Glucagonoma natural history, complications and prognosis: Difference between revisions
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Revision as of 20:01, 11 August 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2] Mohammed Abdelwahed M.D[3]
Overview
If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.
Natural History
- If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia.
- Glucagonoma has a very slow growth rate compared to most malignant tumors.
Complications
- Metastasis: Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Metastasis occurs mainly in the liver but in few cases, it can occur in lymph nodes, peritoneum, lung, and adrenals.
- Weight loss: due to the catabolic effect of glucagon, most patients lose weight.
- Anemia
- Neuropsychiatric manifestations include depression, dementia, psychosis, and agitation
- Reversible dilated cardiomyopathy[1]
Prognosis
- Age, grade, and distant metastases are the most significant predictors of survival.
- Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic neuroendocrine tumors.[2]
- Sixty percent of glucagonomas are malignant. Once the tumor is metastatic, the cure is rare.[3]
Prognosis of glucagonoma depends on the following:
- Whether or not the tumor can be removed by surgery
- The stage of the tumor, the size of the tumor, whether cancer has spread outside the pancreas
- The patient’s general health
- Whether the tumor has just been diagnosed or has recurred
- The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma.
- The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis.
References
- ↑ K. Chang-Chretien, J. T. Chew & D. P. Judge. "Reversible dilated cardiomyopathy associated with glucagonoma". Heart (British Cardiac Society). 90 (7): e44. doi:10.1136/hrt.2004.036905. PMID 15201270.
- ↑ Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV (1996). "The glucagonoma syndrome. Clinical and pathologic features in 21 patients". Medicine (Baltimore). 75 (2): 53–63. PMID 8606627.
- ↑ M. A. Chastain (2001). "The glucagonoma syndrome: a review of its features and discussion of new perspectives". The American journal of the medical sciences. 321 (5): 306–320. PMID 11370794. Unknown parameter
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