Clear Cell Papillary Renal Cell Carcinoma in Von Hippel-Lindau Disease
Priya Rao, Federico A Monzon, Eric Jonasch, Bogdan A Czerniak, Pheroze Tamboli. MD Anderson Cancer Center, Houston, TX; Baylor College of Medicine, Houston, TX
Background: Clear cell papillary RCC is a renal neoplasm that has recently received widespread recognition in the literature. There have been several reports of this tumor arising in a sporadic setting and in patients with end-stage renal disease. However there is limited information available about the clinical, pathologic characteristics and genetic characteristic of this tumor in the setting of Von Hippel-Lindau (VHL) disease.
Design: We identified 29 patients from our VHL clinics database, who underwent resection at our institution. All patients had a clinical diagnosis of VHL. Histologic slides from 16 nephrectomy specimens were available for review.
Results: Three of sixteen patients had tumors that met the histologic criteria for clear cell papillary RCC. Two of the three patients presented with multiple bilateral cystic tumors. The third patient had a single renal tumor, in addition to an adrenal pheochromocytoma. All of the tumors in two patients were of the clear cell papillary type. One patient (#3) had multiple tumors, which included clear cell papillary and clear cell subtypes. The histology of in all cases was similar to that which has been previously reported for clear cell papillary RCC. Of note, all tumors were diffusely and strongly positive for CK7 and negative for racemase. All tumors showed at least focal staining for CD10, which is reportedly less common in tumors arising in the sporadic setting. The results are summarized in Table 1.
|Age/Gender||Other clinical stigmata of VHL||Tumor configuration/Laterality||Number of tumors||Tumor size (cm)||Fuhrman grade||Pathologic stage|
|2||39/F||Hemangioblastoma||Cystic/bilateral||Multiple||0.6-5.3||2||pT1 (all tumors)|
|3||38/F||Hemangioblastoma||Cystic/bilateral||Multiple||0.4-6.5||2||pT1 (all tumors)|