Introduction: Adult nesidioblastosis is a rare entity that has motivated the publication of several case reports, but long-term outcomes are rarely described. Distal or sub-total pancreatectomy is indicated in cases of severe symptoms or lack of response to medical treatment. We report the case of a patient with persistent nesidioblastosis after pancreatectomy keeping under medical treatment for 24 years.
Clinical case: Woman currently with 81-years old, diagnosed with diffuse nesidioblastosis at the age 57 (1991), after distal pancreatectomy performed on suspicion of insulinoma. Because of lack of response to medical treatment the patient underwent re-pancreatectomy 2 years after the first surgery (1993), but again showed no cure criteria. Since diagnosis to the current time, the patient still need medical treatment with two daily injections of octreotide. Diabetes mellitus was diagnosed 5 years after the near-total pancreatectomy (1998), with blood tests showing persistent hypoglycemia. In the last years, the patient maintains unstable glycemic control with hypoglycemia and hyperglycemia, with HbA1c between 6.3 and 9.3%; the attempts to reduce octreotide dose increase the frequency and severity of hypoglycemia. Today, with 81 years old, the patient is treated with octreotide 70 μg/day, keeping glycemic instability, but being clinically well, with no evidence of chronic complications of diabetes.
Discussion and conclusion: The definitive treatment of diffuse nesidioblastosis is difficult and imposes a real challenge to the endocrinologist. The prognosis depends on surgical treatment: if resection is not wide enough, the disease can persist; on the other hand, if it is too large there is a high risk of endocrine and exocrine pancreatic insufficiency. The case we report, despite the persistence of the disease and association with diabetes mellitus, has shown a good performance without major complications throughout these 24 years of follow-up.
17 - 19 Feb 2016
European Society of Endocrinology