Multiple Proinsulin‐Secreting Tumors of the Pancreas Treated by Laparoscopic Distal Pancreatectomy and Splenectomy

Clark, Gary M.; Sadur, C N; Puligandla, Balaram

doi:10.1007/s00268-008-9772-9

Cited by 10 publications

(4 citation statements)

References 18 publications

(14 reference statements)

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“…their plasma glucose concentrations remained above 3.0 mmol/l at the end of the fast, suggesting that there was little or no secretion of insulin by the tumour during the 72-h fasting test. Similar cases of low-secreting EHH have been reported previously, mainly in patients with nesidioblastosis (12,13). In the Mayo Clinic study, three insulinoma patients with postprandial hypoglycaemia had negative fasting tests (2).…”

Section: Discussionsupporting

confidence: 80%

Increased plasma β-hydroxybutyrate levels during the fasting test in patients with endogenous hyperinsulinaemic hypoglycaemia

Buffet

Vezzosi

Maïza

et al. 2013

European Journal of Endocrinology

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Objective: The objective of the present study was to determine whether a plasma b-hydroxybutyrate (BOHB) level O2700 mmol/l during the 72-h fasting test is sufficient to rule out the diagnosis of endogenous hyperinsulinaemic hypoglycaemia (EHH). Research design and methods: We retrospectively studied BOHB levels in 39 patients with EHH who had undergone a 72-h fasting test to make the diagnosis of EHH, and we compared EHH patients with BOHB levels O2700 mmol/l (group 1), EHH patients with BOHB levels !2700 mmol/l (group 2) and 59 controls (median glycaemia: 3.2 mmol/l and median BOHB: 6095 mmol/l). Results: During a 72-h fasting test, nine patients (group 1) had BOHB levels O2700 mmol/l (median 6140 and range 2957-7824) and 30 patients (group 2) had BOHB levels !2700 mmol/l (median 542 and range 0-2607). In group 1, four patients had undergone partial pancreatectomy previously and were evaluated for the recurrence of hypoglycaemia, whereas none of the group 2 patients had been operated. The duration of the fasting test was longer in group 1 than in group 2 (P!0.0001), and at the end of the fasting test, plasma glucose concentrations were not significantly different (PZ0.0617), but insulin (PZ0.004), C-peptide (PZ0.0015) and proinsulin (PZ0.0038) levels were significantly lower in group 1 patients than in group 2 patients, suggesting lower insulin secretion and/or impaired glycaemic counter-regulation. Conclusion: During a fasting test, a BOHB level O2700 mmol/l is observed in some EHH patients, suggesting that BOHB levels cannot rule out the recurrence of EHH, in particular, after partial pancreatectomy.European Journal of Endocrinology 169 91-97

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Section: Discussionsupporting

confidence: 80%

Increased plasma β-hydroxybutyrate levels during the fasting test in patients with endogenous hyperinsulinaemic hypoglycaemia

Buffet

Vezzosi

Maïza

et al. 2013

European Journal of Endocrinology

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“…However, the proinsulin continuously produced by a proinsulinoma is thought to play a role similar to the insulin produced by an insulinoma. Tumors that secrete proinsulin alone, such as that observed in our patient, are very rare (6)(7)(8)(9)(10). In the present case, the immunohistochemical examination did not confirm the production of insulin by the tumors, including the original tumor as well as the liver and lung metastases.…”

Section: Discussioncontrasting

confidence: 75%

The Transformation of a Nonfunctioning Islet Cell Tumor of the Pancreas into a Proinsulinoma under Conditions of Lung Metastasis

Yoshioka

Shibata²,

Uchinami

et al. 2015

Intern. Med.

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We herein report the first case of a nonfunctioning islet cell tumor that transformed into a proinsulinoma during the process of metastasis to the lungs. This phenomenon was confirmed in a 69-year-old woman with an advanced pancreatic islet cell tumor and multiple liver metastases who later developed multiple lung metastases. She underwent pancreatic resection followed by the administration of chemotherapy and survived for seven years. Although the patient initially had hyperglycemia due to diabetes mellitus, she conversely began to manifest hypoglycemic attacks 63 months postoperatively with the concomitant development of multiple lung metastases. An autopsy revealed that only the tumor in the lungs produced proinsulin; no other hormones were detected.

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“…Thus, for insulinoma, if the lesion is clearly localised before surgery, and is near or at the surface of the pancreas and easily defined at surgery, enucleation may be sufficient, provided histopathology demonstrates complete excision and benign features 217. However, this may not be possible, and Kausch–Whipple pancreatoduodenectomy, left pancreatectomy or even total pancreatectomy may be justified in selected cases 218. These operations are also applied to selected cases with localised disease arising from other functioning, as well as non-functioning, NETs of the pancreas 219…”

Section: Treatmentmentioning

confidence: 99%

Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

Ramage

Ahmed

Ardill

et al. 2011

Gut

635

454

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These guidelines update previous guidance published in 2005. They have been revised by a group who are members of the UK and Ireland Neuroendocrine Tumour Society with endorsement from the clinical committees of the British Society of Gastroenterology, the Society for Endocrinology, the Association of Surgeons of Great Britain and Ireland (and its Surgical Specialty Associations), the British Society of Gastrointestinal and Abdominal Radiology and others. The authorship represents leaders of the various groups in the UK and Ireland Neuroendocrine Tumour Society, but a large amount of work has been carried out by other specialists, many of whom attended a guidelines conference in May 2009. We have attempted to represent this work in the acknowledgements section. Over the past few years, there have been advances in the management of neuroendocrine tumours, which have included clearer characterisation, more specific and therapeutically relevant diagnosis, and improved treatments. However, there remain few randomised trials in the field and the disease is uncommon, hence all evidence must be considered weak in comparison with other more common cancers.

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Multiple Proinsulin‐Secreting Tumors of the Pancreas Treated by Laparoscopic Distal Pancreatectomy and Splenectomy

Abstract: The measurement of proinsulin plays an important part in the diagnostic workup of neuroendocrine tumors causing hypoglycemia. These potentially malignant tumors can be treated adequately with minimally invasive surgery.

Cited by 10 publications

References 18 publications

Increased plasma β-hydroxybutyrate levels during the fasting test in patients with endogenous hyperinsulinaemic hypoglycaemia

Increased plasma β-hydroxybutyrate levels during the fasting test in patients with endogenous hyperinsulinaemic hypoglycaemia

The Transformation of a Nonfunctioning Islet Cell Tumor of the Pancreas into a Proinsulinoma under Conditions of Lung Metastasis

Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

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