Glucagonoma natural history, complications and prognosis: Difference between revisions
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*If left untreated, patients with glucagonoma may progress to develop [[necrolytic migratory erythema]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. | *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. | *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. | |||
* | |||
==Prognosis== | ==Prognosis== | ||
* [[Age]], [[grade]], and distant [[metastases]] are the most significant predictors of survival. | * [[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]].<ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref> | * Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic [[neuroendocrine tumors]].<ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627 }}</ref> | ||
* Sixty percent of glucagonomas are [[malignant]]. Once the tumor is [[Metastasis|metastatic]], the cure is rare. | * Sixty percent of glucagonomas are [[malignant]]. Once the tumor is [[Metastasis|metastatic]], the cure is rare.<ref>{{Cite journal | ||
| author = [[M. A. Chastain]] | |||
| title = The glucagonoma syndrome: a review of its features and discussion of new perspectives | |||
| journal = [[The American journal of the medical sciences]] | |||
| volume = 321 | |||
| issue = 5 | |||
| pages = 306–320 | |||
| year = 2001 | |||
| month = May | |||
| pmid = 11370794 | |||
}}</ref> | |||
Prognosis of glucagonoma depends on the following: | Prognosis of glucagonoma depends on the following: | ||
*Whether or not the tumor can be removed by surgery | *Whether or not the tumor can be removed by surgery | ||
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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.
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.[1]
- Sixty percent of glucagonomas are malignant. Once the tumor is metastatic, the cure is rare.[2]
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
- ↑ 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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