papillary renal cell carcinoma

Introduction

Also see renal cell carcinoma

Epidemiology

15-20% of renal cell carcinomas

Pathology

• immunocytochemistry:
  • cytokeratin AE1/AE3: positive
  • vimentin: positive

Genetics

• multiple translocations involving TFE3 on chromosome Xp11
  • t(X;17)(p11;q25) involving ASPSCR1 & TFE3
  • t(X;1)(p11.2;q21.2) involving PRCC & TFE3
  • t(X;1)(p11.2;p34) involving SFPQ with TFE3

Clinical manifestations

• generally presents with hematuria

More general terms

• renal cell carcinoma (hypernephroma, Grawitz tumor)
• papillary adenocarcinoma

More specific terms

• papillary renal cell carcinoma 2; hereditary papillary renal carcinoma (HPRC, RCCP2)
• papillary renal cell carcinoma [MET mutation]
• papillary renal cell carcinoma [RCCP1]
• papillary renal cell carcinoma [RCCP3]

Database

• OMIM: https://mirror.omim.org/entry/605074