Metastatic insulinoma in an adult patient with underlying nesidioblastosis

Rosman, Jonathan; Bravo-Vera, Rafael; Sheikh, Arsalan; Gouller, Alina

doi:10.1007/bf03346338

Cited by 10 publications

(13 citation statements)

References 3 publications

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“…The association of these two entities is a rare clinical finding. To the best of our knowledge, the association between nesidioblastosis and neoplastic diseases of the endocrine pancreas has been described in just a few cases (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). The peculiarity of our case is that the diagnoses of nesidioblastosis and insulinoma were not concomitant.…”

Section: Discussionmentioning

confidence: 53%

Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

et al. 2020

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Section: Discussionmentioning

confidence: 53%

Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

et al. 2020

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“…Nineteen cases of WD-PNET and β-cell nesidioblastosis arising in the same patient have been described previously 4,6-20 (Table 1). Thirteen of the 19 cases occurred in women.…”

Section: Discussionmentioning

confidence: 95%

Concurrent Adult-Onset Diffuse β-Cell Nesidioblastosis and Pancreatic Neuroendocrine Tumor: A Case Report and Review of the Literature

2019

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Nesidioblastosis is an uncommon cause of organic persistent hyperinsulinemic hypoglycemia in adults. We report a case of adult-onset diffuse β-cell nesidioblastosis in a 49-year-old woman who was status-post Roux-en-Y gastric bypass and distal pancreatectomy for a well-differentiated pancreatic neuroendocrine tumor. While the neuroendocrine tumor was suspected to be an insulinoma, persistent hypoglycemia postoperatively suggested either incomplete resection or a second pancreatic neoplasm. Completion pancreatectomy revealed islet β-cell hyperplasia and nuclear pleomorphism consistent with β-cell nesidioblastosis. The patient’s blood glucose levels normalized after completion pancreatectomy. While β-cell nesidioblastosis and insulinomas can coexist in the same patient, pathologists should be aware of β-cell nesidioblastosis as a potential cause for hyperinsulinemic hypoglycemia and should exclude it in patients who have not shown definitive clinical response after surgical excision of a pancreatic neuroendocrine tumor.

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“…reported [8][9][10][11][12][13][14][15][16][17][18]. In the literature review , we collected a total of 13 patients (our included) which presented islet cell tumor associated with nesidioblastosis [8][9][10][11][12][13][14][15][16][17][18] (Table I).…”

Section: Opis Przypadkumentioning

confidence: 99%

“…In the literature review , we collected a total of 13 patients (our included) which presented islet cell tumor associated with nesidioblastosis [8][9][10][11][12][13][14][15][16][17][18] (Table I). There were 10 females and 3 males with a mean age of 43.2 ± 9.1 years.…”

Section: Opis Przypadkumentioning

confidence: 99%

Przetrwała hipoglikemia hiperinsulinemiczna współistniejąca z nieaktywnym wyspiakiem trzustki

Valli

Blandamura

Pastorelli³

et al. 2015

Endokrynologia Polska

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The most common cause of hyperinsulinaemic hypoglycaemia in adult is insulinoma. Although nesidioblastosis is a rare but well-recognised disorder of persistent hypoglycaemia in infants, it is extremely rare in adults. We present a case of a 59-year-old woman with small neuroendocrine tumour of the tail of the pancreas, diagnosed by CT scans and MRI, and hypoglycaemic syndrome. Laparoscopic distal pancreatectomy was performed, and pathologic examination showed a well-differentiated, non-functioning endocrine tumour of the pancreas and diffuse nesidioblastosis in the remnant gland. In the early postoperative period, recurrent hypoglycaemia occurred in spite of oral diazoxide therapy. Plasma proinsulin levels were extremely high. 18F-DOPA positron emission tomography showed a pathologic uptake of tracer in the head and the uncinate process of the pancreas. Subtotal pancreatectomy was suggested but the patient refused operation: she is taking diazoxide 100 mg three times daily. Coexistence of nesidioblastosis with a neuroendocrine tumour makes preoperative diagnosis and management of severe hypoglycaemia more difficult. Nesidioblastosis should be considered in differential diagnosis of hypoglycaemic syndrome, but histological examination is necessary for a definitive tissue diagnosis. StreszczenieNajczęstszą przyczyną hipoglikemii hiperinsulinemicznej u osób dorosłych jest insulinoma. Chociaż przetrwała hipoglikemia hiperinsulinemiczna to rzadkie, lecz łatwo rozpoznawalne zaburzenie przetrwałej hipoglikemii u noworodków, bardzo rzadko występuje u osób dorosłych. Autorzy badania przedstawiają przypadek 59-letniej kobiety z niewielkim guzem neuroendokrynnym ogona trzustki, zdiagnozowanym dzięki tomografii komputerowej oraz rezonansowi magnetycznemu, oraz zespołem hipoglikemicznym. Wykonano laparoskopową dystalną pankreatektomię, a badanie patologiczne wykazało wysoko zróżnicowanego, nieaktywnego, endokrynnego wyspiaka trzustki oraz rozlaną przetrwałą hipoglikemię hiperinsulinemiczną w pozostałej części gruczołu. We wczesnym okresie pooperacyjnym wystąpiła nawracająca hipoglikemia, mimo stosowania doustnego leczenia diazoksydem. Stężenie proinsuliny w osoczu był bardzo wysoki. Pozytonowa tomografia emisyjna z 18F-DOPA wykazała patologiczną absorpcję znacznika w głowie i wyrostku haczykowatym trzustki. Sugerowano subtotalną pankreatektomię, lecz pacjentka nie zgodziła się na operację: przyjmowała dawkę 100 mg diazoksydu trzy razy dziennie. Współistnienie przetrwałej hipoglikemii hiperinsulinemicznej z nowotworem neuroendokrynnym utrudnia diagnostykę przedoperacyjną i leczenie ciężkiej hipoglikemii. Przetrwała hipoglikemia hiperinsulinemiczna powinna być brana pod uwagę w diagnozie różnicującej zespół hipoglikemiczny, lecz badanie histologiczne jest niezbędne do definitywnej diagnozy tkanek. (Endokrynol Pol 2015; 66 (4): 356-360)

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Metastatic insulinoma in an adult patient with underlying nesidioblastosis

Abstract: Although this patient had nesidioblastosis, his recurrent hyperinsulinism was most likely secondary to metastatic insulinoma. Radiofrequency ablation was successfully used for palliative purposes in managing metastatic insulinoma refractory to medical therapy.

Cited by 10 publications

References 3 publications

Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

Nesidioblastosis and Insulinoma: A Rare Coexistence and a Therapeutic Challenge

Concurrent Adult-Onset Diffuse β-Cell Nesidioblastosis and Pancreatic Neuroendocrine Tumor: A Case Report and Review of the Literature

Przetrwała hipoglikemia hiperinsulinemiczna współistniejąca z nieaktywnym wyspiakiem trzustki

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