Presented by Dr. Andres Matoso and prepared by Dr. Harsimar Kaur.
Adult female with kidney tumor.
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1. Question
Adult female with kidney tumor.
Correct
Correct: B.
Histology: This solid and cystic tumor is composed of tumor cells with abundant eosinophilic cytoplasm, enlarged nuclei and prominent nucleoli. The cysts are lined by cells exhibiting hobnail arrangement with voluminous eosinophilic cytoplasm. The cells in the solid areas typically showed diffuse and compact acinar or nested growth. The cytoplasm shows a characteristic granular, fine or coarse stippling, consisting of basophilic to purple inclusions.
Discussion: Eosinophilic solid and cystic RCC (ESC RCC)shows the characteristic morphology of the case presented here, with often tan, solid, and cystic gross appearance and cells exhibiting eosinophilic, voluminous cytoplasm with granular stippling. The tumors are typically low stage and often show prominent nucleoli. There is frequent CK20 positivity, whereas CK7 is usually negative or only focally positive. Cathepsin-K can be positive, which is also positive in epithelioid AMLs but AMLs are negative for PAX8 while ESC RCC is most frequently positive. ESCs RCC are associated with mutations in the TSC1 gene, with or without association with tuberous sclerosis syndrome. While most patients present with organ confined disease and have a favorable outcome, rare cases of metastatic ESC RCC have been documented.
References:
1. Trpkov K, Hes O, Bonert M, et al. Eosinophilic, solid, and cystic renal cell carcinoma: clinicopathologic study of 16 unique, sporadic neoplasms occurring in women. Am J Surg Pathol. 2016;40:60–71.
2. Trpkov K, Abou-Ouf H, Hes O, Lopez JI, Nesi G, Comperat E, Sibony M, Osunkoya AO, Zhou M, Gokden N, Leroy X, Berney DM, Werneck Cunha I, Musto ML, Athanazio DA, Yilmaz A, Donnelly B, Hyndman E, Gill AJ, McKenney JK, Bismar TA. Eosinophilic Solid and Cystic Renal Cell Carcinoma (ESC RCC): Further Morphologic and Molecular Characterization of ESC RCC as a Distinct Entity. Am J Surg Pathol. 2017 Oct;41(10):1299-1308.
3. Palsgrove DN, Li Y, Pratilas CA, Lin MT, Pallavajjalla A, Gocke C, De Marzo AM, Matoso A, Netto GJ, Epstein JI, Argani P. Eosinophilic Solid and Cystic (ESC) Renal CellCarcinomas Harbor TSC Mutations: Molecular Analysis Supports an Expanding Clinicopathologic Spectrum. Am J Surg Pathol. 2018 Sep;42(9):1166-1181.
Incorrect
Correct: B.
Histology: This solid and cystic tumor is composed of tumor cells with abundant eosinophilic cytoplasm, enlarged nuclei and prominent nucleoli. The cysts are lined by cells exhibiting hobnail arrangement with voluminous eosinophilic cytoplasm. The cells in the solid areas typically showed diffuse and compact acinar or nested growth. The cytoplasm shows a characteristic granular, fine or coarse stippling, consisting of basophilic to purple inclusions.
Discussion: Eosinophilic solid and cystic RCC (ESC RCC)shows the characteristic morphology of the case presented here, with often tan, solid, and cystic gross appearance and cells exhibiting eosinophilic, voluminous cytoplasm with granular stippling. The tumors are typically low stage and often show prominent nucleoli. There is frequent CK20 positivity, whereas CK7 is usually negative or only focally positive. Cathepsin-K can be positive, which is also positive in epithelioid AMLs but AMLs are negative for PAX8 while ESC RCC is most frequently positive. ESCs RCC are associated with mutations in the TSC1 gene, with or without association with tuberous sclerosis syndrome. While most patients present with organ confined disease and have a favorable outcome, rare cases of metastatic ESC RCC have been documented.
References:
1. Trpkov K, Hes O, Bonert M, et al. Eosinophilic, solid, and cystic renal cell carcinoma: clinicopathologic study of 16 unique, sporadic neoplasms occurring in women. Am J Surg Pathol. 2016;40:60–71.
2. Trpkov K, Abou-Ouf H, Hes O, Lopez JI, Nesi G, Comperat E, Sibony M, Osunkoya AO, Zhou M, Gokden N, Leroy X, Berney DM, Werneck Cunha I, Musto ML, Athanazio DA, Yilmaz A, Donnelly B, Hyndman E, Gill AJ, McKenney JK, Bismar TA. Eosinophilic Solid and Cystic Renal Cell Carcinoma (ESC RCC): Further Morphologic and Molecular Characterization of ESC RCC as a Distinct Entity. Am J Surg Pathol. 2017 Oct;41(10):1299-1308.
3. Palsgrove DN, Li Y, Pratilas CA, Lin MT, Pallavajjalla A, Gocke C, De Marzo AM, Matoso A, Netto GJ, Epstein JI, Argani P. Eosinophilic Solid and Cystic (ESC) Renal CellCarcinomas Harbor TSC Mutations: Molecular Analysis Supports an Expanding Clinicopathologic Spectrum. Am J Surg Pathol. 2018 Sep;42(9):1166-1181.
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