Definition
Tubulocystic carcinoma is an uncommon multicystic renal cell tumor.
Clinical Features
Incidence
The tumor comprises less than 1% of all renal carcinomas.
Age
Tubulocystic carcinoma occurs most commonly in the fifth and sixth decades of life.
Sex
There is a male predominance.
Site
There is no site predilection.
Treatment
Radical nephrectomy is generally recommended, but partial nephrectomy may be performed for small tumors located in the superficial renal cortex.
Outcome
They usually display an indolent behavior with a few cases with metastasis or recurrence.
Macroscopy
The tumors are solitary well-circumscribed multicystic renal mass. They are frequently 4 cm or less and composed of multiple small- to intermediate-sized cysts and have a white and sponge-like cut surface.
Microscopy
The cysts above described are lined by a single layer of flattened, cuboidal, and hobnailed eosinophilic renal cells with enlarged nuclei with prominent (ISUP/WHO 2016 grade 3) nucleoli (Fig. 1)....
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References and Further Reading
Amin, M. B., MacLennan, G. T., Gupta, R., et al. (2009). Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. The American Journal of Surgical Pathology, 33, 384–392.
Sarungbam, J., Mehra, R., Tomlins, S. A., et al. (2019). Tubulocystic renal cell carcinoma: A distinct clinicopathologic entity with a characteristic genomic profile. Mod Pathol, 32, 701–709.
Yang, X. J., Zhou, M., Hes, O., et al. (2008). Tubulocystic carcinoma of the kidney: Clinicopathologic and molecular characterization. The American Journal of Surgical Pathology, 32, 177–187.
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Caliò, A., Segala, D., Martignoni, G. (2019). Tubulocystic Renal Cell Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4962-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4962-1
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