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Somatostatinoma: Clinico-pathological features of three cases and
literature reviewed. J
Gastroenterol Hepatol. 2007 Jul 20
Somatostatinoma is a rare endocrine tumor that comprises around 1% of
all gastroenteropancreatic endocrine neoplasms. This paper gives an
updated review on somatostatinoma and describes three sporadic cases
of somatostatinoma located in the pancreas, duodenum, and jejunum.
Approximately 200 case histories of somatostatinoma have been
published, with the duodenum being the most frequent site, followed by
the pancreas. Somatostatinomas may be sporadic or associated with
neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von
Hippel-Lindau syndromes. Functional somatostatinomas release excessive
amounts of somatostatin suppressing gallbladder motility and
inhibiting the secretory activity of various endocrine and exocrine
cell types. A triad of mild diabetes mellitus, cholelithiasis, and
diarrhea/steatorrhoea characterizes the somatostatinoma or
'inhibitory' syndrome. Non-functional somatostatinomas tend either to
be asymptomatic or to present with obstructive symptoms. These tumors
are often malignant and by the time they are detected, nearly
two-thirds have already metastasized to the regional lymph nodes or
the liver. A comparison between our three cases and those in the
literature provides useful insights into the clinical management of
these patients. Interestingly, the jejunal somatostatinoma described
here is the second case ever reported.
Somatostatinoma of the Vater's papilla in a patient
with von Recklinghausen's disease.Vojnosanit
Pregl. 2007 Mar;64(3):219-22.
BACKGROUND:
Somatostatinomas of the gastrointestinal tract secret hormon
somatostatin which can cause "inhibitory syndrom" comprising diabetes
mellitus, cholelithiasis and steatorrheic diarrhea. It is also
secreted by the D cells of Langerhans's islands of the pancreas as
well as endocrine cells of the stomack, small bowel, salivary glands
and parafollicular cells of the thiroid gland. Somatostatinomas of the
digestive tract appear within the pancreas and duodenum. Patients
suffering from von Recklinghausens's disease are paticularly prone to
the somatostatinomas of the duodenum. CASE REPORT: In this paper we
presented a 51-year-old female patient with von Recklinghausen's
disease in whom, during the investigation for obstructive jaundice,
tumor of the Vater's papilla was found. The patient was submitted to
Whipple's duodenopancreatectomy. Histology and immunohistochemistry
discovered type B glandular carcinoid tumor with strong
antisomatostatin and mild antigastrin immunoreactivity. The patient
stayed symptom-free more than four years now. CONCLUSION: Patients
with von Recklinghausen's disease should be examined for other tumors,
particularly carcinoids of the duodenum and papilla, especially if the
signs of cholestasis are present.
A case of
pancreatic somatostatinoma.Korean
J Gastroenterol. 2006 Nov;48(5):351-4.
Somatostatinoma is a rare neoplasm usually arising from the pancreas
and duodenum which typically presents with indolent, nonspecific
symptoms in the absence of systemic neuroendocrine manifestations that
characterize somatostatinoma syndrome. It accounts for less than 1% of
all gastrointestinal endocrine tumors with an annual incidence of 1
per 40 million. It is often associated with regional and/or portal
metastasis at the time of diagnosis, and complete tumor resection is
possible only in 60% to 70% of cases. We experienced a case of
pancreatic somatostatinoma recently. A 51-year-old woman presented
with right upper quadrant abdominal pain and loose stool for one
month. A hypermetabolic lesion in the pancreatic head was detected on
positron emission tomography-CT (PET-CT) scan. The tumor was resected
by pylorus preserving pancreaticoduodenectomy. Immunohistochemical
staining of the tumor tissue exhibited diffuse positivity for
somatostatin, but was negative for insulin and glucagon. Herein, we
report a case of pancreatic somatostatinoma diagnosed postoperatively.
Spectrum of
malignant somatostatin-producing neuroendocrine tumors.
Endocr Pract. 2006 Jul-Aug;12(4):394-400.
OBJECTIVE: To
evaluate the clinical manifestations and outcome of patients with
somatostatinomas--rare neuroendocrine tumors of pancreaticoduodenal
origin. METHODS: We searched the medical archives and tumor registry
of our institution for somatostatinomas or somatostatin-staining
tumors for the 12-year period from January 1990 to February 2002. In
addition, we reviewed laboratory databases for patients who had an
elevated serum somatostatin level. Patients with a neuroendocrine
tumor and an elevated serum somatostatin level or somatostatin-positive
tumor immunostaining were included in this study. RESULTS: Eleven
patients qualified (9 men and 2 women; median age at diagnosis, 45
years; age range, 22 to 73). The diagnosis of a somatostatinoma was
made by immunostaining of the tumor in 9 patients and by finding
elevated serum somatostatin levels in 2. Five primary tumors were of
duodenal and 6 of pancreatic origin. Psammoma body formation and
association with neurofibromatosis were seen only in the duodenal
tumors. The known primary tumor sizes varied from 2 to 6 cm. Liver
metastatic lesions were present in 6 patients, abdominal lymph node
involvement was found in 10 patients, and lung, spleen, and ovarian
metastatic involvement was noted in 1 patient each. Diabetes was
present in 4 patients (36%) and cholelithiasis in 7 (64%). The
presence of a mass led to the diagnosis in most patients with primary
duodenal tumors, whereas patients with pancreatic tumors were more
likely to have endocrine manifestations. A Whipple procedure was
performed in 6 patients, distal pancreatectomy in 3, hepatic artery
embolization or ligation in 3, and partial hepatectomy in 1.
Cancer-related death occurred in 4 patients, 1 to 8 years after
diagnosis (median, 4.5 years). At last follow-up, 2 patients were
alive without evidence of disease (8 and 10 years after diagnosis),
and 3 were alive with liver metastatic lesions. The status of 2
patients was unclear. CONCLUSION: Somatostatinomas occurred with
approximately equal frequency in the duodenum and the pancreas. The
duodenal tumors were more likely to be pure somatostatinomas and have
psammoma bodies. Pancreatic tumors were more likely to be
multihormonal. Cholelithiasis and diabetes were seen in 64% and 36%,
respectively, of the patients. Mass effect of the tumor was the usual
manifestation leading to diagnosis. These tumors are slow growing, and
long-term survival is possible.
Somatostatin-producing pancreatic endocrine carcinoma presented as
relapsing cholangitis -- a case report.:
Pancreatology. 2005;5(2-3):295-9. Epub 2005 Apr 21
Somatostatin-producing
endocrine tumors are rare neoplasms usually arising in the pancreas
and duodenum and they account for less than 1% of all gastrointestinal
endocrine tumors. Besides somatostatinoma syndrome, which is
characterized by diabetes mellitus, steatorrhea and cholelithiasis,
patients with somatostatin-producing endocrine tumors commonly
complain of nonspecific symptoms such as vague abdominal pain, weight
loss or changes in bowel habits. Tumor behavior cannot be predicted by
histological features alone, and malignancy is determined by the
presence of metastases. We report here a case of malignant pancreatic
endocrine tumor producing somatostatin presented as relapsing
cholangitis who was treated with Whipple pancreatoduodenectomy.
Periampullary
pancreatic somatostatinoma.Ann
Surg Oncol. 2002 Nov;9(9):869-74.
BACKGROUND:
Somatostatinomas involving the gastrointestinal tract are extremely
rare neoplasms that typically present with indolent, nonspecific
symptoms in the absence of systemic neuroendocrine manifestations that
characterize the somatostatinoma syndrome. Because of a relatively
large size at the time of presentation (average diameter of 5 cm) and
common location within the head of the pancreas, the Whipple procedure
(pancreaticoduodenectomy) serves as the predominant modality for
curative and palliative surgical approaches. METHODS: Two cases of
somatostatinoma involving the minor duodenal papilla with concomitant
pancreatic divisum were reviewed, with a general overview of this
unique islet cell tumor. RESULTS: Unlike typical somatostatinomas,
these two tumors were subcentimeter in size but were associated with
synchronous regional metastasis. CONCLUSIONS: Somatostatinomas are
often associated with regional and/or portal metastases at the time of
diagnosis, and only 60% to 70% of surgical cases result in complete
tumor resection. Predictors of an unfavorable prognosis include size
>3 cm, poor cytological differentiation, regional and/or portal
metastasis, and incomplete surgical resection. Even in the presence of
synchronous metastases, the 5-year overall survival for patients with
somatostatinoma is approximately 40%. Currently, there are no clinical
trials demonstrating significant improvement in survival with the use
of adjuvant therapy.
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