Nonfunctional neuroendocrine tumor of the pancreas: Case report and review of the literature
ABSTRACT INTRODUCTION: Pancreatic neuroendocrine tumors (PNET) have an incidence of one per 100,000 individuals per year. They represent about 1-2% of all pancreatic tumors. PNETs are a heterogeneous group with various clinical presentations and lineage. Non functional PNET (NF-PNET) are incidentally discovered in most cases. This article presents a review of NF-PNET and the case of a patient with this disease, in addition to its diagnosis, clinical presentation, and treatment. PRESENTATION OF CASE: The patient is a 37-year-old asymptomatic man who was sent from his reference unit because of a pancreatic tumor that was visualized incidentally during a laparotomy performed three months before due to an acute abdomen secondary to blunt abdominal trauma. A CT scan was requested that showed a retroperitoneal mass 7.5cm×6.6cm×7cm with infiltration of the duodenal wall. Endoscopy was performed, which reported a duodenal ulcer with nonconclusive histological findings. A percutaneous biopsy was obtained out and a diagnosis of a neuroendocrine tumor was made. Chemotherapy was started because infiltration of the portomesenteric axis was suspected. The patient presented signs of toxicity during his third cycle and therefore was scheduled for exploratory laparotomy. Pancreatoduodenectomy was performed with a histologic diagnosis of a pancreatic neuroendocrine tumor. DISCUSSION: The presentation of a NF-PNET is nonspecific. They continue to be tumors with a low incidence and few studies directed toward early detection and management have been carried out. Currently, CT scans are the studies most used for detection. CONCLUSION: Surgical treatment is preferred in patients without evidence of unresectability with longer survival. The characteristics of NF-PNETs make their detection difficult and new strategies are needed for early detection and management. New studies in early stages with new cytotoxics or analogs are promising.
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ABSTRACT: Purpose Rare endocrine–metabolic diseases (REMD) represent an important area in the field of medicine and pharmacology. The rare diseases of interest to endocrinologists involve all fields of endocrinology, including rare diseases of the pituitary, thyroid and adrenal glands, paraganglia, ovary and testis, disorders of bone and mineral metabolism, energy and lipid metabolism, water metabolism, and syndromes with possible involvement of multiple endocrine glands, and neuroendocrine tumors. Taking advantage of the constitution of a study group on REMD within the Italian Society of Endocrinology, consisting of basic and clinical scientists, a document on the taxonomy of REMD has been produced. Methods and results This document has been designed to include mainly REMD manifesting or persisting into adulthood. The taxonomy of REMD of the adult comprises a total of 166 main disorders, 338 including all variants and subtypes, described into 11 tables. Conclusions This report provides a complete taxonomy to classify REMD of the adult. In the future, the creation of registries of rare endocrine diseases to collect data on cohorts of patients and the development of common and standardized diagnostic and therapeutic pathways for each rare endocrine disease is advisable. This will help planning and performing intervention studies in larger groups of patients to prove the efficacy, effectiveness, and safety of a specific treatment.Journal of endocrinological investigation 11/2014; 38(2). DOI:10.1007/s40618-014-0202-6 · 1.55 Impact Factor