Renal cell carcinoma on a background of autosomal dominant polycystic kidney disease
Presentation
Follow-up renal cysts.
Patient Data
Bilateral renal cysts, most of which appear simple and anechoic. In the left interpolar region is a 4.5 cm heterogeneous solid appearing mass.
Innumerable hepatic and renal cysts. In the left renal interpolar region there is a hypodense mass with enhancing soft tissue at its medial aspect. No enlarged lymph nodes.
Left upper pole renal calculus.
The patient proceeded to a left partial nephrectomy.
Histopathology
MACROSCOPIC DESCRIPTION: 68g specimen, comprising loose fatty tissue 85x35x10mm, and a partial nephrectomy, 45x60x40mm. The entire surface (capsule and cut margin) is inked black. Sections show a cystic lesion, containing hemorrhagic material, with wall thickness 1-15mm. Sections through the thick wall of the cyst show a mottled gelatinous grey/yellow/pale pink cut face. Normal renal parenchyma is not clearly identified. The lesion meets the inked dissection margin. The lesion is present at the capsule surface, which is smooth and does not appear to be involved by tumor. Sections through loose fatty tissue are unremarkable. P
MICROSCOPIC DESCRIPTION: The solid areas within the cyst wall show a Sections taken from the thin area of the cyst show tumor with similar features although much thinner. Again there is a thin fibrous pseudocapsule. The features suggest the tumor size to be 50mm but with prominent central cystic necrosis. Focal dystrophic calcification is noted. Renal carcinoma synoptic report. Site: Left. Size: 50mm Type and morphology: Type 1 papillary carcinoma with papillary and tubular features. The cells show a minimum amount of eosinophilic cytoplasm whilst others show clear cytoplasm. There are focal areas showing foamy macrophages. The tumor has a pushing margin and a pseudocapsule <1mm extending over the surface which in one area is incomplete but the tumor remains pushing. Mitotic figures are present but infrequent. There are focal areas of hemorrhage and necrosis.
DIAGNOSIS: Type 1 papillary carcinoma, sized 50mm, Fuhrman grade 2, with marked cystic central necrotic change.
Case Discussion
Patients with ADPKD have a 50x increased risk of renal cell carcinomas, which typically manifest as atypical renal cysts. Surveillance can be challenging in these patients as each cyst needs to be evaluated on its own merit.
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