Clinical Briefing: Management of Neuroendocrine Tumors
March/April 2009
Neuroendocrine tumors (NETs) are a unique group of rare
malignancies classified as either pancreatic endocrine tumors
(PETS) or alimentary tract carcinoid lesions. Within each
category, tumors may be further classified as functional (causing
hormonal symptoms) or nonfunctional, in which case symptoms are
caused only by tumor growth. NETS are generally characterized by
slow growth rates, tenacity, and variable presentation––attributes that
often combine to confound diagnosis and circumvent treatment
strategies.
The specialists at Penn Gastroenterology have developed an
interdisciplinary team approach to the diagnosis, staging,
and medical and surgical treatment of NETs. The goals of treatment at
Penn include accurate diagnosis and staging, effective symptom control,
curative surgery (when possible), prevention of tumor progression (using
medical, radiological and surgical approaches), genetic counseling
(when indicated), and individualized long-term management
depending on disease progression.
Surgery can be curative and is typically
the firstline treatment for resectable patients. In patients
with unresectable lesions, hormonal therapy with octreotide, a somatostatin
analogue, may inhibit tumor growth.1 Patients may qualify
for various therapeutic or diagnostic research trials as
appropriate.
Case Study 1
Mr. G., a 54-year-old man, was referred to Penn Gastroenterology
for evaluation of cramping abdominal pain and diarrhea of
several months duration. A complete laboratory workup demonstrated
elevated levels of serum gastrin, parathyroid hormone, ionized
calcium, pancreatic polypeptide, and chromogranin A.
Upper endoscopy
revealed erosive esophagitis, hypertrophic gastric folds,
and multiple small submucosal nodules in the duodenum. Gastric
pH was 1.3. Biopsies of the duodenal lesions identified neuroendocrine
tumors. Endoscopic ultrasound identified a small (2.5 cm) pancreatic
lesion but no lymphadenopathy. An MRI performed by Penn Radiology
revealed no additional abdominal lesions. These findings
were confirmed by abdominal OctreoScan® at Penn Nuclear
Medicine.
Mr. G. was diagnosed with multiple endocrine neoplasia type 1 (MEN1)
with Zollinger-Ellison syndrome complicated by hyperparathyroidism and
a probable pancreatic polypeptide-secreting pancreatic NET.
With PPI therapy and gastric analysis to control gastric acid
hypersecretion, Mr. G. had complete resolution of his presenting
symptoms. He subsequently had an uncomplicated 3.5 gland
parathyroidectomy at the Penn Division of Oncologic Surgery.
Multidisciplinary evaluation by Penn Hematology-Oncology,
Endocrine Surgery, Genetics and Interventional Radiology resulted in
a decision not to operate on the pancreatic tumor at that time. At
eight months post-surgery, Mr. G’s condition is stable.
Figure 1. CT scan of the abdomen with contrast showing liver
metastases from a NET in patient 2.
|
|
Case Study 2
Mrs. T, a 56-year-old woman, was referred to Penn with a 10-year
history of the carcinoid syndrome. When originally diagnosed, her
abdominal CT scan revealed two mesenteric lesions (~3 cm) and a
smaller lesion in the terminal ileum. She was treated surgically and
followed with serial imaging and laboratory testing thereafter. When
she subsequently developed cramping diarrhea with a 24-hour urine
test for 5-hydroxyindolacetic acid (5-HIAA), the main urinary
metabolite of serotonin, measuring >250 mg/day (normal =< 6 mg/day)
she was referred to Penn for further management.
At Penn, she described frequent flushing episodes with multiple episodes of
cramping and diarrhea. Abdominal MRI revealed multiple bilateral
liver lesions and a CT-guided liver biopsy was positive for NET
(Fig.1). She began depo-octreotide (20mg/month) with a good
symptomatic response. Follow up 24 hr urine 5-HIAA level was only
mildly elevated at 18.2 and a chromogranin A level (a NET marker)
measured in the last week before her next octreotide dose was 30
(normal =< 18). An OctreoScan revealed bilateral liver lesions but no
disease outside the liver. An echocardiogram failed to identify carcinoid
of the heart.
During subsequent years of monitoring her urinary
5-HIAA and chromogranin A levels and imaging studies were stable
on octreotide maintenance. However, her most recent MRI and
octreoscan demonstrated increasing size of her liver metastases, still
confined to the liver. She is being considered for chemoembolization
by Interventional Radiology and her octreotide dose has been
increased to 30mg/month. She remains symptom-free.
1. Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors:
pancreatic endocrine tumors. Gastroenterology. 2008.135:1469-1492.
Our Team of Faculty
The Division of Gastroenterology at Penn offers a multidisciplinary
approach to GI medicine that combines advanced research with state-of-the-art
care for people with digestive, liver and pancreatic disorders. The dedicated
NET management team at Penn encompasses a variety of clinical disciplines,
including gastroenterology, oncology, oncological surgery, neurology,
neurosurgery, radiation oncology, interventional radiology, pathology,
endocrinology and genetic counseling.
Gastroenterology patients also benefit from
the resources of the Pereleman Center for Advanced Medicine.
Adjacent to the Hospital of the University of Pennsylvania, the Perelman
Center links Penn physicians and researchers in a common commitment to
translational medicine and excellence in care.
Perelman Center for Advanced Medicine
Gastroenterology
David C. Metz, MD
Professor of Medicine
Gregory G. Ginsberg, MD
Professor of Medicine
Michael L. Kochman, MD
Professor of Medicine
Geoffrey Spencer, MD
Instructor of Medicine
Endocrinology
Kolin Hoff, MD
Assistant Professor of Clinical Medicine
Susan J. Mandel, MD, MPH
Associate Professor of Medicine
Peter J. Snyder, MD
Professor of Medicine
Genetics
Katherine L. Nathanson, MD
Assistant Professor of Medicine
Hospital of the University of Pennsylvania
Interventional Radiology
Michael C. Soulen, MD
Professor of Radiology
Nuclear Medicine
Chaitanya Divgi, MD
Professor of Radiology
Daniel Pryma, MD
Assistant Professor of Radiology
Hematology/Oncology
Benjamin Musher, MD
Instructor
Weijing Sun, MD
Associate Professor of Medicine
Ursina R. Teitelbaum, MD
Assistant Professor of Clinical Medicine
Renal
Debbie Cohen-Stein, MD
Assistant Professor of Medicine
Surgical Oncology
Douglas Fraker, MD
Jonathan E. Rhoads Associate Professor of Surgical Science
Access
Perelman Center for Advanced Medicine
3400 Civic Center Boulevard
Philadelphia, PA 19104
Penn Presbyterian Medical Center
38th and Market Streets
218 Wright-Saunders Building
Philadelphia, PA 19104
Penn Medicine at Radnor
250 King of Prussia Road | Module B
Radnor, PA 19087
To refer a patient and/or consult with a doctor:
|
