Retrospective Review of Adrenalectomy in a Tertiary Center in Nepal

Introduction: Adrenal gland surgery is a challenging art as it encompasses diverse physiological derangements which are even life-threatening. Also, it remains a medical finesse to rightly identify the correct patient who will benefit from surgery which include strong biochemical and radiological resource. Soundly performed surgery will lead to oncologic and homeostatic correction leading to near normal course. Methods: This is a retrospective analysis of adrenalectomy cohort over three years in a tertiary center in Nepal identifying diagnosis, preoperative management and postoperative course. The objective of this study is to review the presentation, histopathology and clinical outcomes of patients operated for adrenal mass in our center. Results: There were 42 surgeries performed via open approach. The number of adenoma, phaeochromocytoma, carcinoma and non-functioning tumor were 13, 16, 12 and one respectively. In most of the hypertensive patients, control was achieved with minimal drug and recurrences were observed in 25% of carcinoma in postoperative follow up. Conclusion: A large proportion of operative adrenal mass are observed clinically in our setup. The surgical outcome is satisfactory but judicious perioperative management is essential.

Genetic predictors of insulin-producing pancreatic tumor

Insulinoma is the most common functioning tumor of the pancreas. Approximately 5% of its cases are associated with the multiple endocrine neoplasia syndrome type 1 (MEN1), caused by mutation in the MEN1 gene. MEN1 can be manifested by pituitary and parathyroid adenomas, pancreatic neuroendocrine tumors, tumors of the thyroid gland, adrenals, intestine, carcinoids of lungs and other organs. However, in 5–10% of the patients with clinical manifestation of this syndrome, MEN1 mutations cannot be identified. Moreover, the disease can be caused by various abnormalities (mutations, polymorphisms, etc.) in other genes. More than 30 genes, associated with insulin-producing pancreatic tumors, have been described in the literature. With a known germinal mutation, the prognosis and management of patients with insulinoma can be determined by the hereditary disease with which the tumor is associated. The article emphasizes the need to search for new genetic markers that predispose to the development of insulinoma. The necessity of extended genetic testing of patients with insulinomas is discussed, primarily of young patients with multifocal lesions, family history and associated disorders.

The hypoglicemic syndrome (insulinoma): topical, pathomorphological, and genetic diagnostics and treatment (review, part 2)

Insulinoma is the most common functioning tumor of the pancreas. It consists of β-cells of the Langerhans islets. Insulinoma is malignant in 10% of cases and multifocal in 10% of cases. This review discusses the new capabilities for identification of the exact location of insulinoma, in particular given the latest data on expression of different receptors in the tumor tissue. We discuss the modern concept of molecular and genetic aspects of insulinoma development. In particular, we analyze studies on germinal and somatic mutations and epigenetic abnormalities. We describe new biochemical markers of insulinoma, which may be used for differential diagnosis, timely detection of insulinoma recurrence and progression, individual prognosis, and monitoring of treatment. Also, we discuss application of antitumor medications and optimal drug correction of hypoglycemia in insulinoma patients.