Diagnostic challenge of glucagonoma: case report and literature
review.
Endocr Pract. 2006 Jul-Aug;12(4):422-6.
OBJECTIVE: To
report the diagnostic difficulties encountered in a case of
glucagonoma. METHODS: We provide a literature review and present the
clinical findings, pertinent laboratory data, and results of related
studies in a patient with a glucagonoma. RESULTS: A 54-year-old-man,
with no relevant history of endocrine disorders, presented to the
hospital with a 5-year history of recurrent stomatitis and glossitis,
a more recent weight loss of 11.5 kg, and recurrent pruritic maculae
on the scalp in conjunction with raised erythematous maculae in the
scrotal region and perineum that gradually migrated to the distal
extremities, becoming bullous and painful. The patient was
hospitalized, and because of the dermatologic findings suggestive of
necrolytic migratory erythema, the presence of a glucagonoma was
suspected. His blood glucose levels were in the normal range. Glucagon
levels were found to be elevated, and imaging studies confirmed the
presence of an enlarged mass in the pancreatic tail, without evidence
of extension to surrounding structures. Liver metastatic lesions were
also excluded. After surgical removal of the tumor, the skin and oral
mucosal lesions disappeared spontaneously. The histologic appearance
and immunohistochemical staining results confirmed the diagnosis of a
glucagonoma. Subsequently, all related symptoms resolved, and the
glucagon levels normalized. CONCLUSION: The diagnosis of glucagonoma
is often delayed. Clinicians should be aware of the unusual initial
manifestations of this tumor and the potential for less than a full
spectrum of the characteristic features of the glucagonoma syndrome.
Rare presentation of endocrine pancreatic tumor: a case of diffuse
glucagonoma without metastasis and necrolytic migratory erythema.J
Formos Med Assoc. 2005 May;104(5):363-6.
Glucagonoma is a
very rare endocrine pancreatic tumor. At diagnosis, most glucagonomas
are malignant and often metastatic. Suspicion of glucagonoma is based
on characteristic presentations known as "glucagonoma syndrome".
Glucagonoma is often found in the pancreatic body and/or tail and is
usually large enough to be localized by computed tomography. We report
a case of diffuse glucagonoma necrolytic migratory erythema (NME) in a
45-year-old man with mild diabetes mellitus, mild anemia, and weight
loss over 1.5 years. Diffused enlarged pancreas was noted on abdominal
ultrasonography incidentally during a routine health check-up. The
levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were
within normal limits. No enlarged lymph node or extrapancreatic tumor
mass was found by several imaging studies. Total pancreatectomy was
performed, and the pathology revealed glucagon-producing islet cells
and intrapancreatic vascular emboli of tumor cells. He died due to
internal bleeding and sepsis after surgery. Presentation of diffuse
malignant glucagonoma with tumor emboli but no metastasis or NME is
unusual.
Malignant glucagonoma of the pancreas diagnoses through anemia and
diabetes mellitus.J
Hepatobiliary Pancreat Surg. 2003;10(1):101-5.
Glucagonoma of
the pancreas is a rare tumor with distinct clinical manifestations,
such as necrolytic migratory erythema,weight loss, anemia, diabetes
mellitus, and hypoamino-acidemia. We report the case of a 68-year-old
Japanese man who underwent curative resection for malignant
glucagonoma of the pancreas diagnosed through anemia and diabetes
mellitus. The patient had had diabetes mellitus for 20 years. Anemia
was diagnosed in 1998. On admission, the hemoglobin level was 8.3g/dl,
but the levels of serum iron, vitamin B12, and erythropoietin and, the
number of reticulocytes were within normal limits. The levels of
carcinoembryonic antigen (CEA), carbohydrate antigen (CA)19-9, and
DUPAN-2 were also within normal limits, and exocrine function of the
pancreas (PFD, 75%) was normal. Ultrasonography (US) revealed a
hypoechoic tumor in the distal pancreas. Computed tomography (CT)
demonstrated a high-density area 4 cm in diameter with calcification.
The serum glucagon level was very high (2360 pg/ml), but the levels of
other hormones such as somatostatin or gastrin were within normal
limits, while insulin was low. Glucagonoma of the pancreas was
diagnosed, and distal pancreatectomy with splenectomy was performed.
Histological examination revealed a malignant endocrine tumor,which
was immunohistochemically positive for chromogranin A and glucagon.
Two months after the operation, the serum glucagon level had decreased
to within normal limits and the hemoglobin level had increased to 10.4
g/dl. The case of glucagonoma reported here was found through
diagnostic examinations of anemia and treated by surgical resection,
by which the patient's anemia was largely alleviated. Therefore, we
recommend checking patients who have diabetes mellitus and anemia in
order to diagnose and treat glucagonoma in its early stage.
The
glucagonoma syndrome: a review of its features and discussion of new
perspectives.
Am J Med Sci. 2001 May;321(5):306-20.
Glucagonoma
syndrome is a paraneoplastic phenomenon characterized by an islet
alpha-cell pancreatic tumor, necrolytic migratory erythema, diabetes
mellitus, weight loss, anemia, stomatitis, thromboembolism, and
gastrointestinal and neuropsychiatric disturbances. These clinical
findings in association with hyperglucagonemia and demonstrable
pancreatic tumor establish the diagnosis. Glucagon itself is
responsible for most of the observed signs and symptoms, and its
induction of hypoaminoacidemia is thought to lead to necrolytic
migratory erythema. Liver disease and fatty acid and zinc deficiency
states may also contribute to the pathogenesis of the eruption in some
cases. Most patients are diagnosed too late in the clinical course for
cure, but successful palliation of symptomatology can usually be
achieved with surgical and medical intervention. This paper reviews
the glucagonoma syndrome, paying particular attention to its cutaneous
features, and provides new perspectives in our current understanding
of this phenomenon.
Gastrointestinal endocrine tumours. Glucagonomas.
Baillieres Clin Gastroenterol. 1996
Dec;10(4):697-705.
Glucagonoma is
an uncommon, challenging but treatable disease with varied
manifestations. Despite its predominantly malignant nature, prolonged
symptom-free survival can be achieved using a targeted combination of
surgery, hepatic artery embolization and somatostatin analogues. Given
the difficult management issues, an initial assessment in an
experienced tertiary referral centre may also be of benefit. This
chapter has looked at the long-term follow-up of 18 such patients over
a 25-year period. Given the rarity of the tumour, the numbers are
small, but valuable lessons can be learnt from the study in the
clinical management of these patients.
Glucagonoma. A
tumor disease with multiple clinical manifestations.
Lakartidningen. 1996 Aug 28;93(35):2935-9.
Glucagon-producing
neoplasms are rare pancreatic tumours that may give rise to a
characteristic syndrome including, diabetes and typical skin
manifestations (necrolytic migrating erythema). Dermatological
problems are often the first signs of the disease and the diagnosis is
easily overlooked. In most series reported to date, glucagonomas had
already metastasized at diagnosis, which means that curative surgery
was possible to perform in less than half of the patients. To increase
awareness of glucagonoma symptomatology a review of the syndrome is
presented together with the clinical histories of three patients,
recently treated. These cases illustrate aspects of modern diagnosis
and treatment.
Glucagonoma
syndrome.
Am J Med. 1987 May 29;82(5B):25-36.
The
glucagonoma syndrome is characterized by a necrolytic migratory
erythematous rash, angular stomatitis, painful glossitis, a
normochromic normocytic anemia, mild diabetes mellitus, weight loss, a
tendency to thrombosis, and neuropsychiatric disturbances. The
diagnosis is made by finding a high plasma glucagon concentration in
the absence of any other cause, such as renal failure or severe
stress. A pancreatic alpha-cell tumor can be identified and stained by
immunocytochemistry with glucagon antibodies. Optimal treatment is
surgical removal, but approximately 50 percent of the tumors have
metastasized by the time of diagnosis. Since the tumor is
slow-growing, remission can be obtained by hepatic artery embolization
to shrink hepatic secondaries or by shrinkage, in about 10 percent of
patients, with the combination chemotherapeutic regimen of
5-fluorouracil and streptozotocin. The rash frequently responds to
administration of zinc, a high-protein diet, and control of the
diabetes with insulin. Alongside the alpha cell in the islets of
Langerhans is the D-cell, which produces somatostatin and may well act
physiologically as a paracrine inhibitor of glucagon release. A newly
developed, long-acting somatostatin analogue, SMS 201-995, which the
patient can self-administer as a subcutaneous injection, has proven
effective in suppressing glucagon secretion from glucagonomas and, in
some cases, causing remission of clinical symptoms.
Glucagonoma.
Nippon Geka Gakkai Zasshi. 1984
Sep;85(9):1039-43.
A 36 year old
woman was admitted because of upper abdominal pain, fullness and
weight loss. Pancreatic scintigram revealed abnormal accumulation of
the radioisotope in the pancreatic head, and hepatic scintigram showed
multiple filling defect in the bilateral lobe. Celiac angiogram
demonstrated a tumor stain at the pancreatic head, encasement of the
splenic artery and metastasis to the liver. The diagnosis of malignant
glucagonoma was substantiated by high serum glucagon level of 1,100
pg/ml. Streptozotocin of 1.5g was administered intravenously once a
week, totalling 9g. Thereafter, blood level of glucagon declined to
the normal range, accompanied by improvement of diabetes mellitus and
weight gain. At laparotomy, there was an over fist-sized mass at the
body and tail of the pancreas, infiltrating the pancreatic head and
periaortic region, and was found unresectable. On light microscopy,
biopsied specimen was seen to be a tumor that contained glucagon.
Secretary granules resembling A cell granules were observed by
electron microscopy. She died of emaciation 6 years after the onset of
the disease. Eighteen cases of glucagonoma reported in Japan, and 64
cases in Europe and the United States were reviewed in terms of the
diagnosis and treatment.
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