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Papillary renal cell carcinoma(RCCP)

MedGen UID:
266300
Concept ID:
C1306837
Neoplastic Process
Synonyms: Papillary renal carcinoma, malignant - (subtype); Papillary renal cell carcinoma, bilateral - (subtype); Papillary renal cell carcinoma, familial - (subtype); Papillary renal cell carcinoma, multiple - (subtype); Papillary renal cell carcinoma, sporadic - (subtype); RCCP
SNOMED CT: Papillary renal cell carcinoma (733607005); Papillary renal cell carcinoma (733608000)
 
HPO: HP:0006766
Monarch Initiative: MONDO:0017884
Orphanet: ORPHA319298

Definition

The presence of renal cell carcinoma in the renal papilla. [from HPO]

Conditions with this feature

Papillary renal cell carcinoma type 1
MedGen UID:
237026
Concept ID:
C1336839
Neoplastic Process
Hereditary papillary renal cell carcinoma is characterized by the development of multiple, bilateral papillary renal tumors (Zbar et al., 1995). The transmission pattern is consistent with autosomal dominant inheritance with incomplete penetrance. Papillary renal cell carcinoma is histologically and genetically distinct from 2 other forms of inherited renal carcinoma, von Hippel Lindau disease (193300), caused by mutation in the VHL gene (608537) on chromosome 3, and a form associated with the chromosome translocation t(3;8), as described by Cohen et al. (1979). Bodmer et al. (2002) reviewed the molecular genetics of familial and nonfamilial cases of renal cell carcinoma, including the roles of VHL, MET, and translocations involving chromosomes 1, 3, and X. For background information and a discussion of genetic heterogeneity of nonpapillary renal cell carcinoma, see RCC (144700). See also a hereditary syndrome of predisposition to uterine leiomyomas and papillary renal cell carcinoma (HLRCC; 150800) caused by germline mutation in the FH gene (136850).
Hyperparathyroidism 2 with jaw tumors
MedGen UID:
310065
Concept ID:
C1704981
Neoplastic Process
The spectrum of CDC73-related disorders includes the following phenotypes: Hyperparathyroidism-jaw tumor (HPT-JT) syndrome. Primary hyperparathyroidism, the main finding of HPT-JT syndrome, occurs in up to 95% of affected individuals; onset is typically in late adolescence or early adulthood. HPT-JT-associated primary hyperparathyroidism is usually caused by a single parathyroid adenoma. In approximately 10%-15% of individuals, primary hyperparathyroidism is caused by parathyroid carcinoma. Ossifying fibromas of the mandible or maxilla, also known as cementifying fibromas and cemento-ossifying fibromas, occur in 30%-40% of individuals with HPT-JT syndrome. Although benign, these tumors can be locally aggressive and may continue to enlarge if not treated. Approximately 20% of individuals with HPT-JT syndrome have kidney lesions, most commonly cysts; renal hamartomas and (more rarely) Wilms tumor have also been reported. Benign and malignant uterine tumors appear to be common in women with HPT-JT syndrome. Parathyroid carcinoma. Most parathyroid carcinomas are functional, resulting in hyperparathyroidism and a high serum calcium level; however, nonfunctioning parathyroid carcinomas are also rarely described in individuals with a CDC73-related disorder. A germline CDC73 pathogenic variant has been identified in 20%-29% of individuals with apparently sporadic parathyroid carcinoma. Familial isolated hyperparathyroidism (FIHP). FIHP is characterized by primary hyperparathyroidism without other associated syndromic features. Individuals with CDC73-related FIHP tend to have a more severe clinical presentation and younger age of onset than individuals with FIHP in whom a CDC73 pathogenic variant has not been identified.

Professional guidelines

PubMed

Zhao S, Li Y, Xu J, Shen L
Neurol Res 2024 Mar;46(3):227-242. Epub 2024 Jan 22 doi: 10.1080/01616412.2023.2287340. PMID: 38007705
Maughan BL
Curr Oncol Rep 2022 Sep;24(9):1201-1208. Epub 2022 Apr 19 doi: 10.1007/s11912-022-01269-1. PMID: 35438388Free PMC Article
Garcia-Donas J, Rodriguez-Moreno JF, Romero-Laorden N, Rodriguez-Antona C
Urol Oncol 2015 Apr;33(4):179-86. Epub 2014 Feb 1 doi: 10.1016/j.urolonc.2013.09.015. PMID: 24495452

Recent clinical studies

Etiology

Msaouel P, Genovese G, Tannir NM
Hematol Oncol Clin North Am 2023 Oct;37(5):977-992. Epub 2023 May 25 doi: 10.1016/j.hoc.2023.04.019. PMID: 37244822
Clingan MJ, Zhang Z, Caserta MP, Cox KL, Gupta V, Baumgarten DA, Zhai QJ, Alexander LF
Radiographics 2023 May;43(5):e220116. doi: 10.1148/rg.220116. PMID: 37053100
Mohanty SK, Lobo A, Cheng L
Hum Pathol 2023 Jun;136:123-143. Epub 2022 Sep 6 doi: 10.1016/j.humpath.2022.08.006. PMID: 36084769
Lobo J, Ohashi R, Amin MB, Berney DM, Compérat EM, Cree IA, Gill AJ, Hartmann A, Menon S, Netto GJ, Raspollini MR, Rubin MA, Tan PH, Tickoo SK, Tsuzuki T, Turajlic S, Zhou M, Srigley JR, Moch H
Histopathology 2022 Oct;81(4):426-438. Epub 2022 Jun 10 doi: 10.1111/his.14700. PMID: 35596618
Pal SK, Tangen C, Thompson IM Jr, Balzer-Haas N, George DJ, Heng DYC, Shuch B, Stein M, Tretiakova M, Humphrey P, Adeniran A, Narayan V, Bjarnason GA, Vaishampayan U, Alva A, Zhang T, Cole S, Plets M, Wright J, Lara PN Jr
Lancet 2021 Feb 20;397(10275):695-703. Epub 2021 Feb 13 doi: 10.1016/S0140-6736(21)00152-5. PMID: 33592176Free PMC Article

Diagnosis

Lobo J, Ohashi R, Amin MB, Berney DM, Compérat EM, Cree IA, Gill AJ, Hartmann A, Menon S, Netto GJ, Raspollini MR, Rubin MA, Tan PH, Tickoo SK, Tsuzuki T, Turajlic S, Zhou M, Srigley JR, Moch H
Histopathology 2022 Oct;81(4):426-438. Epub 2022 Jun 10 doi: 10.1111/his.14700. PMID: 35596618
Mendhiratta N, Muraki P, Sisk AE Jr, Shuch B
Urol Oncol 2021 Jun;39(6):327-337. Epub 2021 May 24 doi: 10.1016/j.urolonc.2021.04.013. PMID: 34034966
Ooi A
Semin Cancer Biol 2020 Apr;61:158-166. Epub 2019 Nov 2 doi: 10.1016/j.semcancer.2019.10.016. PMID: 31689495Free PMC Article
Zhao J, Eyzaguirre E
Arch Pathol Lab Med 2019 Sep;143(9):1154-1158. Epub 2019 Jan 23 doi: 10.5858/arpa.2018-0121-RS. PMID: 30672334
Li L, Parwani AV
Arch Pathol Lab Med 2011 May;135(5):671-4. doi: 10.5858/2009-0769-RSR.1. PMID: 21526966

Therapy

Maughan BL, Sirohi D
Curr Treat Options Oncol 2023 Sep;24(9):1199-1212. Epub 2023 Jul 6 doi: 10.1007/s11864-023-01107-x. PMID: 37407886
Dong Y, Xu J, Sun B, Wang J, Wang Z
Mol Diagn Ther 2022 Mar;26(2):203-227. Epub 2022 Mar 10 doi: 10.1007/s40291-021-00568-w. PMID: 35266116Free PMC Article
de Vries-Brilland M, McDermott DF, Suárez C, Powles T, Gross-Goupil M, Ravaud A, Flippot R, Escudier B, Albigès L
Cancer Treat Rev 2021 Sep;99:102228. Epub 2021 May 20 doi: 10.1016/j.ctrv.2021.102228. PMID: 34111642
Pal SK, Tangen C, Thompson IM Jr, Balzer-Haas N, George DJ, Heng DYC, Shuch B, Stein M, Tretiakova M, Humphrey P, Adeniran A, Narayan V, Bjarnason GA, Vaishampayan U, Alva A, Zhang T, Cole S, Plets M, Wright J, Lara PN Jr
Lancet 2021 Feb 20;397(10275):695-703. Epub 2021 Feb 13 doi: 10.1016/S0140-6736(21)00152-5. PMID: 33592176Free PMC Article
Li L, Parwani AV
Arch Pathol Lab Med 2011 May;135(5):671-4. doi: 10.5858/2009-0769-RSR.1. PMID: 21526966

Prognosis

Angori S, Lobo J, Moch H
Curr Opin Urol 2022 Jul 1;32(4):344-351. Epub 2022 Jun 9 doi: 10.1097/MOU.0000000000001000. PMID: 35674688Free PMC Article
Mendhiratta N, Muraki P, Sisk AE Jr, Shuch B
Urol Oncol 2021 Jun;39(6):327-337. Epub 2021 May 24 doi: 10.1016/j.urolonc.2021.04.013. PMID: 34034966
Zhao J, Eyzaguirre E
Arch Pathol Lab Med 2019 Sep;143(9):1154-1158. Epub 2019 Jan 23 doi: 10.5858/arpa.2018-0121-RS. PMID: 30672334
Ricketts CJ, De Cubas AA, Fan H, Smith CC, Lang M, Reznik E, Bowlby R, Gibb EA, Akbani R, Beroukhim R, Bottaro DP, Choueiri TK, Gibbs RA, Godwin AK, Haake S, Hakimi AA, Henske EP, Hsieh JJ, Ho TH, Kanchi RS, Krishnan B, Kwiatkowski DJ, Liu W, Merino MJ, Mills GB, Myers J, Nickerson ML, Reuter VE, Schmidt LS, Shelley CS, Shen H, Shuch B, Signoretti S, Srinivasan R, Tamboli P, Thomas G, Vincent BG, Vocke CD, Wheeler DA, Yang L, Kim WY, Robertson AG; Cancer Genome Atlas Research Network, Spellman PT, Rathmell WK, Linehan WM
Cell Rep 2018 Apr 3;23(1):313-326.e5. doi: 10.1016/j.celrep.2018.03.075. PMID: 29617669Free PMC Article
Cancer Genome Atlas Research Network, Linehan WM, Spellman PT, Ricketts CJ, Creighton CJ, Fei SS, Davis C, Wheeler DA, Murray BA, Schmidt L, Vocke CD, Peto M, Al Mamun AA, Shinbrot E, Sethi A, Brooks S, Rathmell WK, Brooks AN, Hoadley KA, Robertson AG, Brooks D, Bowlby R, Sadeghi S, Shen H, Weisenberger DJ, Bootwalla M, Baylin SB, Laird PW, Cherniack AD, Saksena G, Haake S, Li J, Liang H, Lu Y, Mills GB, Akbani R, Leiserson MD, Raphael BJ, Anur P, Bottaro D, Albiges L, Barnabas N, Choueiri TK, Czerniak B, Godwin AK, Hakimi AA, Ho TH, Hsieh J, Ittmann M, Kim WY, Krishnan B, Merino MJ, Mills Shaw KR, Reuter VE, Reznik E, Shelley CS, Shuch B, Signoretti S, Srinivasan R, Tamboli P, Thomas G, Tickoo S, Burnett K, Crain D, Gardner J, Lau K, Mallery D, Morris S, Paulauskis JD, Penny RJ, Shelton C, Shelton WT, Sherman M, Thompson E, Yena P, Avedon MT, Bowen J, Gastier-Foster JM, Gerken M, Leraas KM, Lichtenberg TM, Ramirez NC, Santos T, Wise L, Zmuda E, Demchok JA, Felau I, Hutter CM, Sheth M, Sofia HJ, Tarnuzzer R, Wang Z, Yang L, Zenklusen JC, Zhang J, Ayala B, Baboud J, Chudamani S, Liu J, Lolla L, Naresh R, Pihl T, Sun Q, Wan Y, Wu Y, Ally A, Balasundaram M, Balu S, Beroukhim R, Bodenheimer T, Buhay C, Butterfield YS, Carlsen R, Carter SL, Chao H, Chuah E, Clarke A, Covington KR, Dahdouli M, Dewal N, Dhalla N, Doddapaneni HV, Drummond JA, Gabriel SB, Gibbs RA, Guin R, Hale W, Hawes A, Hayes DN, Holt RA, Hoyle AP, Jefferys SR, Jones SJ, Jones CD, Kalra D, Kovar C, Lewis L, Li J, Ma Y, Marra MA, Mayo M, Meng S, Meyerson M, Mieczkowski PA, Moore RA, Morton D, Mose LE, Mungall AJ, Muzny D, Parker JS, Perou CM, Roach J, Schein JE, Schumacher SE, Shi Y, Simons JV, Sipahimalani P, Skelly T, Soloway MG, Sougnez C, Tam A, Tan D, Thiessen N, Veluvolu U, Wang M, Wilkerson MD, Wong T, Wu J, Xi L, Zhou J, Bedford J, Chen F, Fu Y, Gerstein M, Haussler D, Kasaian K, Lai P, Ling S, Radenbaugh A, Van Den Berg D, Weinstein JN, Zhu J, Albert M, Alexopoulou I, Andersen JJ, Auman JT, Bartlett J, Bastacky S, Bergsten J, Blute ML, Boice L, Bollag RJ, Boyd J, Castle E, Chen YB, Cheville JC, Curley E, Davies B, DeVolk A, Dhir R, Dike L, Eckman J, Engel J, Harr J, Hrebinko R, Huang M, Huelsenbeck-Dill L, Iacocca M, Jacobs B, Lobis M, Maranchie JK, McMeekin S, Myers J, Nelson J, Parfitt J, Parwani A, Petrelli N, Rabeno B, Roy S, Salner AL, Slaton J, Stanton M, Thompson RH, Thorne L, Tucker K, Weinberger PM, Winemiller C, Zach LA, Zuna R
N Engl J Med 2016 Jan 14;374(2):135-45. Epub 2015 Nov 4 doi: 10.1056/NEJMoa1505917. PMID: 26536169Free PMC Article

Clinical prediction guides

Takamatsu D, Kohashi K, Kiyozawa D, Kinoshita F, Ieiri K, Baba M, Eto M, Oda Y
Pathol Res Pract 2023 Feb;242:154313. Epub 2023 Jan 16 doi: 10.1016/j.prp.2023.154313. PMID: 36669395
Angori S, Lobo J, Moch H
Curr Opin Urol 2022 Jul 1;32(4):344-351. Epub 2022 Jun 9 doi: 10.1097/MOU.0000000000001000. PMID: 35674688Free PMC Article
Dong Y, Xu J, Sun B, Wang J, Wang Z
Mol Diagn Ther 2022 Mar;26(2):203-227. Epub 2022 Mar 10 doi: 10.1007/s40291-021-00568-w. PMID: 35266116Free PMC Article
de Vries-Brilland M, McDermott DF, Suárez C, Powles T, Gross-Goupil M, Ravaud A, Flippot R, Escudier B, Albigès L
Cancer Treat Rev 2021 Sep;99:102228. Epub 2021 May 20 doi: 10.1016/j.ctrv.2021.102228. PMID: 34111642
Cancer Genome Atlas Research Network, Linehan WM, Spellman PT, Ricketts CJ, Creighton CJ, Fei SS, Davis C, Wheeler DA, Murray BA, Schmidt L, Vocke CD, Peto M, Al Mamun AA, Shinbrot E, Sethi A, Brooks S, Rathmell WK, Brooks AN, Hoadley KA, Robertson AG, Brooks D, Bowlby R, Sadeghi S, Shen H, Weisenberger DJ, Bootwalla M, Baylin SB, Laird PW, Cherniack AD, Saksena G, Haake S, Li J, Liang H, Lu Y, Mills GB, Akbani R, Leiserson MD, Raphael BJ, Anur P, Bottaro D, Albiges L, Barnabas N, Choueiri TK, Czerniak B, Godwin AK, Hakimi AA, Ho TH, Hsieh J, Ittmann M, Kim WY, Krishnan B, Merino MJ, Mills Shaw KR, Reuter VE, Reznik E, Shelley CS, Shuch B, Signoretti S, Srinivasan R, Tamboli P, Thomas G, Tickoo S, Burnett K, Crain D, Gardner J, Lau K, Mallery D, Morris S, Paulauskis JD, Penny RJ, Shelton C, Shelton WT, Sherman M, Thompson E, Yena P, Avedon MT, Bowen J, Gastier-Foster JM, Gerken M, Leraas KM, Lichtenberg TM, Ramirez NC, Santos T, Wise L, Zmuda E, Demchok JA, Felau I, Hutter CM, Sheth M, Sofia HJ, Tarnuzzer R, Wang Z, Yang L, Zenklusen JC, Zhang J, Ayala B, Baboud J, Chudamani S, Liu J, Lolla L, Naresh R, Pihl T, Sun Q, Wan Y, Wu Y, Ally A, Balasundaram M, Balu S, Beroukhim R, Bodenheimer T, Buhay C, Butterfield YS, Carlsen R, Carter SL, Chao H, Chuah E, Clarke A, Covington KR, Dahdouli M, Dewal N, Dhalla N, Doddapaneni HV, Drummond JA, Gabriel SB, Gibbs RA, Guin R, Hale W, Hawes A, Hayes DN, Holt RA, Hoyle AP, Jefferys SR, Jones SJ, Jones CD, Kalra D, Kovar C, Lewis L, Li J, Ma Y, Marra MA, Mayo M, Meng S, Meyerson M, Mieczkowski PA, Moore RA, Morton D, Mose LE, Mungall AJ, Muzny D, Parker JS, Perou CM, Roach J, Schein JE, Schumacher SE, Shi Y, Simons JV, Sipahimalani P, Skelly T, Soloway MG, Sougnez C, Tam A, Tan D, Thiessen N, Veluvolu U, Wang M, Wilkerson MD, Wong T, Wu J, Xi L, Zhou J, Bedford J, Chen F, Fu Y, Gerstein M, Haussler D, Kasaian K, Lai P, Ling S, Radenbaugh A, Van Den Berg D, Weinstein JN, Zhu J, Albert M, Alexopoulou I, Andersen JJ, Auman JT, Bartlett J, Bastacky S, Bergsten J, Blute ML, Boice L, Bollag RJ, Boyd J, Castle E, Chen YB, Cheville JC, Curley E, Davies B, DeVolk A, Dhir R, Dike L, Eckman J, Engel J, Harr J, Hrebinko R, Huang M, Huelsenbeck-Dill L, Iacocca M, Jacobs B, Lobis M, Maranchie JK, McMeekin S, Myers J, Nelson J, Parfitt J, Parwani A, Petrelli N, Rabeno B, Roy S, Salner AL, Slaton J, Stanton M, Thompson RH, Thorne L, Tucker K, Weinberger PM, Winemiller C, Zach LA, Zuna R
N Engl J Med 2016 Jan 14;374(2):135-45. Epub 2015 Nov 4 doi: 10.1056/NEJMoa1505917. PMID: 26536169Free PMC Article

Recent systematic reviews

Moraes FCA, Vilbert M, Alves VFC, de Oliveira Almeida G, Priantti JN, Madeira T, Stecca C, Fernandes MR, Dos Santos NPC
Int J Mol Sci 2023 Dec 18;24(24) doi: 10.3390/ijms242417582. PMID: 38139411Free PMC Article
Dong Y, Xu J, Sun B, Wang J, Wang Z
Mol Diagn Ther 2022 Mar;26(2):203-227. Epub 2022 Mar 10 doi: 10.1007/s40291-021-00568-w. PMID: 35266116Free PMC Article
Deng J, Li L, Xia H, Guo J, Wu X, Yang X, Hong Y, Chen Q, Hu J
Medicine (Baltimore) 2019 Jul;98(27):e16309. doi: 10.1097/MD.0000000000016309. PMID: 31277173Free PMC Article
Chiarello MA, Mali RD, Kang SK
AJR Am J Roentgenol 2018 Oct;211(4):812-821. Epub 2018 Jul 31 doi: 10.2214/AJR.17.19462. PMID: 30063398Free PMC Article
Giles RH, Choueiri TK, Heng DY, Albiges L, Hsieh JJ, Linehan WM, Pal S, Maskens D, Paseman B, Jonasch E, Malouf G, Molina AM, Pickering L, Shuch B, Srinivas S, Srinivasan R, Tannir NM, Bex A
Eur Urol 2017 Dec;72(6):974-983. Epub 2017 Jul 16 doi: 10.1016/j.eururo.2017.06.040. PMID: 28720391

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