Case ID: 503

Publication date: 08 Jan, 2016

Consensus grade: Hereditary leiomyomatosis and RCC-associated RCC

User Diagnosis Difficulty Comment
Pathologist 1 Hereditary leiomyomatosis and RCC-associated RCC Not typical
Pathologist 2 Tubulocystic RCC Typical
Pathologist 3 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 4 Renal cell carcinoma, unclassified Not typical

The low power photos are not in good focus. The high power have very prominent nucleoli and suggest hereditary leiomyomatosis-associated renal cell carcinoma but are not diagnostic of it.

Pathologist 5 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 6 Insufficient tumor for diagnosis Not typical
Pathologist 7 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 8 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 9 Renal cell carcinoma, unclassified Not typical
Pathologist 10 Succinate dehydrogenase deficient renal carcinoma Not typical
Pathologist 11 Hereditary leiomyomatosis and RCC-associated RCC Not typical

Best to confirm by ancillary studies

Pathologist 12 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 13 Hereditary leiomyomatosis and RCC-associated RCC Not typical
Pathologist 14 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 15 Other Not typical
Pathologist 16 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 17 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 18 Hereditary leiomyomatosis and RCC-associated RCC Not typical
Pathologist 19 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 20 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 21 Hereditary leiomyomatosis and RCC-associated RCC Not typical
Pathologist 22 Hereditary leiomyomatosis and RCC-associated RCC Typical
Pathologist 23 Acquired cystic disease-associated RCC Typical


Case description (by case creator):

Kidney tumor in 44-year-old male. Tumor 8 cm in the largest diameter, pT2 with dissemination into the pleural space and mediastinum. Tumor arranged in tubulocystic pattern. Cells are moderate size with slightly irregular nuclei, prominent deep red nucleoli. Tumor positive for CK 7, AMACR, vimentin. Polysomy of chromosomes 7 and 17 disclosed as well as mutation of FH gene.
Dg: familiar leiomyomatosis asscociated renal cell carcinoma (tubulocystic-like pattern)