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Mucinous tubular and spindle renal cell carcinoma

MedGen UID:
1640204
Concept ID:
C4707257
Neoplastic Process
Synonyms: carcinoma, renal, tubular, malignant; mucinous tubular and spindle cell carcinoma of the kidney; Mucinous tubular and spindle cell renal carcinoma; mucinous tubular and spindle cell renal carcinoma; mucinous tubular and spindle renal cell carcinoma; Renal mucinous tubular and spindle cell carcinoma; renal mucinous tubular spindle cell carcinoma
SNOMED CT: Mucinous tubular and spindle cell renal carcinoma (764990003); Renal mucinous tubular and spindle cell carcinoma (764991004); Renal mucinous tubular and spindle cell carcinoma (764990003)
 
Monarch Initiative: MONDO:0003011
Orphanet: ORPHA319322

Definition

Mucinous tubular and spindle cell renal carcinoma is a rare subtype of renal cell carcinoma characterized, histologically, by tubular architecture and sheets of spindle cells embedded in a mucinous/myxoid stroma and, macroscopically, by a solid, generally well-circumscribed, partially encapsulated tumor of variable size, with a homogenously colored, bulging cut surface, occassionally containing areas of hemorrhage or necrosis, usually located in the cortex. Patients can present abdominal/flank pain, adbominal mass and/or hematuria, however most are asymptomatic and tumor is discovered incidentally. Indolent behavior is frequent and association with nephrolithiasis and end-stage kidney disease has been noted. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMucinous tubular and spindle renal cell carcinoma

Professional guidelines

PubMed

Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Ged Y, Chen YB, Knezevic A, Donoghue MTA, Carlo MI, Lee CH, Feldman DR, Patil S, Hakimi AA, Russo P, Voss MH, Motzer RJ
Clin Genitourin Cancer 2019 Aug;17(4):268-274.e1. Epub 2019 May 2 doi: 10.1016/j.clgc.2019.04.006. PMID: 31151928Free PMC Article
Sarlos DP, Banyai D, Peterfi L, Szanto A, Kovacs G
Anticancer Res 2018 Dec;38(12):6663-6667. doi: 10.21873/anticanres.13033. PMID: 30504374

Recent clinical studies

Etiology

Kang H, Xu W, Chang S, Yuan J, Bai X, Zhang J, Guo H, Ye H, Wang H
Jpn J Radiol 2022 Nov;40(11):1175-1185. Epub 2022 May 30 doi: 10.1007/s11604-022-01294-x. PMID: 35644814
Baniak N, Barletta JA, Hirsch MS
Adv Anat Pathol 2021 Jul 1;28(4):228-250. doi: 10.1097/PAP.0000000000000301. PMID: 34009777
Kwon R, Argani P, Epstein JI, Lombardo KA, Wang X, Pierorazio PM, Mehra R, Matoso A
Am J Surg Pathol 2021 Apr 1;45(4):450-462. doi: 10.1097/PAS.0000000000001629. PMID: 33239504
Fleming S
Semin Diagn Pathol 2015 Mar;32(2):114-23. Epub 2015 Mar 9 doi: 10.1053/j.semdp.2015.02.004. PMID: 25804446
Wu XR, Chen YH, Sha JJ, Zhao L, Huang JW, Bo JJ, Liu DM, Huang YR
Diagn Pathol 2013 Dec 11;8:206. doi: 10.1186/1746-1596-8-206. PMID: 24330589Free PMC Article

Diagnosis

Baniak N, Barletta JA, Hirsch MS
Adv Anat Pathol 2021 Jul 1;28(4):228-250. doi: 10.1097/PAP.0000000000000301. PMID: 34009777
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Zhao M, He XL, Teng XD
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Fleming S
Semin Diagn Pathol 2015 Mar;32(2):114-23. Epub 2015 Mar 9 doi: 10.1053/j.semdp.2015.02.004. PMID: 25804446
Wu XR, Chen YH, Sha JJ, Zhao L, Huang JW, Bo JJ, Liu DM, Huang YR
Diagn Pathol 2013 Dec 11;8:206. doi: 10.1186/1746-1596-8-206. PMID: 24330589Free PMC Article

Therapy

Kobari Y, Yoshida K, Minoda R, Fukuda H, Hata K, Unagami K, Iizuka J, Ishida H, Nagashima Y, Takagi T
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Ann Diagn Pathol 2012 Oct;16(5):407-10. Epub 2011 Jun 17 doi: 10.1016/j.anndiagpath.2011.04.001. PMID: 21684183

Prognosis

Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Kwon R, Argani P, Epstein JI, Lombardo KA, Wang X, Pierorazio PM, Mehra R, Matoso A
Am J Surg Pathol 2021 Apr 1;45(4):450-462. doi: 10.1097/PAS.0000000000001629. PMID: 33239504
Nathany S, Monappa V
Arch Pathol Lab Med 2020 Jan;144(1):115-118. Epub 2019 Mar 13 doi: 10.5858/arpa.2017-0506-RS. PMID: 30865490
Zhao M, He XL, Teng XD
Diagn Pathol 2015 Sep 17;10:168. doi: 10.1186/s13000-015-0402-1. PMID: 26377921Free PMC Article
Wu XR, Chen YH, Sha JJ, Zhao L, Huang JW, Bo JJ, Liu DM, Huang YR
Diagn Pathol 2013 Dec 11;8:206. doi: 10.1186/1746-1596-8-206. PMID: 24330589Free PMC Article

Clinical prediction guides

Zhao M, Yin X, Yang X, Gan H, Chen N, Duan G, Bai Y, Teng X, Xu J, Fang R, Wang S, Zhong S, Wang X, Teng L
Mod Pathol 2024 Aug;37(8):100536. Epub 2024 Jun 8 doi: 10.1016/j.modpat.2024.100536. PMID: 38852815
Kang H, Xu W, Chang S, Yuan J, Bai X, Zhang J, Guo H, Ye H, Wang H
Jpn J Radiol 2022 Nov;40(11):1175-1185. Epub 2022 May 30 doi: 10.1007/s11604-022-01294-x. PMID: 35644814
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Zhao M, He XL, Teng XD
Diagn Pathol 2015 Sep 17;10:168. doi: 10.1186/s13000-015-0402-1. PMID: 26377921Free PMC Article
Wu XR, Chen YH, Sha JJ, Zhao L, Huang JW, Bo JJ, Liu DM, Huang YR
Diagn Pathol 2013 Dec 11;8:206. doi: 10.1186/1746-1596-8-206. PMID: 24330589Free PMC Article

Recent systematic reviews

Algaba F, Akaza H, López-Beltrán A, Martignoni G, Moch H, Montironi R, Reuter V
Eur Urol 2011 Oct;60(4):634-43. Epub 2011 Jul 3 doi: 10.1016/j.eururo.2011.06.047. PMID: 21741159

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