Primary carcinoma of renal calyx

Renal calyx carcinoma (RCXC) may mimic collecting duct carcinoma (CDC) or urothelial carcinoma (UC) of the renal pelvis. RCXC is distinguished from CDC and UC of the renal pelvis as having the tumor epicenter in the renal calyx, with limited involvement of the surrounding renal pelvis surface urothelium. In this study, we summarize our experience with this entity. Ten cases of RCXC, including 9 cases with urothelial differentiation (RCXC-UC) and 1 case with salivary gland-type differentiation (RCXC-SC), were identified. Ten consecutive cases of UC were selected for comparison, with extensive renal pelvis involvement and with secondary renal parenchymal invasion. Two cases of collecting duct carcinoma (CDC) were also examined. Immunohistochemistry (IHC) was performed on representative tissue blocks for PAX8, PAX2, CK5, CK7, CK20, p63, GATA3, AMACR, RCC, CD10, vimentin, S100, and MSA. The 10 cases of RCXC (M:F=4:6, ages: 62-91 years, mean: 76) presented with renal masses of 3-6cm. Ureteroscopic studies and renal pelvic washings showed atypical/malignant cells in three cases. Seven patients were treated with nephrectomy followed by radiation±chemotherapy, and all cases developed metastases to lymph nodes or liver/lung/bone. In all 7 cases with nephrectomy, there was extensive renal parenchymal involvement with infiltrating borders and diffuse spread along collecting ducts. Six RCXC-UC contained focal squamous differentiation. The RCXC-SC displayed features of adenoid cystic and basaloid features. In situ UC, with or without papillary components, was identified in the calyces in all 7 nephrectomy cases with remaining renal pelvis harboring small tumor burden in 5 cases, and no tumor in another 2 cases. Of the three cases without nephrectomy, no tumor in the renal pelvis could be visualized with endoscopy, however one case was associated with UC of the urinary bladder. Of 10 control UC cases, tumor was limited to the tip of renal papilla in 7 cases, extensive in 3 cases, and with no extension into the collecting ducts. RCXC-UC were all positive for p63, CK5, CK7, and PAX2, with all negative for RCC. PAX8 (70% positive) and GATA3 (50% positive) reactivity was variable. The 10 UC cases shared IHC properties with RCXC-UC but frequent negativity for PAX8 and positivity for GATA3. RCXC is an aggressive neoplasm with high risk of metastases. Similar to CDC, it is located in the renal papilla and rarely with clinically visible renal pelvis tumor or ureteral urine positive for tumor cells. Unlike CDC and non-calyceal UC, RCXC shows predominantly urothelial and squamous differentiation and is associated with an in situ component of adjacent renal calyces. By IHC, RCXC exhibited features intermediate between UC and CDC with decreased or negative immunoreactivity for PAX8 and GATA.