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Endocrine Practice : Official Journal... Feb 2023To review the epidemiology, presentation, diagnosis, and management of head and neck paragangliomas. (Review)
Review
OBJECTIVE
To review the epidemiology, presentation, diagnosis, and management of head and neck paragangliomas.
METHODS
A literature review of english language papers with focus on most current literature.
RESULTS
Paragangliomas (PGLs) are a group of neuroendocrine tumors that arise in the parasympathetic or sympathetic ganglia. Head and neck PGLs (HNPGLs) comprise 65% to 70% of all PGLs and account for 0.6% of all head and neck cancers. The majority of HNPGLs are benign, and 6% to 19% of all HNPGLs develop metastasis outside the tumor site and significantly compromise survival. PGLs can have a familial etiology with germline sequence variations in different susceptibility genes, with the gene encoding succinate dehydrogenase being the most common sequence variation, or they can arise from somatic sequence variations or fusion genes. Workup includes biochemical testing to rule out secretory components, although it is rare in HNPGLs. In addition, imaging modalities, such as computed tomography and magnetic resonance imaging, help in monitoring in surgical planning. Functional imaging with DOTATATE-positron emission tomography, 18F-fluorodeoxyglucose, or 18F-fluorohydroxyphenylalanine may be necessary to rule out sites of metastases. The management of HNPGLs is complex depending on pathology, location, and aggressiveness of the tumor. Treatment ranges from observation to resection to systemic treatment. Similarly, the prognosis ranges from a normal life expectancy to a 5-year survival of 11.8% in patients with distant metastasis.
CONCLUSION
Our review is a comprehensive summary of the incidence, mortality, pathogenesis, presentation, workup and management of HNPGLs.
Topics: Humans; Fluorodeoxyglucose F18; Head and Neck Neoplasms; Paraganglioma; Paraganglioma, Extra-Adrenal; Succinate Dehydrogenase; Tomography, X-Ray Computed
PubMed: 36252779
DOI: 10.1016/j.eprac.2022.10.002 -
JAMA Surgery Oct 2022Adrenalectomy is the definitive treatment for multiple adrenal abnormalities. Advances in technology and genomics and an improved understanding of adrenal...
IMPORTANCE
Adrenalectomy is the definitive treatment for multiple adrenal abnormalities. Advances in technology and genomics and an improved understanding of adrenal pathophysiology have altered operative techniques and indications.
OBJECTIVE
To develop evidence-based recommendations to enhance the appropriate, safe, and effective approaches to adrenalectomy.
EVIDENCE REVIEW
A multidisciplinary panel identified and investigated 7 categories of relevant clinical concern to practicing surgeons. Questions were structured in the framework Population, Intervention/Exposure, Comparison, and Outcome, and a guided review of medical literature from PubMed and/or Embase from 1980 to 2021 was performed. Recommendations were developed using Grading of Recommendations, Assessment, Development and Evaluation methodology and were discussed until consensus, and patient advocacy representation was included.
FINDINGS
Patients with an adrenal incidentaloma 1 cm or larger should undergo biochemical testing and further imaging characterization. Adrenal protocol computed tomography (CT) should be used to stratify malignancy risk and concern for pheochromocytoma. Routine scheduled follow-up of a nonfunctional adrenal nodule with benign imaging characteristics and unenhanced CT with Hounsfield units less than 10 is not suggested. When unilateral disease is present, laparoscopic adrenalectomy is recommended for patients with primary aldosteronism or autonomous cortisol secretion. Patients with clinical and radiographic findings consistent with adrenocortical carcinoma should be treated at high-volume multidisciplinary centers to optimize outcomes, including, when possible, a complete R0 resection without tumor disruption, which may require en bloc radical resection. Selective or nonselective α blockade can be used to safely prepare patients for surgical resection of paraganglioma/pheochromocytoma. Empirical perioperative glucocorticoid replacement therapy is indicated for patients with overt Cushing syndrome, but for patients with mild autonomous cortisol secretion, postoperative day 1 morning cortisol or cosyntropin stimulation testing can be used to determine the need for glucocorticoid replacement therapy. When patient and tumor variables are appropriate, we recommend minimally invasive adrenalectomy over open adrenalectomy because of improved perioperative morbidity. Minimally invasive adrenalectomy can be achieved either via a retroperitoneal or transperitoneal approach depending on surgeon expertise, as well as tumor and patient characteristics.
CONCLUSIONS AND RELEVANCE
Twenty-six clinically relevant and evidence-based recommendations are provided to assist surgeons with perioperative adrenal care.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Cosyntropin; Glucocorticoids; Humans; Hydrocortisone; Pheochromocytoma; Surgeons
PubMed: 35976622
DOI: 10.1001/jamasurg.2022.3544 -
World Journal of Gastroenterology Aug 2016Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs... (Review)
Review
Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs (G-NETs) and duodenal NETs (D-NETs) are the common types of upper GI-NETs based on tumor location. G-NETs are classified into three distinct subgroups: type I, II, and III. Type I G-NETs, which are the most common subtype (70%-80% of all G-NETs), are associated with chronic atrophic gastritis, including autoimmune gastritis and Helicobacter pylori associated atrophic gastritis. Type II G-NETs (5%-6%) are associated with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome (MEN1-ZES). Both type I and II G-NETs are related to hypergastrinemia, are small in size, occur in multiple numbers, and are generally benign. In contrast, type III G-NETs (10%-15%) are not associated with hypergastrinemia, are large-sized single tumors, and are usually malignant. Therefore, surgical resection and chemotherapy are generally necessary for type III G-NETs, while endoscopic resection and follow-up, which are acceptable for the treatment of most type I and II G-NETs, are only acceptable for small and well differentiated type III G-NETs. D-NETs include gastrinomas (50%-60%), somatostatin-producing tumors (15%), nonfunctional serotonin-containing tumors (20%), poorly differentiated neuroendocrine carcinomas (< 3%), and gangliocytic paragangliomas (< 2%). Most D-NETs are located in the first or second part of the duodenum, with 20% occurring in the periampullary region. Therapy for D-NETs is based on tumor size, location, histological grade, stage, and tumor type. While endoscopic resection may be considered for small nonfunctional D-NETs (G1) located in the higher papilla region, surgical resection is necessary for most other D-NETs. However, there is no consensus regarding the ideal treatment of D-NETs.
Topics: Duodenal Neoplasms; Endoscopy, Gastrointestinal; Humans; Neuroendocrine Tumors; Stomach Neoplasms
PubMed: 27570419
DOI: 10.3748/wjg.v22.i30.6817 -
Ulusal Cerrahi Dergisi 2015Gallbladder paraganglioma is a very rare tumor, and only a few cases have been reported. Most of these cases were asymptomatic and found incidentally during operation....
Gallbladder paraganglioma is a very rare tumor, and only a few cases have been reported. Most of these cases were asymptomatic and found incidentally during operation. Our case involved a 57-year-old female patient complaining of intermittent right upper quadrant pain. Preoperative imaging demonstrated a mass in the neck of the gallbladder. Laparoscopic cholecystectomy was performed, and a frozen section of the gallbladder demonstrated a benign mass. The postoperative pathologic examination reported gallbladder paraganglioma and chronic cholecystitis. Immunohistochemically, the chief cells and sustentacular cells showed diffuse positivity with vimentin, synaptophysin, and S-100.
PubMed: 26668523
DOI: 10.5152/UCD.2014.2691 -
Translational Cancer Research Nov 2019Neuroendocrine tumors derived from the adrenal medulla and the adrenal gland are called pheochromocytoma (PHEO) and paraganglioma (PGL), respectively. PGLs are... (Review)
Review
Neuroendocrine tumors derived from the adrenal medulla and the adrenal gland are called pheochromocytoma (PHEO) and paraganglioma (PGL), respectively. PGLs are neuroepithelial cell clusters associated with sympathetic or parasympathetic nerves, and are scattered throughout the body. According to the World Health Organization, the presence of distant metastases indicates malignant PGL. Most PGLs are benign, but their high secretion of catecholamines in the blood is associated with high blood pressure, cardiovascular disease, and death. Further research on PGL is required to provide a theoretical basis for clinical diagnosis and treatment. This article reviews the genetics, clinical manifestations, benign and malignant forms, current major diagnostic methods, and progress in the treatment of PGL.
PubMed: 35117020
DOI: 10.21037/tcr.2019.10.11