Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia. NETs are generally subclassified by site of origin, stage, and histologic characteristics. Appropriate diagnosis and treatment of NETs often involves collaboration between specialists in multiple disciplines, using specific biochemical, radiologic, and surgical methods. Specialists include pathologists, endocrinologists, radiologists (including nuclear medicine specialists), and medical, radiation, and surgical oncologists. These guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine and adrenal tumors and are intended to assist with clinical decision-making. This article is focused on the 2021 NCCN Guidelines principles of genetic risk assessment and counseling and recommendations for well-differentiated grade 3 NETs, poorly differentiated neuroendocrine carcinomas, adrenal tumors, pheochromocytomas, and paragangliomas.

ADRENAL GLAND TUMOURS: A 2-YEAR TERTIARY CARE HOSPITAL BASED CASE SERIES

Introduction: Adrenal gland tumors are rare. Asymptomatic adrenal tumours are found in 2-10% of the population worldwide, out of which, adrenocortical carcinomas(ACC) have an incidence of 0.5-2 cases/million population/year. ACC accounts for 0.05% -0.2% of all malignancies with a bimodal age distribution, in the first two decades and then in the fifth decade. They can be asymptomatic and diagnosed as Incidentalomas or present with signs and symptoms of hormone imbalance. Materials and methods: In this 2 year hospital-based retrospective case series from June,2018 to May,2020 , 5 cases of adrenal gland tumors diagnosed in the Department of Pathology , GMCH, were retrieved from the archives and reviewed. Each case was analyzed with respect to age , sex, site and tumor characteristics. Results: In this 2-year period, 5 cases of adrenal tumors were found. The age range of the patients was 8-54 years. Out of 5 cases,2 cases were male and 3 cases were female. Out of 5 cases of , 4 were on the left side and 1 was on the right side.2 out of the 5 cases were diagnosed as Adrenocortical carcinoma(low grade), 1 as Phaeochromocytoma and 2 as Adrenocortical Adenoma. Conclusion: Adrenal gland tumors are rare, so the pathologic classification and determination of prognosis are very challenging.

PARTIAL LAPAROSCOPIC ADRENALECTOMY - ANATOMICAL BASIS AND OPERATION TECHNIQUE

The aim: To optimize the indications for partial laparoscopic adrenalectomy (PLA), to give a detailed outline of a PLA technique and to provide technical tips to ensure safe and highly-effectiveness, based on the knowledge of adrenal anatomy and blood supply. Materials and methods: Between January 2010 and September 2018, our department performed 47 adrenal glands surgeries. The operations included 29 total laparoscopic adrenalectomies (TLA), 4 open adrenalectomies (OA) and 14 partial laparoscopic adrenalectomies (PLA). Results: The histopathological examination of all operated patients detected 9 (19.1%) malignant tumors, including 5 metastatic tumors. Benign tumors includes 24 (63%) adenomas, 8 (21%) pheochromocytomas, 4 (10,5%) cysts and 2 (5,5%) ganglioneuromas. Post-PLA histopathological findings revealed 6 adenomas, 2 pheochromocytomas, 4 cysts and 2 ganglioneuromas. Conclusions: Keeping in mind anatomical features of adrenal gland blood supply in highly-selected patients, PLA can be performed in a number of patients. 2 mm of a minimal resection margin is enough to preserve a false tumor recurrence. Anatomically grounded PLA is becoming a new standard of benign adrenal gland tumors treatment, providing an opportunity to save more adrenal gland functional tissue and to prevent hipocorticism development in postoperative period.

Schwannoma Localized Retroperitoneally in a 14-Year-Old Boy

Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children.