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Tubulocystic renal cell carcinoma

MedGen UID:
925623
Concept ID:
C4288091
Neoplastic Process
Synonyms: Tubulocystic renal cell cancer; Tubulocystic Renal Cell Carcinoma; tubulocystic renal cell carcinoma
SNOMED CT: Tubulocystic renal cell carcinoma (733602004); Tubulocystic renal cell carcinoma (733603009)
 
Monarch Initiative: MONDO:0017890
Orphanet: ORPHA319325

Definition

Tubulocystic renal cell carcinoma is an extremely rare subtype of renal cell carcinoma most frequently characterized by a small, solitary, well-circumscribed, unencapsulated renal tumor composed of multiple small to medium-sized cysts with a white or gray, spongy ("bubble wrap-like") cut surface. Patients are usually asymptomatic or could manifest with abdominal pain, abdominal distension and/or hematuria. Progression, recurrence and metastasis rarely occur although lymph node, bone, pleura and liver metastases have been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTubulocystic renal cell carcinoma

Professional guidelines

PubMed

Siegel C
J Urol 2019 Jan;201(1):10. doi: 10.1097/01.ju.0000550109.94023.60. PMID: 30577338
Honda Y, Nakamura Y, Goto K, Terada H, Sentani K, Yasui W, Sekino Y, Hayashi T, Teishima J, Matsubara A, Akagi M, Fuji T, Baba Y, Iida M, Awai K
Abdom Radiol (NY) 2018 Jul;43(7):1540-1545. doi: 10.1007/s00261-018-1643-8. PMID: 29796844
Delahunt B, Srigley JR, Montironi R, Egevad L
Urology 2014 May;83(5):969-74. Epub 2014 Mar 22 doi: 10.1016/j.urology.2014.02.004. PMID: 24661331

Recent clinical studies

Etiology

Renshaw AA, Gould EW
Diagn Cytopathol 2018 Aug;46(8):707-710. Epub 2018 Apr 6 doi: 10.1002/dc.23933. PMID: 29624918
Honda Y, Goto K, Nakamura Y, Terada H, Sentani K, Yasui W, Sekino Y, Hayashi T, Teishima J, Matsubara A, Fuji T, Kaichi Y, Higaki T, Baba Y, Iida M, Awai K
Abdom Radiol (NY) 2017 Jul;42(7):1850-1856. doi: 10.1007/s00261-017-1189-1. PMID: 28534069
Cornelis F, Hélénon O, Correas JM, Lemaitre L, André M, Meuwly JY, Sengel C, Derchi L, Yacoub M, Verkarre V, Grenier N
Eur Radiol 2016 Apr;26(4):1108-15. Epub 2015 Jul 24 doi: 10.1007/s00330-015-3923-9. PMID: 26201293
Sangle NA, Mao R, Shetty S, Schiffman JD, Dechet C, Layfield L, Agarwal N, Liu T
Indian J Pathol Microbiol 2013 Oct-Dec;56(4):428-33. doi: 10.4103/0377-4929.125361. PMID: 24441240
Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, Hes O, Moch H, Montironi R, Tickoo SK, Zhou M, Argani P; ISUP Renal Tumor Panel
Am J Surg Pathol 2013 Oct;37(10):1469-89. doi: 10.1097/PAS.0b013e318299f2d1. PMID: 24025519

Diagnosis

Xing S, Liu A, Yang X, Chen L, Xu D
Int J Immunopathol Pharmacol 2021 Jan-Dec;35:20587384211002966. doi: 10.1177/20587384211002966. PMID: 33726547Free PMC Article
Sarungbam J, Mehra R, Tomlins SA, Smith SC, Jayakumaran G, Al-Ahmadie H, Gopalan A, Sirintrapun SJ, Fine SW, Zhang Y, Amin MB, Reuter VE, Chen YB, Tickoo SK
Mod Pathol 2019 May;32(5):701-709. Epub 2019 Jan 8 doi: 10.1038/s41379-018-0185-5. PMID: 30622286Free PMC Article
Honda Y, Nakamura Y, Goto K, Terada H, Sentani K, Yasui W, Sekino Y, Hayashi T, Teishima J, Matsubara A, Akagi M, Fuji T, Baba Y, Iida M, Awai K
Abdom Radiol (NY) 2018 Jul;43(7):1540-1545. doi: 10.1007/s00261-018-1643-8. PMID: 29796844
Cornelis F, Hélénon O, Correas JM, Lemaitre L, André M, Meuwly JY, Sengel C, Derchi L, Yacoub M, Verkarre V, Grenier N
Eur Radiol 2016 Apr;26(4):1108-15. Epub 2015 Jul 24 doi: 10.1007/s00330-015-3923-9. PMID: 26201293
Ishibashi Y, Koie T, Fujita N, Satoh T, Mikami J, Hatakeyama S, Ohyama C, Tobisawa Y, Yoneyama T
J Med Case Rep 2014 Jul 31;8:265. doi: 10.1186/1752-1947-8-265. PMID: 25081056Free PMC Article

Therapy

Steiner P, Hora M, Stehlik J, Martinek P, Vanecek T, Petersson F, Michal M, Korabecna M, Travnicek I, Hes O
Virchows Arch 2013 Feb;462(2):183-92. Epub 2013 Jan 8 doi: 10.1007/s00428-012-1367-z. PMID: 23296808

Prognosis

Xing S, Liu A, Yang X, Chen L, Xu D
Int J Immunopathol Pharmacol 2021 Jan-Dec;35:20587384211002966. doi: 10.1177/20587384211002966. PMID: 33726547Free PMC Article
Ulamec M, Skenderi F, Zhou M, Krušlin B, Martínek P, Grossmann P, Peckova K, Alvarado-Cabrero I, Kalusova K, Kokoskova B, Rotterova P, Hora M, Daum O, Dubova M, Bauleth K, Slouka D, Sperga M, Davidson W, Rychly B, Perez Montiel D, Michal M, Hes O
Appl Immunohistochem Mol Morphol 2016 Aug;24(7):521-30. doi: 10.1097/PAI.0000000000000213. PMID: 26447894
Tran T, Jones CL, Williamson SR, Eble JN, Grignon DJ, Zhang S, Wang M, Baldridge LA, Wang L, Montironi R, Scarpelli M, Tan PH, Simper NB, Comperat E, Cheng L
Histopathology 2016 May;68(6):850-7. Epub 2015 Oct 21 doi: 10.1111/his.12840. PMID: 26310887
Zhao M, Teng X, Ru G, Zhao Z, Hu Q, Han L, He X
Int J Clin Exp Pathol 2015;8(9):11124-31. Epub 2015 Sep 1 PMID: 26617832Free PMC Article
Ishibashi Y, Koie T, Fujita N, Satoh T, Mikami J, Hatakeyama S, Ohyama C, Tobisawa Y, Yoneyama T
J Med Case Rep 2014 Jul 31;8:265. doi: 10.1186/1752-1947-8-265. PMID: 25081056Free PMC Article

Clinical prediction guides

Sangle NA, Mao R, Shetty S, Schiffman JD, Dechet C, Layfield L, Agarwal N, Liu T
Indian J Pathol Microbiol 2013 Oct-Dec;56(4):428-33. doi: 10.4103/0377-4929.125361. PMID: 24441240
Rao Q, Zhang XM, Tu P, Xia QY, Shen Q, Zhou XJ, Shi QL
Int J Clin Exp Pathol 2013;6(7):1452-7. Epub 2013 Jun 15 PMID: 23826432Free PMC Article

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