Glucagonoma natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Glucagonoma}} | {{Glucagonoma}} | ||
{{CMG}}{{AE}}{{PSD}} | {{CMG}}; {{AE}} {{PSD}}, {{MAD}} | ||
==Overview== | ==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== | ==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 == | ||
* | Complications of glucagonoma include: | ||
* [[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 [[Adrenal gland|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]]<ref>{{Cite journal | |||
| author = [[K. Chang-Chretien]], [[J. T. Chew]] & [[D. P. Judge]] | |||
| title = Reversible dilated cardiomyopathy associated with glucagonoma | |||
| journal = [[Heart (British Cardiac Society)]] | |||
| volume = 90 | |||
| issue = 7 | |||
| pages = e44 | |||
| doi = 10.1136/hrt.2004.036905 | |||
| pmid = 15201270 | |||
}}</ref> | |||
==Prognosis== | ==Prognosis== | ||
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]]. | ||
*The stage of the tumor | *The stage of the [[tumor]], the size of the [[tumor]], whether [[cancer]] has [[spread]] outside the [[pancreas]]. | ||
*The patient’s general | *The patient’s general health. | ||
*Whether the tumor has just been diagnosed or has recurred | *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. | *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]]. | |||
Additionally: | |||
* [[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> | |||
* 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> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
{{WH}} | |||
[[Category: | {{WS}} | ||
[[Category:Medicine]] | |||
[[Category:Endocrinology]] | [[Category:Endocrinology]] | ||
Latest revision as of 16:44, 20 November 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
Complications of glucagonoma include:
- 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
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.
Additionally:
- 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]
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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