Introduction: Insulinomas are the most common form of functioning pancreatic neuroendocrine tumors (NET) with an estimated incidence of 13/million per year. Less than 10% are malignant and rarely with distant metastases, carrying a poor prognosis.
Case report: We report a case of a 73-years-old woman attended at our ER for recurrent syncope, with irrelevant medical history. Several radiology exams were preformed, revealing numerous liver metastases and a probable metastatic lesion in L3 vertebral body, justifying a complete investigation for occult malignancy. The chest, pelvis and abdominal CT scan showed a lesion with tumor-like characteristics in the tail of the pancreas. The PET scan, bone scintigrafy and somatostatin receptor scintigrafy also suggested extensive metastization in the liver, cervical spine and both humerus of a primitive NET of the pancreas. Biopsy of a hepatic metastatic nodule was preformed, and the anatomopathological examination confirmed the diagnosis of a well differentiated NET (G2-WHO 2010(Ki-67 index of 6%)). The laboratory findings were:chromogranin A 91 nmol/l (<6.0), gastrin 392 pg/ml (<90.0), fasting insulin 34 μUI/l (<30), random insulin 54 μUI/l for a random glycemia of 33 mg/dl. Other biochemical tests were unremarkable and the screening for MEN1 was negative.
The patient was discharged under octreotide 100 μg, t.i.d. but kept frequent and severe hypoglycemias which motivated a re-admittance. In spite of an intensive treatment with octreotide 100 μg, t.i.d., diazoxide 100 mg, t.i.d., dexamethasone 0.5 mgq.d. and isotonic or hypertonic continuous glucose solutions, the patient kept severe hypoglycemia and chemotherapy was proposed because there was no surgical eligibility. After three cycles of chemotherapy(streptozocin 725 mg combined with epirubicin 87 mg), under monthly octreotide 30 mg, diazoxide 100 mg, t.i.d., prednisolone 20 mg, q.d. and a nutritional plan, the patient was discharged and remains with no further hypoglycemia or pain complaints (ECOG 0).
Conclusion: The rarity of this case and the absence of clear guidelines illustrate the difficulties in its treatment and the need for a multidisciplinary approach. The severity of the patients symptoms imposed the use of multiple therapies and the option of a classic systemic chemotherapy, with good results. In spite of the poor prognosis carried by an advanced malignancy (T2NxM1 ENETS and AJCC/UICC 2011), the patient is asymptomatic and has a good quality of life.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.
05 - 09 May 2012
European Society of Endocrinology