Hereditary clear cell renal cell carcinoma

Hereditary clear cell renal cell carcinoma

Definition

A manifestation of von Hippel-Lindau disease or other familial renal cell cancer syndromes that present as a malignant epithelial neoplasm of the kidney. It is characterized by the presence of lipid-containing clear cells within a vascular network. The tumor usually is bilateral and polycentric, and metastasizes to unusual sites. Late metastasis is common.

Also known as hereditary clear cell renal carcinoma, hereditary clear cell renal cell adenocarcinoma, Hereditary clear cell renal cell cancer, hereditary clear cell renal cell carcinoma, hereditary conventional (clear cell) renal cell carcinoma — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Adult mammalian kidney Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Adult mammalian kidney Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Genitourinary system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Genitourinary system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Nephron tubule Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Renal system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Renal system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Upper urinary tract Disease Has Associated Anatomic Site NCIT · CC BY 4.0