manifest in either classic or attenuated form. Classic FAP is characterized by hundreds to thousands of adenomatous colonic polyps, beginning on average at age 16 years (range 7-36 years). For those with the classic form of FAP, 95% of individuals have polyps by age 35 years; CRC is inevitable without colectomy. The mean age of CRC diagnosis in untreated individuals is 39 years (range 34-43 years). The attenuated form is characterized by multiple colonic polyps (average of 30), more proximally located polyps, and a diagnosis of CRC at a later age than in classic FAP. For those with an attenuated form, there is a 70% lifetime risk of CRC and the mean age of diagnosis is 50-55 years. Extracolonic manifestations are variably present and include polyps of the stomach and duodenum, osteomas, dental abnormalities, congenital hypertrophy of the retinal pigment epithelium (CHRPE), benign cutaneous lesions, desmoid tumors, adrenal masses, and other associated cancers. GAPPS is characterized by proximal gastric polyposis, increased risk of gastric adenocarcinoma, and no duodenal or colonic involvement in most individuals reported.
Cowden syndrome 7
MedGen UID:
908796
Concept ID:
C4225179
Disease or Syndrome
Cowden syndrome is a genetic disorder characterized by multiple noncancerous, tumor-like growths called hamartomas and an increased risk of developing certain cancers.\n\nAlmost everyone with Cowden syndrome develops hamartomas. These growths are most commonly found on the skin and mucous membranes (such as the lining of the mouth and nose), but they can also occur in the intestine and other parts of the body. The growth of hamartomas on the skin and mucous membranes typically becomes apparent by a person's late twenties.\n\nCowden syndrome is associated with an increased risk of developing several types of cancer, particularly cancers of the breast, a gland in the lower neck called the thyroid, and the lining of the uterus (the endometrium). Other cancers that have been identified in people with Cowden syndrome include kidney cancer, colorectal cancer, and an agressive form of skin cancer called melanoma. Compared with the general population, people with Cowden syndrome develop these cancers at younger ages, often beginning in their thirties or forties. People with Cowden syndrome are also more likely to develop more than one cancer during their lifetimes compared to the general population. Other diseases of the breast, thyroid, and endometrium are also common in Cowden syndrome. Additional signs and symptoms can include an enlarged head (macrocephaly) and a rare, noncancerous brain tumor called Lhermitte-Duclos disease. A small percentage of affected individuals have delayed development, intellectual disability, or autism spectrum disorder, which can affect communication and social interaction.\n\nSome people do not meet the strict criteria for a clinical diagnosis of Cowden syndrome, but they have some of the characteristic features of the condition, particularly the cancers. These individuals are often described as having Cowden-like syndrome. Both Cowden syndrome and Cowden-like syndrome are caused by mutations in the same genes.\n\nThe features of Cowden syndrome overlap with those of another disorder called Bannayan-Riley-Ruvalcaba syndrome. People with Bannayan-Riley-Ruvalcaba syndrome also develop hamartomas and other noncancerous tumors.  Some people with Cowden syndrome have relatives diagnosed with Bannayan-Riley-Ruvalcaba syndrome, and other affected individuals have the characteristic features of both conditions. Based on these similarities, researchers have proposed that Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome represent a spectrum of overlapping features known as PTEN hamartoma tumor syndrome (named for the genetic cause of the conditions) instead of two distinct conditions.\n\n
Thyroid cancer, nonmedullary, 4
MedGen UID:
907624
Concept ID:
C4225293
Neoplastic Process
Nonmedullary thyroid cancer (NMTC) refers to neoplasms originating from the thyroid follicular cells and represents 80 to 95% of all thyroid cancers. Approximately 5% of NMTC occurs on the background of a familial predisposition. Although papillary thyroid carcinoma (PTC) is usually the most frequent thyroid lesion in NMTC families, multinodular goiter (MNG) and follicular thyroid adenoma also occur (summary by Pereira et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of NMTC, see NMTC1 (188550).
Thyroid cancer, nonmedullary, 2
MedGen UID:
904175
Concept ID:
C4225426
Neoplastic Process
Nonmedullary thyroid cancer (NMTC) comprises thyroid cancers of follicular cell origin and accounts for more than 95% of all thyroid cancer cases. The remaining cancers originate from parafollicular cells (medullary thyroid cancer, MTC; 155240). NMTC is classified into 4 groups: papillary, follicular, Hurthle cell (607464), and anaplastic. Approximately 5% of NMTC is hereditary, occurring as a minor component of a familial cancer syndrome (e.g., familial adenomatous polyposis, 175100, Carney complex, 160980) or as a primary feature (familial NMTC or FNMTC). Papillary thyroid cancer (PTC) is the most common histologic subtype of FNMTC, accounting for approximately 85% of cases (summary by Vriens et al., 2009). Follicular thyroid cancer (FTC) accounts for approximately 15% of NMTC and is defined by invasive features that result in infiltration of blood vessels and/or full penetration of the tumor capsule, in the absence of the nuclear alterations that characterize papillary carcinoma. FTC is rarely multifocal and usually does not metastasize to the regional lymph nodes but tends to spread via the bloodstream to the lung and bones. An important histologic variant of FTC is the oncocytic (Hurthle cell, oxyphilic) follicular carcinoma composed of eosinophilic cells replete with mitochondria (summary by Bonora et al., 2010). For a general phenotypic description and a discussion of genetic heterogeneity of NMTC, see NMTC1 (188550).
Thyroid cancer, nonmedullary, 1
MedGen UID:
1648293
Concept ID:
C4721429
Neoplastic Process
Nonmedullary thyroid cancer (NMTC) comprises thyroid cancers of follicular cell origin and accounts for more than 95% of all thyroid cancer cases. The remaining cancers originate from parafollicular cells (medullary thyroid cancer, MTC; 155240). NMTC is classified into 4 groups: papillary, follicular (188470), Hurthle cell (607464), and anaplastic. Approximately 5% of NMTC is hereditary, occurring as a component of a familial cancer syndrome (e.g., familial adenomatous polyposis, 175100; Carney complex, 160980) or as a primary feature (familial NMTC or FNMTC). Papillary thyroid cancer (PTC) is the most common histologic subtype of FNMTC, accounting for approximately 85% of cases (summary by Vriens et al., 2009). PTC is characterized by distinctive nuclear alterations including pseudoinclusions, grooves, and chromatin clearing. PTCs smaller than 1 cm are referred to as papillary microcarcinomas. These tumors have been identified in up to 35% of individuals at autopsy, suggesting that they may be extremely common although rarely clinically relevant. PTC can also be multifocal but is typically slow-growing with a tendency to spread to lymph nodes and usually has an excellent prognosis (summary by Bonora et al., 2010). Genetic Heterogeneity of Susceptibility to Nonmedullary Thyroid Cancer Other susceptibilities to nonmedullary thyroid cancer include NMTC2 (188470), caused by mutation in the SRGAP1 gene (606523); NMTC3 (606240), mapped to chromosome 2q21; NMTC4 (616534), caused by mutation in the FOXE1 gene (602617); and NMTC5 (616535), caused by mutation in the HABP2 gene (603924). A susceptibility locus for familial nonmedullary thyroid carcinoma with or without cell oxyphilia (TCO; 603386) has been mapped to chromosome 19p.

Professional guidelines

PubMed

Gordon AJ, Dublin JC, Patel E, Papazian M, Chow MS, Persky MJ, Jacobson AS, Patel KN, Suh I, Morris LGT, Givi B
JAMA Otolaryngol Head Neck Surg 2022 Dec 1;148(12):1156-1163. doi: 10.1001/jamaoto.2022.3360. PMID: 36326739Free PMC Article
Haddad RI, Bischoff L, Ball D, Bernet V, Blomain E, Busaidy NL, Campbell M, Dickson P, Duh QY, Ehya H, Goldner WS, Guo T, Haymart M, Holt S, Hunt JP, Iagaru A, Kandeel F, Lamonica DM, Mandel S, Markovina S, McIver B, Raeburn CD, Rezaee R, Ridge JA, Roth MY, Scheri RP, Shah JP, Sipos JA, Sippel R, Sturgeon C, Wang TN, Wirth LJ, Wong RJ, Yeh M, Cassara CJ, Darlow S
J Natl Compr Canc Netw 2022 Aug;20(8):925-951. doi: 10.6004/jnccn.2022.0040. PMID: 35948029
Stan MN, Papaleontiou M, Schmitz JJ, Castro MR
J Clin Endocrinol Metab 2022 Apr 19;107(5):1417-1430. doi: 10.1210/clinem/dgab917. PMID: 34953163Free PMC Article

Curated

UK NICE Guideline NG230, Thyroid cancer: assessment and management

Recent clinical studies

Etiology

Chan WWL, Kwong DLW
Methods Mol Biol 2022;2534:225-241. doi: 10.1007/978-1-0716-2505-7_16. PMID: 35670979
Lo CY
Methods Mol Biol 2022;2534:39-56. doi: 10.1007/978-1-0716-2505-7_4. PMID: 35670967
Lam AK
Methods Mol Biol 2022;2534:1-15. doi: 10.1007/978-1-0716-2505-7_1. PMID: 35670964
Xu J, Ding K, Mu L, Huang J, Ye F, Peng Y, Guo C, Ren C
Front Endocrinol (Lausanne) 2022;13:801925. Epub 2022 Feb 24 doi: 10.3389/fendo.2022.801925. PMID: 35282434Free PMC Article
Mao J, Zhang Q, Zhang H, Zheng K, Wang R, Wang G
Front Endocrinol (Lausanne) 2020;11:265. Epub 2020 May 15 doi: 10.3389/fendo.2020.00265. PMID: 32477264Free PMC Article

Diagnosis

Viola N, Agate L, Caprio S, Lorusso L, Brancatella A, Ricci D, Sgrò D, Ugolini C, Piaggi P, Vitti P, Elisei R, Santini F, Latrofa F
Endocr Relat Cancer 2023 Jul 1;30(7) Epub 2023 Jun 2 doi: 10.1530/ERC-23-0042. PMID: 37043372
Volpi EM, Ramirez-Ortega MC, Carrillo JF
Front Endocrinol (Lausanne) 2023;14:1163309. Epub 2023 Mar 7 doi: 10.3389/fendo.2023.1163309. PMID: 36960399Free PMC Article
Lo CY
Methods Mol Biol 2022;2534:39-56. doi: 10.1007/978-1-0716-2505-7_4. PMID: 35670967
Lam AK
Methods Mol Biol 2022;2534:1-15. doi: 10.1007/978-1-0716-2505-7_1. PMID: 35670964
Lloyd RV, Buehler D, Khanafshar E
Head Neck Pathol 2011 Mar;5(1):51-6. Epub 2011 Jan 8 doi: 10.1007/s12105-010-0236-9. PMID: 21221869Free PMC Article

Therapy

Chan WWL, Chan S, Kwong DLW
Methods Mol Biol 2022;2534:243-257. doi: 10.1007/978-1-0716-2505-7_17. PMID: 35670980
Chan WWL, Kwong DLW
Methods Mol Biol 2022;2534:225-241. doi: 10.1007/978-1-0716-2505-7_16. PMID: 35670979
Waguespack SG, Drilon A, Lin JJ, Brose MS, McDermott R, Almubarak M, Bauman J, Casanova M, Krishnamurthy A, Kummar S, Leyvraz S, Oh DY, Park K, Sohal D, Sherman E, Norenberg R, Silvertown JD, Brega N, Hong DS, Cabanillas ME
Eur J Endocrinol 2022 Apr 29;186(6):631-643. doi: 10.1530/EJE-21-1259. PMID: 35333737Free PMC Article
Stan MN, Papaleontiou M, Schmitz JJ, Castro MR
J Clin Endocrinol Metab 2022 Apr 19;107(5):1417-1430. doi: 10.1210/clinem/dgab917. PMID: 34953163Free PMC Article
Miccoli P, Bakkar S
Updates Surg 2017 Jun;69(2):145-150. Epub 2017 Apr 12 doi: 10.1007/s13304-017-0449-5. PMID: 28405952

Prognosis

Akgun E, Sager S, Beytur F, Nazari A, Ozturk T, Teksoz S, Sonmezoglu K
Indian J Cancer 2023 Oct 1;60(4):556-561. Epub 2023 Jun 9 doi: 10.4103/ijc.IJC_9_21. PMID: 38090963
Moleti M, Aversa T, Crisafulli S, Trifirò G, Corica D, Pepe G, Cannavò L, Di Mauro M, Paola G, Fontana A, Calapai F, Cannavò S, Wasniewska M
Front Endocrinol (Lausanne) 2023;14:1270518. Epub 2023 Sep 19 doi: 10.3389/fendo.2023.1270518. PMID: 37795368Free PMC Article
Megwalu UC, Moon PK
Thyroid 2022 May;32(5):560-570. Epub 2022 Mar 15 doi: 10.1089/thy.2021.0662. PMID: 35132899
Bergdorf K, Ferguson DC, Mehrad M, Ely K, Stricker T, Weiss VL
Endocr Relat Cancer 2019 Jun;26(6):601-614. doi: 10.1530/ERC-19-0074. PMID: 30965283Free PMC Article
Hay ID
Endocrinol Metab Clin North Am 1990 Sep;19(3):545-76. PMID: 2261906

Clinical prediction guides

Volpi EM, Ramirez-Ortega MC, Carrillo JF
Front Endocrinol (Lausanne) 2023;14:1163309. Epub 2023 Mar 7 doi: 10.3389/fendo.2023.1163309. PMID: 36960399Free PMC Article
Zhu L, Zhang X, Zhang S, Zhang Q, Cao L, Zhang Y, Wang D, Liang X, Wu W, Wu S, Jiang R, Liu Y, Zhao X, Zhou G, Xu K, Meng Z
Clin Exp Med 2023 Oct;23(6):2209-2220. Epub 2023 Jan 30 doi: 10.1007/s10238-023-00998-2. PMID: 36715834
Lam AK
Methods Mol Biol 2022;2534:93-108. doi: 10.1007/978-1-0716-2505-7_7. PMID: 35670970
Lo CY
Methods Mol Biol 2022;2534:39-56. doi: 10.1007/978-1-0716-2505-7_4. PMID: 35670967
Wang T, Shi J, Li L, Zhou X, Zhang H, Zhang X, Wang Y, Liu L, Sheng L
Front Immunol 2022;13:840811. Epub 2022 Apr 20 doi: 10.3389/fimmu.2022.840811. PMID: 35515000Free PMC Article

Recent systematic reviews

Moleti M, Aversa T, Crisafulli S, Trifirò G, Corica D, Pepe G, Cannavò L, Di Mauro M, Paola G, Fontana A, Calapai F, Cannavò S, Wasniewska M
Front Endocrinol (Lausanne) 2023;14:1270518. Epub 2023 Sep 19 doi: 10.3389/fendo.2023.1270518. PMID: 37795368Free PMC Article
Gao X, Yang Y, Wang Y, Huang Y
Int J Hyperthermia 2023 Dec;40(1):2244713. doi: 10.1080/02656736.2023.2244713. PMID: 37604507
Wei X, Wang X, Xiong J, Li C, Liao Y, Zhu Y, Mao J
Biomed Res Int 2022;2022:9959649. Epub 2022 May 18 doi: 10.1155/2022/9959649. PMID: 35647194Free PMC Article
Donaldson LB, Yan F, Morgan PF, Kaczmar JM, Fernandes JK, Nguyen SA, Jester RL, Day TA
Endocrine 2021 Apr;72(1):27-39. Epub 2020 Oct 6 doi: 10.1007/s12020-020-02505-z. PMID: 33025563Free PMC Article
Mao J, Zhang Q, Zhang H, Zheng K, Wang R, Wang G
Front Endocrinol (Lausanne) 2020;11:265. Epub 2020 May 15 doi: 10.3389/fendo.2020.00265. PMID: 32477264Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2022
      UK NICE Guideline NG230, Thyroid cancer: assessment and management

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