LWBK1006-21 LWW-Govindan-Review December 12, 2011 19:6
288 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology ReviewANSWERS
Answer 21.1. The answer is B.
Hereditary papillary renal cell carcinoma is an autosomal-dominant
hereditary cancer syndrome characterized by mutations in the MET pro-
tooncogene, which encodes the hepatocyte growth factor/scatter factor
receptor tyrosine kinase. Although MET overexpression has been demon-
strated in a number of epithelial cancers, HRPC is the first cancer syn-
drome for which germ line MET mutations have been identified. Individ-
uals with this syndrome are at risk for developing multifocal, bilateral
papillary type 1 kidney cancer in their fifth and sixth decades of life, with
age-dependent penetrance of 67% by 60 years of age.Answer 21.2. The answer is D.
Renal cell cancer is currently divided into clear cell and nonclear cell sub-
types. The nonclear cell subtypes have been further divided into papillary,
chromophobe, and collecting duct tumors. Several renal cancers cannot
be accurately subtyped, often because of poor histologic differentiation
such that the originating subtype cannot be easily recognized. Such tumors
are often classified as “sarcomatoid.” Granular cell carcinoma has been
used in the past but is not currently an accepted pathologic classification.
Thus, a second opinion and pathologic review should be requested. The
risk of recurrence in this case is low, and adjuvant sunitinib has not been
shown to decrease the risk of recurrence. Patients who do have recurrence
after nephrectomy almost always recur systemically, and thus additional
local radiotherapy is not indicated. Likewise, the role of extensive lymph
node dissection is highly controversial and generally not recommended.
In any case, formal retroperitoneal lymph node dissection as is carried out
in testes cancer would certainly not be standard in patients with localized
renal cell carcinoma.Answer 21.3. The answer is C.
Patients with renal cancer can develop recurrent disease more than
5 years after initial definitive therapy. Development of a single metastatic
site is not unusual. Patients with a single site of metastatic disease and
a long interval between initial diagnosis and recurrent metastatic dis-
ease typically have a good prognosis. Thus, aggressive surgical resec-
tion including orthopedic stabilization is indicated. Because orthopedic
surgery such as this often leaves microscopic tumors behind in the sur-
gical field, additional radiotherapy to the site is appropriate. Radiation
alone is insufficient to completely eradicate metastatic renal cancer and
does not repair the underlying structural bony abnormality. High-dose
interleukin-2 is approved for metastatic renal cancer but is less effective
than aggressive local surgery for complete eradication of disease when
possible. Temsirolimus improves survival in patients with poor progno-
sis metastatic disease, which does not apply here. In addition, it is not
curative.