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Abramson Cancer CenterNCI CANCERLIT® Search: Islet Cell Carcinoma - July 2002
National Cancer Institute®
Last Modified: July 1, 2002
- [57-year-old patient with collapse during automobile driving]
- Radio-frequency ablation for symptom control in a patient with metastatic pancreatic insulinoma.
- Anterior segmentectomy of the pancreatic head for islet cell tumors.
- Genotype-phenotype analysis in multiple endocrine neoplasia type 1.
- Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome.
- Gastrinoma.
- Blind distal pancreatectomy for occult insulinoma, an inadvisable procedure.
- Insulinoma in pregnancy: a case report and review of the literature.
- Endoscopic ultrasound in the diagnosis and staging of pancreatic cancer.
- [Recurrent perforations of small intestines, gastroduodenal ulcers and diarrhea in a middle-aged woman]
- Gastric carcinoma resembling pancreatic mixed acinar-endocrine carcinoma.
- Gastrinoma.
Table of Contents
1
UI - 11963761
AU - Hempfling W; Rust C; Furst H; Aust DE; Reiser M; Jacob K; Engelhardt D
TI -
[57-year-old patient with collapse during automobile driving]
SO - Internist (Berl) 2002 Feb;43(2):259-62
AD - Medizinische Klinik II, Klinikum Grosshadern,
Ludwig-Maximilians-Universitat, Marchioninistrasse 15, 81377 Munchen.
2
UI - 11966750
AU - Scott A; Hinwood D; Donnelly R
TI -
Radio-frequency ablation for symptom control in a patient with
metastatic pancreatic insulinoma.
SO - Clin Endocrinol (Oxf) 2002 Apr;56(4):557-9
AD - Division of Vascular Medicine, Nottingham University, Derbyshire Royal
Infirmary, London Road, Derby DE1 2QY, UK. xoe06@dial.pipex.com
Malignant insulinomas are very rare endocrine tumours with a variable
clinical course. We describe a 51-year-old man who had two large
insulinomas resected from the body of the pancreas and 19 years later,
having again become symptomatic, was found to have hepatic metastases.
Medical treatment with diazoxide and octreotide failed to control his
symptoms, but repeated hepatic embolization effected both symptomatic
and biochemical improvements for a further 5 years. When symptoms
recurred but further embolization failed to control his symptoms the
hepatic metastases were treated by outpatient percutaneous
radio-frequency ablation. He remains symptom-free 18 months later and
levels of insulin and pro-insulin have nearly normalized. The survival,
with liver metastases, for 27 years in a man with a malignant insulinoma
has not been described previously. Malignant insulinoma may follow a
rather indolent course and symptoms respond well to locally destructive
therapies. Hepatic embolization is less traumatic than hepatic lobe
resection and radio-frequency ablation offers an alternative if vascular
access to the tumour is no longer possible.
3
UI - 11893944
AU - Sakamoto Y; Tanaka N; Nagai M; Nobori M; Otani T; Makuuchi M
TI -
Anterior segmentectomy of the pancreatic head for islet cell tumors.
SO - Pancreas 2002 Apr;24(3):317-9
4
UI - 12049533
AU - Kouvaraki MA; Lee JE; Shapiro SE; Gagel RF; Sherman SI; Sellin RV; Cote
TI -
GJ; Evans DB
Genotype-phenotype analysis in multiple endocrine neoplasia type 1.
SO - Arch Surg 2002 Jun;137(6):641-7
AD - Department of Surgical Oncology, The University of Texas MD Anderson
Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
HYPOTHESIS: Multiple endocrine neoplasia type 1 (MEN 1) syndrome is an
autosomal dominant disorder caused by germline mutations in the MEN1
gene and characterized by multiple endocrine tumors, most notably in the
parathyroid glands, pituitary, and pancreas. The syndrome demonstrates
variable expressivity and considerable genetic heterogeneity. Patient
data were examined for possible associations between genotype and
phenotype. DESIGN: We reviewed recorded medical data from 1975 to 2001
on patients with MEN 1 and compared specific types and locations of MEN1
gene mutations with manifestations of the syndrome. PATIENTS AND
RESULTS: We identified 109 affected patients from 24 MEN 1 kindreds. The
phenotypic expression of MEN 1 in affected individuals included
hyperparathyroidism in 74%, pancreatic endocrine tumors in 51%, and
pituitary tumors in 35%. Twelve of 14 insulinomas occurred in patients
with pituitary tumors. Mutation analysis was completed in 14 of 24
kindreds (80 of the 109 patients). Mutations were most common in exons 2
(31%), 9 (15%), and 10 (23%). All 21 patients with frameshift mutations
(and known pancreatic endocrine tumor status) had such tumors. Pituitary
tumors were associated with frameshift mutations in exon 2. CONCLUSIONS:
The type and location of MEN1 mutations may be associated with the
phenotypic expression of specific tumors. Such information may assist in
the genetic counseling and surveillance of at-risk patients. A specific
genotype-phenotype correlation is unlikely because of the heterogeneity
of the mutations in the MEN1 gene.
5
UI - 11702127
AU - Mortele KJ; Oei A; Bauters W; Timmermans F; Cuvelier C; Kunnen M; Ros PR
TI -
Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient
with Verner-Morrison syndrome.
SO - Eur Radiol 2001;11(10):1952-5
AD - Department of Radiology, University Hospital Gent, De Pintelaan 185,
9000 Gent, Belgium. kmortele@partners.org
We describe a rare case of a pancreatic VIPoma diagnosed in a patient
presenting with watery diarrhea, hypokalemia, and achlorhydria, the
so-called WDHA or Verner-Morrison syndrome. Emphasis is placed on the
dynamic gadolinium-enhanced MR profile of the tumor, characteristics
which have not been illustrated previously, to the best of our
knowledge.
6
UI - 12057114
AU - Li ML; Norton JA
TI -
Gastrinoma.
SO - Curr Treat Options Oncol 2001 Aug;2(4):337-46
AD - Department of Surgery, University of California, San Francisco, USA.
Gastrinoma treatment has evolved considerably in the last 20 years. In
particular, the advent of effective acid-reducing pharmacologic agents
has changed the primary morbidity of this disease entity from one of
acid hypersecretion to one of tumor growth and spread. Thus, while
symptoms can be temporized using histamine receptor antagonists, proton
pump inhibitors, or somatostatin analogs, cure can be effected only by
surgical means. Recent advances in operative techniques and pre- and
intra-operative imaging studies, including routine duodenotomy,
somatostatin-receptor scintigraphy, and intraoperative ultrasound, have
allowed for identification and subsequent resection of more than 95% of
gastrinoma tumors. Most experts agree that all sporadic cases of
localized gastrinoma should be excised. In addition, debulking of
metastatic tumor may improve symptoms and survival when cure cannot be
ascertained. There is, however, some controversy as to the surgical
approach for gastrinoma found in the setting of multiple endocrine
neoplasia, type 1. Because of the usual multiplicity and particular
indolence of these tumors, two primary strategies have emerged:
aggressive approaches have been advocated in an effort to eradicate all
present and potential tumor; and less aggressive, or nonoperative,
approaches have been suggested because it is unclear whether
intervention offers survival or disease-free benefit in this population.
We advocate surgical intervention for patients with gastrinoma and
multiple endocrine neoplasia, type 1 when tumors exceed 2.5 cm in size.
This tumor size has been associated with a higher likelihood of hepatic
metastases, which ultimately affects survival. The role of adjuvant
therapies for gastrinoma remains limited.
7
UI - 12081066
AU - Hirshberg B; Libutti SK; Alexander HR; Bartlett DL; Cochran C; Livi A;
TI -
Chang R; Shawker T; Skarulis MC; Gorden P
Blind distal pancreatectomy for occult insulinoma, an inadvisable
procedure.
SO - J Am Coll Surg 2002 Jun;194(6):761-4
AD - Division of Intramural Research, National Institute of Diabetes,
Digestive and Kidney Diseases, National Institutes of Health, Bethesda,
MD, USA.
BACKGROUND: Fasting hypoglycemia with neuroglycopenic symptoms corrected
by administration of glucose are the hallmarks for the diagnosis of
insulinoma. Surgical resection is the treatment of choice for
insulinomas, but localization of these lesions can be challenging. Blind
distal pancreatectomy has been advocated for occult insulinomas not
detected on imaging studies or during abdominal exploration. With the
advent of newer localization techniques, we challenge the wisdom of this
approach. STUDY DESIGN: The records of patients (multiple endocrine
neoplasia excluded) with pathologically proved insulinoma who were
screened at our institution or referred to us after a failed blind
distal pancreatectomy were reviewed. All records included patient
history and results of physical examination and routine blood and urine
tests. The diagnosis of insulinoma was confirmed during a supervised
fast. Patients with biochemically proved insulinoma underwent
localization studies and operation. Studies included CT scans, MRI,
transabdominal ultrasound, intraoperative ultrasonography, angiography
(more recently, Ca++-stimulated arteriography), and venous sampling.
RESULTS: From 1970 to 2000, 99 patients (34 men, 65 women; mean age 43
years) underwent operation. All patients with benign tumors (92) were
cured after operation. Seventeen patients were referred to the NIH after
a failed blind distal pancreatectomy. Of these, 5 were diagnosed as
having factitious hypoglycemia. In the other 12 patients a tumor was
localized in the pancreatic head. Two patients incorrectly diagnosed
with nesidioblastosis after initial surgery were subsequently cured by
resection of an insulinoma. CONCLUSIONS: The use of preoperative imaging
studies, most notably Ca++-stimulated arteriography, and intraoperative
ultrasonography permits detection of virtually all insulinomas,
including reopcrated cases. When a tumor is not detected, the procedure
should be terminated and the patient referred to a center capable of
performing advanced preoperative and intraoperative localization
techniques. With the preoperative and intraoperative imaging strategies
currently available, the use of blind distal pancreatectomy for occult
insulinoma should be abolished.
8
UI - 11961480
AU - Takacs CA; Krivak TC; Napolitano PG
TI -
Insulinoma in pregnancy: a case report and review of the literature.
SO - Obstet Gynecol Surv 2002 Apr;57(4):229-35
AD - Department of Obstetrics and Gynecology, Malcolm Grow Medical Center,
Andrews Air Force Base, Maryland 20762-6600, USA. catakacs@aol.com
Insulinomas are rare tumors with an incidence of approximately four
cases per million person-years. Nineteen cases of insulinoma during
pregnancy have been reported. Hypoglycemic symptoms usually appear
during the first trimester. A 28-year-old primigravida was admitted at 6
weeks of gestation after referral for uncontrolled seizures. Her
previous seizure work-up included a normal EEG and a normal magnetic
resonance imaging of the brain. Elevated fasting insulin and C-peptide
levels accompanied severe hypoglycemia. The patient was managed with
glucose monitoring, frequent small meals, and rare doses of glucagon.
Postpartum testing was consistent with insulinoma, and magnetic
resonance imaging indicated a mass in the tail of the pancreas. During
surgical exploration with intraoperative ultrasound, two insulinomas
were removed from the tail of the pancreas. The hypoglycemic episodes
resolved and the fasting glucose levels normalized. Insulinomas are rare
in pregnancy and can be difficult to diagnose. Symptoms may resolve
during the second and third trimesters, possibly due to changes in
glucose metabolism associated with pregnancy. Misdiagnosis has been
fatal. Careful management during pregnancy and aggressive treatment
after delivery are essential. TARGET AUDIENCE: Obstetricians and
Gynecologists, Family Physicians. LEARNING OBJECTIVES: After completion
of this article, the reader will be able to describe the pathophysiology
of an insulinoma, to list the potential tests used to make the diagnosis
of insulinoma, and to outline potential treatment options for a patient
with an insulinoma.
9
UI - 11831609
AU - Levy MJ; Wiersema MJ
TI -
Endoscopic ultrasound in the diagnosis and staging of pancreatic cancer.
SO - Oncology (Huntingt) 2002 Jan;16(1):29-38, 43; discussion 44, 47-9, 53-6
AD - Division of Gastroenterology and Hepatology, The Mayo Clinic Rochester,
Minnesota 55905, USA. levy.michael@mayo.edu
Patients with signs and symptoms suggestive of a pancreatic neoplasm
typically undergo initial imaging with transabdominal ultrasound or
computed tomography. This evaluation often reveals the presence of a
pancreatic mass or fullness. At times, the nature of the lesion is
poorly characterized, with uncertainty remaining as to whether the
lesion is an inflammatory mass or a neoplasm, and if it is cystic or
solid. In these circumstances, endoscopic procedures such as endoscopic
retrograde cholangiopancreatography and/or endoscopic ultrasound may be
required. These procedures offer other means of tissue sampling, disease
staging, and an option for palliative therapy. In this article, we
review the role of endoscopy for the diagnosis and staging of pancreatic
tumors, with a particular focus on endoscopic ultrasound.
10
UI - 12078143
AU - Harjola VP; Farkkila M
TI -
[Recurrent perforations of small intestines, gastroduodenal ulcers and
diarrhea in a middle-aged woman]
SO - Duodecim 2000;116(7):755-60
AD - HYKS:n sisatautien klinikka Haartmaninkatu 4, 00290 Helsinki.
veli-pekka.harjola@hyks.fi
11
UI - 12094386
AU - Fukunaga M
TI -
Gastric carcinoma resembling pancreatic mixed acinar-endocrine
carcinoma.
SO - Hum Pathol 2002 May;33(5):569-73
AD - Department of Pathology, Jikei University School of Medicine, Tokyo,
Japan.
A case of gastric carcinoma resembling pancreatic mixed acinar-endocrine
carcinoma of 77-year-old female is presented. This type of gastric tumor
has not been previously reported. The endoscopic mucosal resection
specimen of the fundus contained a 1.2 x 0.9 x 0.3 cm,
well-circumscribed, tan, soft nodular tumor with protruded configuration
with a central recess. Histologically, the tumor was confined to the
mucosa and submucosa and was characterized by three growth patterns;
acinar, solid, and glandular. The growth patterns were intermingled. The
tumor cells in the acinar component had round nuclei with prominent
nucleoli and diastase-resistant, periodic acid-Schiff-positive,
eosinophilic cytoplasm. Immunohistochemically, the tumor cells were
positive for CAM5.2, cytokeratin (CK) 7, CK 20, trypsin, lipase,
alpha-1-antitrypsin, and alpha-1-antichymotrypsin. The tumor cells in
the solid component were positive for Grimelius stain and chromogranin
A. The findings indicated that the tumor showed acinar and endocrine
differentiation. There was no heterotopic pancreas tissue in the
specimen. The patient was well without tumor at the 7-month follow-up.
It is important to know the existence of this type of gastric cancer and
to not confuse it with a metastatic lesion of the pancreatic origin.
Copyright 2002, Elsevier Science (USA). All rights reserved.
12
UI - 12095598
AU - McIntyre TP; Stahlfeld KR; Sell HW Jr
TI -
Gastrinoma.
SO - Am J Surg 2002 Jun;183(6):666-7
AD - Department of Surgery, Mercy Hospital of Pittsburgh, 1400 Locust St.,
Pittsburgh, PA 15232-4738, USA.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.
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