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Pancreatic neuroendocrine tumors with transformation to insulinoma: an unusual presentation of a rare disease.

Nahmias A, Grozinsky-Glasberg S, Salmon A, Gross DJ - Endocrinol Diabetes Metab Case Rep (2015)

Bottom Line: In two cases everolimus therapy resulted in the abolishment of hypoglycemia and induced significant tumor regression; however these beneficial responses were transient.These cases highlight the exceptional ability of pNETs to change biological behavior in parallel with disease progression.Nonfunctional pNET can gain new features such as insulin secretion with related morbidity.Gain of function in a previously nonfunctional pNET signifies tumor progression and is usually associated with poor prognosis.Everolimus proved to be a viable treatment for hypoglycemia in insulinoma patients and was also proven highly effective in the patients presented here.As disease progresses, the effect of everolimus on hypoglycemia wanes.

View Article: PubMed Central - PubMed

Affiliation: Neuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Hadassah-Hebrew University Medical Center , Jerusalem , Israel.

ABSTRACT

Unlabelled: Approximately 35% of the pancreatic neuroendocrine tumors (pNETs) are functional, the most common of which is an insulinoma. Rarely can initially nonfunctioning tumor undergo biological transformation to a hormone-secreting tumor with subsequent changes in the clinical picture. We present here three unique patients with long-standing pNETs who developed life-threatening hyperinsulinemic hypoglycemia along with tumor progression. In two of the patients, everolimus (Afinitor) was administered in an attempt to control both tumor growth and hypoglycemia. In two cases everolimus therapy resulted in the abolishment of hypoglycemia and induced significant tumor regression; however these beneficial responses were transient. These cases highlight the exceptional ability of pNETs to change biological behavior in parallel with disease progression. Our experience concurs with recently published studies demonstrating the utility of everolimus for the control of both hypoglycemia and tumor progression.

Learning points: Nonfunctional pNET can gain new features such as insulin secretion with related morbidity.Gain of function in a previously nonfunctional pNET signifies tumor progression and is usually associated with poor prognosis.Everolimus proved to be a viable treatment for hypoglycemia in insulinoma patients and was also proven highly effective in the patients presented here.As disease progresses, the effect of everolimus on hypoglycemia wanes. We report for the first time the development of hypoglycemia during everolimus treatment.

No MeSH data available.


Related in: MedlinePlus

68Gallium-DOTATATE PET–CT of the third patient showing bilobar hepatic metastatic spread and a retroperitoneal conglomerate of lymph nodes.
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fig3: 68Gallium-DOTATATE PET–CT of the third patient showing bilobar hepatic metastatic spread and a retroperitoneal conglomerate of lymph nodes.

Mentions: One year later retroperitoneal lymph nodes spread was diagnosed; everolimus was started at a dosage of 10 mg/day with ensuing disease stabilization. 68Gallium-DOTATATE PET–CT imaging revealed bilobar hepatic metastatic spread and a retroperitoneal 7.5 cm conglomerate of lymph nodes (Fig. 3). At this stage the patient developed recurrent episodes of severe symptomatic hypoglycemia of 1.9 mmol/l, with inappropriately increased insulin (750 pmol/l, normal range=22–180) and C-Peptide (1621 pmol/l, normal range=268–1274) levels, while still receiving everolimus.


Pancreatic neuroendocrine tumors with transformation to insulinoma: an unusual presentation of a rare disease.

Nahmias A, Grozinsky-Glasberg S, Salmon A, Gross DJ - Endocrinol Diabetes Metab Case Rep (2015)

68Gallium-DOTATATE PET–CT of the third patient showing bilobar hepatic metastatic spread and a retroperitoneal conglomerate of lymph nodes.
© Copyright Policy - license
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4477235&req=5

fig3: 68Gallium-DOTATATE PET–CT of the third patient showing bilobar hepatic metastatic spread and a retroperitoneal conglomerate of lymph nodes.
Mentions: One year later retroperitoneal lymph nodes spread was diagnosed; everolimus was started at a dosage of 10 mg/day with ensuing disease stabilization. 68Gallium-DOTATATE PET–CT imaging revealed bilobar hepatic metastatic spread and a retroperitoneal 7.5 cm conglomerate of lymph nodes (Fig. 3). At this stage the patient developed recurrent episodes of severe symptomatic hypoglycemia of 1.9 mmol/l, with inappropriately increased insulin (750 pmol/l, normal range=22–180) and C-Peptide (1621 pmol/l, normal range=268–1274) levels, while still receiving everolimus.

Bottom Line: In two cases everolimus therapy resulted in the abolishment of hypoglycemia and induced significant tumor regression; however these beneficial responses were transient.These cases highlight the exceptional ability of pNETs to change biological behavior in parallel with disease progression.Nonfunctional pNET can gain new features such as insulin secretion with related morbidity.Gain of function in a previously nonfunctional pNET signifies tumor progression and is usually associated with poor prognosis.Everolimus proved to be a viable treatment for hypoglycemia in insulinoma patients and was also proven highly effective in the patients presented here.As disease progresses, the effect of everolimus on hypoglycemia wanes.

View Article: PubMed Central - PubMed

Affiliation: Neuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Hadassah-Hebrew University Medical Center , Jerusalem , Israel.

ABSTRACT

Unlabelled: Approximately 35% of the pancreatic neuroendocrine tumors (pNETs) are functional, the most common of which is an insulinoma. Rarely can initially nonfunctioning tumor undergo biological transformation to a hormone-secreting tumor with subsequent changes in the clinical picture. We present here three unique patients with long-standing pNETs who developed life-threatening hyperinsulinemic hypoglycemia along with tumor progression. In two of the patients, everolimus (Afinitor) was administered in an attempt to control both tumor growth and hypoglycemia. In two cases everolimus therapy resulted in the abolishment of hypoglycemia and induced significant tumor regression; however these beneficial responses were transient. These cases highlight the exceptional ability of pNETs to change biological behavior in parallel with disease progression. Our experience concurs with recently published studies demonstrating the utility of everolimus for the control of both hypoglycemia and tumor progression.

Learning points: Nonfunctional pNET can gain new features such as insulin secretion with related morbidity.Gain of function in a previously nonfunctional pNET signifies tumor progression and is usually associated with poor prognosis.Everolimus proved to be a viable treatment for hypoglycemia in insulinoma patients and was also proven highly effective in the patients presented here.As disease progresses, the effect of everolimus on hypoglycemia wanes. We report for the first time the development of hypoglycemia during everolimus treatment.

No MeSH data available.


Related in: MedlinePlus