Clinical case of rare neuroendocrine tumor of pancreas - proinsulinoma

Abstract

Proinsulinoma is very rare neuroendocrine tumor of pancreas with proinsulin activity. Today there is no data about its true prevalence and occurrence within the syndrome of Multiple Endocrine Neoplasia (MEN) type 1. We have found no one clinical cases of the disease in available domestic literature, but only few clinical cases in foreign literature. Actually the management of patients with proinsulinoma is unknown, because such clinical guidelines are absent. Therefore, it is essential to describe particularly every new case of this neuroendocrine tumor in order to obtain sufficient data about proinsulinoma, because it is unclear whether the following management of such patients is correct or not. The clinical case of patient with proinsulinoma is described in the article. Atypical clinical presentation of hypoglycemia is reported. All stages of diagnostic research with main errors, the disputed key issues of follow up are discussed in detail, especially differential diagnosis of hypoglycemia in real clinical practice, screening of syndrome of MEN type 1 and the protocol of further observation.