Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático

Chacón, H. Jaramillo; Devia, D. González; Panqueva, R. P. López; Solano, D. Cañon; Matallana, D. Aguirre; Rubiano, Adriana Margarita Rey; Gomez, Juan M.; Flórez, R. F. Dussan

doi:10.53853/encr.7.2.607

Cited by 3 publications

(3 citation statements)

References 43 publications

(83 reference statements)

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“…Failure of initial surgical intervention such as enucleation or partial pancreatectomy and medical therapy such as diazoxide, somatostatin analogs, or everolimus necessitating definitive surgery for recurrent insulinomatosis is seen in 95% of cases reviewed. 10,14 Octreotide was not attempted in our patient because of her severe hypoglycemia but has been noted to help in other cases. 11,13,16 As our case demonstrates, magnetic resonance imaging is not always the best test for diagnosis of recurrence of insulinomas; EUS is an accurate modality for diagnosis.…”

Section: Discussionmentioning

confidence: 94%

“…9 Medical management includes diazoxide, somatostatin analogs, or everolimus; verapamil, steroids, and phenytoin have also been used; however, in 95% of cases, surgical management is ultimately required. [10][11][12][13][14][15][16] We present a case of recurrent insulinomatosis requiring total pancreatectomy after recurrence of disease almost 20 years after enucleation and failure of medical management without any known underlying familial genetic predisposition.…”

Section: Introductionmentioning

confidence: 99%

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The Medical and Surgical Management of Recurrent Multicenter Insulinomatosis Without Known Genetic Predisposition

Jaquillard,

Pusateri,

Krishna

2024

ACG Case Rep J

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Insulinomas are rare neuroendocrine tumors characterized by episodic hypoglycemia. Typically, insulinomas are benign, solitary, intrapancreatic, and measure less than 2 cm in diameter. When insulinomas are multicenter or recurrent, they are often associated with genetic conditions such as multiple endocrine neoplasia type 1, neurofibromatosis type 1, or von Hippel-Lindau disease. Most insulinomas can be resolved with surgery. Multicenter and recurrent insulinomas, known as insulinomatosis, may require additional medical and surgical management. We report a distinctive case involving recurrent multicenter insulinomatosis devoid of any identified genetic familial predisposition. The patient's complex medical history spans nearly 2 decades, marked by unsuccessful attempts at resolution through surgical enucleation and noninvasive medical management, culminating in the decision for total pancreatectomy.

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Section: Discussionmentioning

confidence: 94%

Section: Introductionmentioning

confidence: 99%

The Medical and Surgical Management of Recurrent Multicenter Insulinomatosis Without Known Genetic Predisposition

Jaquillard,

Pusateri,

Krishna

2024

ACG Case Rep J

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“…Insulinomatosis was a benign disease in all patients except one who presented with metastatic disease ( 8 ). After this description, others emerged describing new cases, all highlighting that, in insulinomatosis, the recurrence rate of hypoglycemia after surgery is high ( 16 – 19 ), contrasting with the absence of recurrent hypoglycemia in sporadic well-differentiated insulinomas ( 8 ) and with a low recurrence rate of hypoglycemia, even with high rates of tumoral recurrence, in cases with MEN1-related insulinoma undergoing pancreatic surgery (~7%) ( 6 – 8 , 20 , 21 ).…”

Section: Discussionmentioning

confidence: 99%

Case report: Insulinomatosis: description of four sporadic cases and review of the literature

Lourenço,

Corrêa-Giannella,

Siqueira

et al. 2024

Front. Endocrinol.

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The best-known etiologies of hyperinsulinemic hypoglycemia are insulinoma, non-insulinoma pancreatogenous hypoglycemic syndrome, autoimmune processes, and factitious hypoglycemia. In 2009, a disease not associated with classic genetic syndromes and characterized by the presence of multiple pancreatic lesions was described and named insulinomatosis. We present the clinical and pathologic features of four patients with the diagnosis of insulinomatosis, aggregated new clinical data, reviewed extensively the literature, and illustrated the nature and evolution of this recently recognized disease. One of our patients had isolated (without fasting hypoglycemia) postprandial hypoglycemia, an occurrence not previously reported in the literature. Furthermore, we reported the second case presenting malignant disease. All of them had persistent/recurrent hypoglycemia after the first surgery even with pathology confirming the presence of a positive insulin neuroendocrine tumor. In the literature review, 27 sporadic insulinomatosis cases were compiled. All of them had episodes of fasting hypoglycemia except one of our patients. Only two patients had malignant disease, and one of them was from our series. The suspicion of insulinomatosis can be raised before surgery in patients without genetic syndromes, with multiple tumors in the topographic investigation and in those who had persistent or recurrent hypoglycemia after surgical removal of one or more tumors. The definitive diagnosis is established by histology and immunohistochemistry and requires examination of the “macroscopically normal pancreas.” Our case series reinforces the marked predominance in women, the high frequency of recurrent hypoglycemia, and consequently, a definitive poor response to the usual surgical treatment.

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Complete remission of recurrent multiple insulin-producing neuroendocrine tumors of the pancreas with somatostatin analogs: a case report and literature review

et al. 2022

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Hyperinsulinemic hypoglycemia is most commonly caused by a single, sporadic insulinoma. Multicentric insulinoma disease (insulinomatosis) as well as metachronous neuroendocrine tumors of the pancreas, known also as neuroendocrine adenomatosis, represent a very rare condition, if not associated with multiple endocrine neoplasia type 1 syndrome (MEN1) or Von Hippel Lindau disease. We report a 9-year follow-up of a 41-year-old woman, initially presenting with hypoglycemic syndrome caused by two insulin-producing tumors, who underwent subtotal pancreasectomy in 2012, with histology compatible with multiple small neuroendocrine tumors. An approximately 1-cm insulin-producing tumor recurred at subsequent biochemical and radiological follow-up, and was cured with the somatostatin analog octreotide as a single treatment, until remission of symptoms and complete regression of the pancreatic lesion achieved after only 16 months of treatment. The possible mechanisms for these findings are discussed and the literature is briefly reviewed.

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Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático

Cited by 3 publications

References 43 publications

The Medical and Surgical Management of Recurrent Multicenter Insulinomatosis Without Known Genetic Predisposition

The Medical and Surgical Management of Recurrent Multicenter Insulinomatosis Without Known Genetic Predisposition

Case report: Insulinomatosis: description of four sporadic cases and review of the literature

Complete remission of recurrent multiple insulin-producing neuroendocrine tumors of the pancreas with somatostatin analogs: a case report and literature review

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