Renal cell carcinoma
In this photograph, the kidney has been split through the middle. The tumor (a renal
cell carcinoma) is seen as two round, irregular masses in the upper part of the picture.
Very little normal kidney remains, but can be seen in the lower half of the photograph
just above the white marker. Because of their size, renal cell carcinomas can often be
felt as a mass in the abdomen.
Papillary renal carcinoma
Approximately 85% of renal cell cancers are adenocarcinomas, for the most part of proximal
tubular origin. Most of the remainder are transitional cell carcinomas of the renal
pelvis, which are discussed in the PDQ summary on transitional cell cancer of the renal
pelvis and ureter. Adenocarcinomas may be separated into clear cell and granular cell
carcinomas, although the 2 cell types may occur together in some tumors. Some
investigators have found that granular cell tumors have a worse prognosis, but this
finding is not universal. The distinction between well-differentiated renal
adenocarcinomas and renal adenomas can be difficult. The diagnosis is usually made
arbitrarily on the basis of size of the mass, but size alone should not influence the
treatment approach, since metastases can occur with lesions as small as 0.5 centimeters.
STAGE I RENAL CELL CANCER
Surgical resection is the accepted, often curative therapy for stage I renal cell cancer.
Resection may be simple or radical. The latter operation includes removal of the kidney,
adrenal gland, perirenal fat, and Gerota's fascia, with or without a regional lymph node
dissection. Some, but not all, surgeons
believe the radical operation yields superior results. In patients who are not candidates
for surgery, external radiation therapy or arterial embolization can provide palliation.
In patients with bilateral stage I neoplasms (concurrent or subsequent), bilateral partial
nephrectomy or unilateral partial nephrectomy with contralateral radical nephrectomy when
technically feasible may be a preferred
alternative to bilateral nephrectomy with dialysis or transplantation. There is
increasing evidence that a partial nephrectomy is curative in selected cases. It is
important to have a pathologist examine the gross specimen as well as the frozen section
from the parenchymal margin of excision.
1. Radical nephrectomy.
2. Simple nephrectomy.
3. Partial nephrectomy (selected patients).[1,3]
4. External-beam irradiation (palliative).
5. Arterial embolization (palliative).[3,4]
6. Clinical trials.
Improved understanding of the genetic basis of human renal cell carcinoma has
come from studies of families with a predisposition to develop renal carcinoma. This
discusses von Hippel-Lindau disease, one inherited disorder that confers a predisposition
to develop renal carcinoma. In addition to VHL, renal carcinoma is found in association
with a constitutional chromosome 3;8 translocation, and with hereditary non polyposis
colon carcinoma. Families have been described with several members affected with papillary
Identification of hereditary papillary renal carcinoma, a new hereditary cancer
The index patient with renal cell carcinoma was referred to the National Institutes of
Health for consideration of experimental immunotherapy. The patient gave a family history
of renal cell carcinoma. He reported that his father, brother, nephew and 2 uncles had
renal cell carcinoma. Further study of this family demonstrated that 3 generations were
affected with papillary renal cell carcinoma, an uncommon
histologic type of renal cell carcinoma.
Like VHL, the gross appearance of the kidneys from affected family members was striking.
Multiple tumors of varying size were present in both kidneys of affected family members.
Each renal tumor had a papillary growth pattern. The disorder was not linked to
polymorphic markers on chromosome 3p (the site of the VHL gene) and there was no loss of
heterozygosity at loci on 3p in renal tumors. No constitutional chromosomal alterations
were detected in affected individuals; no germline VHL mutations were detected in affected
individuals. The inherited disorder is this family appeared to be different from
recognized hereditary cancer syndromes.
Characteristics of papillary renal carcinoma
Clear cell renal carcinomas comprise about 80% of sporadic renal cell carcinomas
while papillary renal carcinomas comprise about 5-10% of sporadic renal cell
Karyotypes prepared from papillary renal carcinomas show characteristic genetic
changes distinct from those found in karyotypes prepared from clear cell renal
carcinomas. Papillary renal carcinomas show trisomy of chromosomes 7, 16 and
17, and in male patients, a loss of the Y chromosome.
Identification of 12 families with papillary renal carcinoma
Possibly family 150 was an isolated occurrence, a reflection of some as yet
unknown environmental factor. Alternatively, this family may represent a distinct
type of inherited cancer. Papillary renal carcinomas were often detected
incidentally in asymptomatic individuals or during screening of asymptomatic members of
renal carcinoma families. Families with papillary renal
cell carcinoma have been identified in Spain, Sweden, Australia, the Netherlands,
the United States and Canada. The results suggest that the predisposition to develop
papillary renal cell carcinoma may be inherited, and that hereditary papillary renal
carcinoma constitutes a distinct class of inherited cancer.
Identification of renal tumors by computerized tomography in members of
papillary renal carcinoma
Renal tumors were detected by computerized tomography in asymptomatic
members of papillary renal carcinoma families; renal tumors were unusually
hypovascular and could be mistakenly identified as cysts unless measurements of
density were obtained. However, when region of interest density measurements
were obtained these lesions demonstrated a 15-30 unit increase in Hounsfield
density after intravenous contrast .
CT scan of familial papillary renal
Computerized tomography of the abdomen of an asymptomatic 34 years old member of
an HPRC family. A renal tumor is present in the kidney (arrow).