OncoLog, Volume 56, Number 4/5, April/May 2011 Page: 4
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Understanding and Managing Multiple
Endocrine Neoplasia Syndromes
[Continued from page 3]cological symptoms. The only excep-
tion is hypoglycemia caused by insulin-
producing tumors, which remains
relatively difficult to treat medically.
The primary treatment for PDNETs
is surgical resection. "Surgical resection
for MEN 1-associated gastrinomas is
associated with a durable return to
normal gastrin levels," said Elizabeth
Grubbs, M.D., an assistant professor in
the Department of Surgical Oncology.
Unfortunately, in PDNETs there is a
high chance of tumor recurrence even
after complete surgical resection. In
addition, unlike most of the other tu-
mors caused by the MEN disorders,
pancreatic tumors can be difficult to
treat surgically, and the pancreas has
no "back-up" glands to compensate
for its resection. However, new re-
search is offering some promising
chemotherapeutic options for patients
with advanced pancreatic tumors from
MENi or other causes.
A multicenter clinical trial recently
compared the effectiveness of evero-
limus, a mammalian target of rapa-
mycin inhibitor, to that of placebo in
patients with unresectable or metastatic
pancreatic neuroendocrine tumors.
The researchers found that the 18-
month progression-free survival
rate was significantly higher in the
everolimus group (34%) than in the
placebo group (9%). Dr. Yao, the
principal investigator for the trial at
MD Anderson, said, "We found that
everolimus is actually very good at
controlling insulin secretion from
these tumors as well."
Genetic testing
Since the MEN syndromes are
caused by mutations, there is a grow-
ing role for genetic testing in their
treatment. Many MEN patients know
of their family history, but others un-
dergo genetic testing after they devel-
op a characteristic disease. Thereasa
Rich, a genetic counselor with MD
Anderson's MEN Specialty Clinic,
said, "If we see early medullary thyroid
carcinoma, for instance, we often rec-
ommend genetic testing, since thisdisease is very rare in younger patients
without MEN mutations."
This testing also helps inform treat-
ment options, especially for MEN2.
However, the field of MEN-specific
treatment is still in its infancy. The
MEN Specialty Clinic at MD Ander-
son is a new interdisciplinary venture
that integrates the results of genetic
testing and treatment. Dr. Grubbs and
Ms. Rich have recently received a
grant to characterize in more detail
the various mutations that cause
MEN2. Ms. Rich said, "Many RET
mutations have different effects. For
instance, the RET918 mutation causes
very aggressive medullary thyroid tu-
mors, whereas some other mutations
may not cause such aggressive disease
and could be treated later." Using this
knowledge, endocrinologists, surgeons,
and genetic counselors may be able
to personalize treatment for each pa-
tient's specific mutation.
A way forward
Currently, management of the MEN
disorders is often limited to treatments
for each tumor that arises. And be-
cause of the rarity of the syndromes,
individual institutions generally have
not seen enough MEN patients to
make clinical trials feasible. However,
because of the rapid increase in na-
tional and interdisciplinary collabora-
tion, clinical trials and integrated
treatment strategies are starting to
be available for these and other rare
disorders.
"This increased collaboration
is rapidly leading to new treatments
for these cancers," Dr. Yao said, "It's
a very productive time for the treat-
ment of rare diseases."
FOR MORE INFORMATION
Dr. Gilbert Cote...................773-792-2840
Dr. Elizabeth Grubbs...........713-792-0665
Dr. Jeffrey Lee....................773-792-7218
Dr Nancy Perrier.................713-794-1345
Thereasa Rich .....................713-563-1908
Dr. Steven Waguespack .....713-792-2841
Dr. James Yao.....................713-792-2828Two-Stage Resectio
Regimens Lengthen
with Liver Metastas
By Bryan Tutt
New surgical tech-
niques and new
chemotherapy drugs
have pushed up the
median survival times
for patients with liver
metastases from
colorectal cancer
since the 1990s.
Patients with colorectal cancer
metastases involving both lobes of the
liver were once considered poor candi-
dates for surgery because removing all
the metastases in a single hepatectomy
would not leave enough healthy liver
tissue for the patient to survive. Two-
stage liver resection-in which a
limited liver resection is performed to
remove some metastases and to help
physicians determine whether a second,
more extensive resection would benefit
the patient-was widely adopted in the
late 1990s, making surgery possible for
more patients.
Because the surgery is usually done
only in patients who have responded
to chemotherapy-and because several
new and potent chemotherapy drugs
were approved for the treatment of col-
orectal cancer in the 1990s and early
2000s-some physicians have ques-
tioned whether the survival benefits of
two-stage resection are the result of
selection bias.
Advances in chemotherapy
Patients with colorectal cancer
with or without metastatic disease
typically are treated by resection of the
primary colorectal tumor followed by
chemotherapy. The chemotherapy regi-
men for these patients has evolved rapid-
ly in recent years. Most patients with4 OncoLog * April/May 2011
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University of Texas M.D. Anderson Cancer Center. OncoLog, Volume 56, Number 4/5, April/May 2011, periodical, April 2011; Houston, Texas. (https://texashistory.unt.edu/ark:/67531/metapth639462/m1/4/: accessed April 19, 2024), University of North Texas Libraries, The Portal to Texas History, https://texashistory.unt.edu; crediting UNT Libraries Government Documents Department.