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Presented by George Netto, M.D. and prepared by Robert E LeBlanc, M.D.
Case 1: 65 year old man with a 4.5 cm mass in the right kidney.
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Question 1 of 1
1. Question
Week 548: Case 1
65 year old man with a 4.5 cm mass in the right kidney.images/NETT1a.jpg
images/NETT1b.jpg
images/NETT1c.jpgCorrect
Answer: Tubulocystic carcinoma
Histology: Tubulocystic carcinomas average 4.3 cm (ranging in size from 0.5 to 17.5 cm) and are well circumscribed, in contrast to the infiltrative border of CDC. Necrosis and hemorrhage are not seen. Multilocular cystic cut surfaces composed of cysts ranging from microscopic to 1 cm in diameter impart a unique sponge like appearance. Serosanguenous material may fill some of the cysts. The intervening stroma is paucicelluar, fibrous and focally edematous lacking the dense inflammatory infiltrate and desmoplastic response that are characteristic of CDC. Ovarian like stroma is not a feature of tubulocystic carcinoma, a point to remember in the differential with mixed epithelial stromal tumor (MEST) of kidney. Cysts are lined by moderately atypical epithelial cells with hobnail appearance. The neoplastic polygonal epithelial cells have moderate amount of amphophilic granular cytoplasm with vesicular nuclei and prominent nucleoli. Only occasional mitotic figures are encountered.
Discussion: Tubulocystic carcinomas are almost always organ confined with no associated renal sinus or renal vein invasion. Prognosis is good with less that 10% rate of reported metastases. The differential with CDC can be resolved based on morphologic features. PAX8, PAX2, CD10 , p504s and CK7 positivity comprise the typical immunoprofile. The latter two markers are usually negative in CDC. Trisomy 17 has been shown in some tubulocystic carcinomas raising a possible relationship to PRCC. Similarities in gene expression profiling have also been shown.
Incorrect
Answer: Tubulocystic carcinoma
Histology: Tubulocystic carcinomas average 4.3 cm (ranging in size from 0.5 to 17.5 cm) and are well circumscribed, in contrast to the infiltrative border of CDC. Necrosis and hemorrhage are not seen. Multilocular cystic cut surfaces composed of cysts ranging from microscopic to 1 cm in diameter impart a unique sponge like appearance. Serosanguenous material may fill some of the cysts. The intervening stroma is paucicelluar, fibrous and focally edematous lacking the dense inflammatory infiltrate and desmoplastic response that are characteristic of CDC. Ovarian like stroma is not a feature of tubulocystic carcinoma, a point to remember in the differential with mixed epithelial stromal tumor (MEST) of kidney. Cysts are lined by moderately atypical epithelial cells with hobnail appearance. The neoplastic polygonal epithelial cells have moderate amount of amphophilic granular cytoplasm with vesicular nuclei and prominent nucleoli. Only occasional mitotic figures are encountered.
Discussion: Tubulocystic carcinomas are almost always organ confined with no associated renal sinus or renal vein invasion. Prognosis is good with less that 10% rate of reported metastases. The differential with CDC can be resolved based on morphologic features. PAX8, PAX2, CD10 , p504s and CK7 positivity comprise the typical immunoprofile. The latter two markers are usually negative in CDC. Trisomy 17 has been shown in some tubulocystic carcinomas raising a possible relationship to PRCC. Similarities in gene expression profiling have also been shown.
