A Clinicopathological Study of Malignant Insulinoma in a Contemporary Series

Yu, Run; Nissen, Nicholas N.; Hendifar, A.; Tang, Longwen; Song, Yu‐Li; Chen, Yuan‐Jia; Fan, Xuemo

doi:10.1097/mpa.0000000000000718

Cited by 21 publications

(23 citation statements)

References 35 publications

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“…The relatively low proliferative index of the primary tumour could explain the delayed secondary presentation 14 years later. In addition, the functional transformation (non-functional to insulin secreting) is in line with the previous case reports ( 6 , 7 , 8 , 9 , 10 , 11 ), albeit remaining a rare phenomenon. Alternatively, the development of a metastatic insulin-secreting pNET after a previous successful resection of a non-functional pNET could have been a coincidence, with genetic instability contributing to it.…”

Section: Discussionsupporting

confidence: 91%

“…Two cases have been described by Yu et al ( 6 ), one of which included a patient with grade 2 pNET with lymph node metastases who underwent pancreatico-duodenectomy and received adjuvant chemotherapy, but represented 2 years later with hepatic metastases. She finally showed symptoms of hypoglycaemia 7 years after her original diagnosis, although she received continuous treatment (chemotherapy) over this time-frame.…”

Section: Discussionmentioning

confidence: 99%

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Metastatic insulinoma presenting 14 years after benign tumour resection: a rare case and management dilemma

Keen

Iqbal

Owen

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary We present a 60-year-old woman who underwent successful surgical resection (partial pancreatectomy) for a low grade non-functioning pancreatic neuroendocrine tumour (pNET), with no biochemical or radiological features of recurrence on follow-up visits for 5 years. Fourteen years after the initial surgery, she developed spontaneous severe hypoglycaemic episodes which required hospitalisation, with subsequent investigations confirming the diagnosis of a metastatic insulin-secreting pNET (insulinoma). Medical management of her severe spontaneous hypoglycaemic episodes remained challenging, despite optimum use of diazoxide and somatostatin analogue therapy. Based on a discussion at the regional neuroendocrine tumour multidisciplinary team meeting, she underwent an elective hepatic trans-arterial embolization which was unfortunately unsuccessful. She ended up requiring an emergency right hemihepatectomy and left retroperitoneal mass resection which finally stabilised her clinical condition. Learning points: Ours is only the seventh case report of a previously benign pNET presenting as a functional insulin secreting metastatic tumour. However, it is the first case report, in which the metastatic functional pNET presented after such a long hiatus (14 years). There is currently no clear consensus regarding the length of follow-up of non-functional pNET which are deemed cured post-surgical resection, with most guidelines advocating a median follow up of 5 years (1). The delayed presentation in our case suggests additional considerations should be made regarding optimal post-operative surveillance duration based on the age of the patient, location of the tumour, lymph node spread and Ki-67 index. Hepatic artery embolization and/or partial hepatectomy remains a treatment option for pNET patients with significant hepatic metastasis.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Metastatic insulinoma presenting 14 years after benign tumour resection: a rare case and management dilemma

Keen

Iqbal

Owen

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…We searched the PubMed database, and to the best of our knowledge, we collected the clinical, pathologic, and biochemical data of all previously reported cases of malignant insulinoma in patients with DM ( Table 1). As reported by Yu et al [26], short hypoglycemic symptoms duration before diagnosis of tumor seems to be common in patients with malignant insulinoma (median duration: 30 days). Furthermore, as shown in the same study, high proinsulin and CgA levels and a large proinsulin/insulin molar ratio should raise the suspicion of malignant insulinoma in patients with hyperinsulinemic hypoglycemia.…”

Section: Discussionmentioning

confidence: 59%

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Ciacciarelli

Caruso

Rengo

et al. 2020

Case Reports in Endocrinology

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Insulinoma is an insulin-producing pancreatic neuroendocrine tumor that can be malignant in about 10% of cases. Locoregional invasion, lymph node metastases, or remote metastases are the main criteria of malignant insulinoma. Its incidence in patients with pre-existing diabetes mellitus (DM) is exceptionally rare. In this report, we describe a 66-year-old man with long-standing type 2 DM who presented with recurrent episodes of diaphoresis due to severe hypoglycemia despite the withdrawal of insulin therapy, hypercalcitoninemia, and biochemical and radiological findings suggestive of metastatic malignant insulinoma. Unfortunately, after few days of diazoxide treatment, edema, hypotension, oliguria, and water retention were observed, patient's clinical status deteriorated rapidly, and he died in our department from acute renal failure.

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“…The diagnostic criteria for PNETs were identical to those used in our previous studies 29, 55, 56 . For example, the clinical and laboratory diagnostic criteria of insulinoma included symptoms of hypoglycemia, hypoglycemia (serum levels of glucose <50 mg/dl), hyperinsulinemia (elevated serum levels of insulin or high serum levels of proinsulin at time of hypoglycemia), please see our previously published paper 55 and most recently published paper 57 . The pathological diagnosis of all PNETs was made by 2 experienced pathologists.…”

Section: Methodsmentioning

confidence: 99%

Prognostic relevance of UCH-L1 and α-internexin in pancreatic neuroendocrine tumors

Song

Qiao

et al. 2017

Sci Rep

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Prognostic biomarkers for the pancreatic neuroendocrine tumors are needed. Proteomic study on insulinoma has been rarely reported. We identified the differential expression of proteins between insulinoma and their paired tissues by proteomic analysis, and evaluated the prognostic significance of specific proteins in pancreatic neuroendocrine tumors including insulinoma. The differential expression of select proteins was validated in more than 300 tumors using immunohistochemical staining and western blot. Methylation of UCH-L1 promoter in tumors was examined by methylation specific PCR and validated by sequencing. The concurrent expression of UCH-L1 and α-internexin was correlated with the prognosis in 2 independent collectives of patients with tumors. Sixty-two and 219 proteins were significantly down-regulated and up-regulated in insulinomas, respectively. Demethylation of UCH-L1 promoter was associated with UCH-L1 expression in tumors (p = 0.002). The concurrent expression of UCH-L1 and α-internexin in pancreatic neuroendocrine tumors was significantly associated with better overall survival and disease-free survival in the combination of both cohorts (log rank p = 3.90 × 10−4 and p = 3.75 × 10−5, respectively) and in each of cohorts. The prognostic value of both proteins was also validated in patients with stage II and III tumors (p = 0.017 and p = 0.006, respectively). The proteins UCH-L1 and α-internexin could be independent prognostic biomarkers of pancreatic neuroendocrine tumors.

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A Clinicopathological Study of Malignant Insulinoma in a Contemporary Series

Abstract: Malignant insulinoma seems to arise from and behave like nonfunctioning pancreatic neuroendocrine tumor oncologically but with metachronous hyperinsulinemic hypoglycemia. High proinsulin levels and proinsulin/insulin molar ratio may suggest malignant insulinoma.

Cited by 21 publications

References 35 publications

Metastatic insulinoma presenting 14 years after benign tumour resection: a rare case and management dilemma

Metastatic insulinoma presenting 14 years after benign tumour resection: a rare case and management dilemma

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Prognostic relevance of UCH-L1 and α-internexin in pancreatic neuroendocrine tumors

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