Perioperative Management of a Rare Pancreatic Insulinoma Resection: Anaesthetic Challenge!

Insulinoma is a rare, mostly benign and solitary neuroendocrine tumour of the β-cells of islets of langerhans of pancreas. Clinically it presents with a classical ‘Whipple Triad’ encompassing symptomatic hypoglycemia, fasting hypoglycemia (<50 mg/dl) and immediate relief of symptoms after glucose administration. Definitive treatment is laparoscopic or open surgical excision of the tumour. We report and discuss the distinctive anaesthetic considerations and implications during perioperative period. A comprehensive approach including preoperative optimization of blood glucose levels with various drugs and dietary modifications, scrupulous hemodynamic and blood sugar monitoring with prompt initiation of dextrose infusion during surgical handling of tumour and meticulous management of rebound hyperglycemia with insulin infusion in postoperative period remains the essence for better outcome in these subset of patients.

Multiple endocrine neoplasia syndrome

Background. Multiple endocrine neoplasia type 1 is a rare inherited disease that combines heterogeneous autosomal inherited disorders, the pathogenesis of which is based on hyperplasia or tumor transformation of several neuro- endocrine glands. In this pathology, treatment protocols are insufficient. Surgical treatment, suppressive or replacement therapy, radiation and chemotherapy are mainly used. We presented a successful experience of combined radiotherapy of multiple pancreatic insulinoma as the main clinical manifestation of the syndrome of multiple endocrine neoplasia. The combination of intra-tissue brachytherapy and megavolt remote therapy in TFD, isoeffective 50 Gy, allowed to achieve reliable control of hypoglycemic attacks within 2 years of follow-up. Purpose – to acquaint the medical community with successful experience of combined radiation treatment of multiple pancreatic insulinoma as a main clinical manifestation of the multiple endocrine neoplasia syndrome. Materials and Methods. Clinical case of patient L., 30 years old, who received combined radiation therapy for clinically significant manifestations of hormonally active multiple insulinoma of the pancreas in the Department of Radiological Oncology of State Organization «Grigoriev Institute for Medical Radiology and Oncology of the National Academy of Medical Sciences of Ukraine». Results. In May 2019, palliative contact brachytherapy was performed on the MULTISOURCE device. Total focal dose (TFD) for cells in the pancreas GENUS 3 Gy 5 times a week, TFD 18 Gy. At the end of brachytherapy, attacks of hypoclycemia were stopped. In March 2020, remote radiation therapy was performed on a linear accelerator CLINAC-600C (6MeV) in the mode of classical fractionation (single focal dose (SFD) 1.8 Gy 5 times a week), TFD for the entire volume of the pancreas 30.6 Gy. In May 2021, CT scan showed that the process in the pancreas was stabilized, but in connection with the resumption of hypoglycemia and the emergence of dyshormonal osteopathy, a course of remote radiation therapy for the entire pancreas, SFD 1.8 Gy, TFD 20 Gy simultaneously with the introduction of bisphosphonates. Conclusions. Our patient demonstrates type I syndrome of multiple neo- plasms, neuroendocrine tumor of pancreas G1 T3N0M0 IIB stage. Due to multi-stage combined radiation therapy for 2 years it is possible to achieve stabilization of the process and reliable control of hypoglycemic attacks, which indicates the effectiveness of this method as an alternative to surgery in unresectable cases.

Carcinoid Syndrome and Hyperinsulinemic Hypoglycemia Associated with Neuroendocrine Neoplasms: A Critical Review on Clinical and Pharmacological Management

The carcinoid syndrome (CS) and hyperinsulinemic hypoglycemia (HH) represent two of the most common clinical syndromes associated with neuroendocrine neoplasms (NENs). The former is mainly related to the serotonin secretion by a small bowel NEN, whereas the latter depends on an insulin hypersecretion by a pancreatic insulinoma. Both syndromes/conditions can affect prognosis and quality of life of patients with NENs. They are often diagnosed late when patients become strongly symptomatic. Therefore, their early detection and management are a critical step in the clinical management of NEN patients. A dedicated and experienced multidisciplinary team with appropriate therapeutic strategies is needed and should be encouraged to optimize clinical outcomes. This review aims to critically analyze clinical features, evidence and treatment options of CS and HH and therefore to improve their management.

Case report of a pancreatic insulinoma misdiagnosed as epilepsy

A 55-year-old patient had spent 12 years with unexplained seizures, initially diagnosed as epilepsy and then as a psychiatric disorder. When she was admitted with hypoglycaemia, a fasting test was performed showing blood sugar levels as low as 1 mmol/L with symptoms of neuroglycopenia. Insulinoma was suspected and an MRI showed a large tumour in the tail region of the pancreas. A Dodecanetetraacetic acid-Tyr3-octreotate (DOTATATE) positron emission tomography CT indicated no malignancy and showed no signs of metastasis. The patient underwent surgery, leaving her asymptomatic.