Tubulocystic Renal Cell Carcinoma

This tumor is composed of small to intermediate- sized tubules admixed with larger cysts, which are lined by a single layer of flattened, cuboidal/columnar, and hobnail epithelium. The nuclei are enlarged and irregular, with intermediate to large (WHO / International Society of Urological Pathology [ISUP] grade 3) nucleoli. Cytoplasmic features include abundant eosinophilic oncocytoma-like aspects. The stroma is fibrotic. Components identical to those of papillary RCC can exist. The diagnosis of tubulocystic RCC should be restricted to tumours with the classic histological features. Tubulocystic RCC can contain poorly differentiated or sarcomatoid areas.Tubulocystic RCC shows gains of chromosomes 7 and 17 and loss of the Y chromosome, suggesting a close relationship with papillary RCC (previous paragraph from: Moch H et al. (Eds): WHO Classification of Tumours of the Urinary System and Male Genital Organs (4th edition). IARC: Lyon 2016).

Tubulocystic renal cell carcinoma in the older literature has variably been described as Bellinian epithelioma and low grade collecting duct carcinoma. The term tubulocystic renal cell carcinoma was first used by Amin et al. at the 2004 United States and Canadian Academy of Pathology annual meeting where they described a series of 29 cases defining the characteristics of this tumor; the series was subsequently published in 2009 (Amin MB, et al. Am J Surg Pathol. 2009 Mar;33(3):384-92. [PubMed link]). According to the ISUP, the term should not be used in situations in which there is a tubulocystic pattern admixed with the usual elements of papillary renal cell carcinoma or collecting duct carcinoma. Tubulocystic-like architecture may be variably present in many renal tumors, including renal oncocytoma, chromophobe renal cell carcinoma, fumarate hydratase-deficient renal cell carcinoma/hereditary leiomyomatosis renal cell carcinoma-associated renal cell carcinoma, and collecting duct carcinoma, among others. Many publications on tubulocystic carcinomas have included tumors with areas showing papillary architecture. In recent years, there have been some publications describing tubulocystic renal cell carcinoma with high-grade or poorly differentiated areas exhibiting tubulopapillary and nonglandular (solid/sheet-like, nested, cord-like, rhabdoid) components in addition to areas showing tubulocystic architecture (all the previous paragraph from: Sarungbam J, et al. Tubulocystic renal cell carcinoma: a distinct clinicopathologic entity with a characteristic genomic profile. Mod Pathol. 2019;32(5):701-709. [PubMed link]).

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Cimadamore A, Cheng L, Scarpelli M, Massari F, Mollica V, Santoni M, Lopez-Beltran A, Montironi R, Moch H. Towards a new WHO classification of renal cell tumor: what the clinician needs to know-a narrative review. Transl Androl Urol. 2021;10(3):1506-1520. [PubMed link]
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. Tubulocystic renal cell carcinoma: a distinct clinicopathologic entity with a characteristic genomic profile. Mod Pathol. 2019;32(5):701-709. [PubMed link]
Bhullar JS, Bindroo S, Varshney N, Mittal V. Tubulocystic Renal Cell Carcinoma: A Rare Renal Tumor. J Kidney Cancer VHL. 2014;1(5):56-62. [PubMed link]
Kuroda N, Matsumoto H, Ohe C, Mikami S, Nagashima Y, Inoue K, Perez-Montiel D, Petersson F, Michal M, Hes O, Yang XJ. Review of tubulocystic carcinoma of the kidney with focus on clinical and pathobiological aspects. Pol J Pathol. 2013;64(4):233-7. [PubMed link]
Amin MB, MacLennan GT, Gupta R, Grignon D, Paraf F, Vieillefond A, Paner GP, Stovsky M, Young AN, Srigley JR, Cheville JC. Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. Am J Surg Pathol. 2009 Mar;33(3):384-92. [PubMed link]