Case reportManagement of a metastasized high grade insulinoma (G3) with refractory hypoglycemia: Case report and review of the literature
Introduction
Insulinomas are rare with an occurrence of approximately 4/1,000,000. The median age at diagnosis is around 47 with a slight female predominance [1]. Yet they are also the most common functioning neuroendocrine pancreatic tumor as well as the most common cause of hyperinsulinemic hypoglycemia in adults [2]. In general they are solitary and benign [3]. The proliferation rates reported in the literature range from Ki67 1–25% and grading usually corresponds to G1/2. To our knowledge only one case with a proliferation of 25% (G3) has been reported [4]. The present report describes a yet unreported case of a malignant, metastasized and functional active insulinoma with severe hypoglycemia and an unusually high proliferation rate with a Ki67 of 70%.
Section snippets
Case report
A 45 years old patient was admitted to hospital after a first episode of syncope. He presented blood glucose levels partly lower than 20 mg/dl without sufficient increase after oral administration of glucose. Abdominal ultrasound demonstrated multiple liver metastases and a biopsy was taken leading to the diagnosis of a high grade neuroendocrine tumor with a Ki67 rate of 70% (G3). Approximately 30% of the tumor cells showed intense insulin production (Fig. 1). An abdominal CT-Scan revealed a
Discussion
Herein, we report the rare case of a malignant insulinoma G3 with disseminated hepatic and bone metastases. In this case the clinical challenge proved to be the combination of an unusually high proliferation rate (Ki 67 of 70%) with massive insulin production, leading to a rapidly increasing tumor load and virtually uncontrollable hypoglycemia. In the literature various medical, surgical and interventional methods have been described to improve glycemic control by means of reducing tumor load
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Cited by (10)
Insulinoma: A retrospective study analyzing the differences between benign and malignant tumors
2018, PancreatologyCitation Excerpt :In our protocol, biochemical and imaging follow-up evaluations are done every 6 months in the first two years, every year for the next 3 years and be spread out to longer intervals if there is no evidence of tumor recurrence. The early identification of tumor recurrence that led to the adoption of several therapeutic measures (e.g. somatostatin analogues use, hepatic chemoembolization) [19,25] that could halt the progression of metastatic disease and improve the survival of the patients [20]. Our data help to base the literature about these tumors, reinforcing that although insulinoma is usually a single benign and surgically treated neoplasia, the malignant one is difficult to treat.
Emerging therapies for advanced insulinomas and glucagonomas
2023, Endocrine-Related CancerTreatment Landscape of Advanced High-Grade Neuroendocrine Neoplasms
2023, Clinical Advances in Hematology and OncologyManagement of Well-Differentiated High-Grade (G3) Neuroendocrine Tumors
2019, Current Treatment Options in Oncology
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