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Adrenalectomy
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Mikael Petrosyan, Timothy D. Kane
Given the frequency of extra-adrenal disease associated with some adrenal tumors (i.e. pheochromocytoma), the remainder of the retroperitoneum should be palpated when performing an adrenalectomy. This includes palpation of the para-aortic chain and the organ of Zuckerkandl at the aortic bifurcation. Additionally, the bladder should also be checked for masses.
The Adrenal Glands
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
This tumor commonly originates from the adrenal medulla and is unilateral 90% of the time. Extraadrenal tumors can be found in the organ of Zuckerkandl, but have been described almost anywhere in the sympathetic nervous system. Extraadrenal pheochromocytoma, also called paragangliomas, can be identified in the carotid body, aortic body, orbit, throughout the course of the vagus nerve, along the course of paraaortic sympathetic chain, and in the visceral autonomic paraganglia which includes the urinary bladder, duodenum, and the endocardium. The incidence of malignant pheochromocytoma is about 13%.5 It also can occur in conjunction with neuroectodermal dysplasia such as von Recklinghausen’s disease.
Pheochromocytoma — Clinical Manifestations, Diagnosis and Management
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
William M. Manger, R. W. Gifford
Pheochromocytomas arise from chromaffin cells in the adrenal medullae (90% of tumors), the organ of Zuckerkandl, and in chromaffin cells associated with sympathetic nerves and plexuses in extra-adrenal sites in the abdomen (including the urinary bladder), chest (< 2.0% of tumors), neck (< 0.1% of tumors); rarely tumors arise at the base of the skull, in the heart or spermatic cord. Multiple and extra-adrenal tumors occur more frequently in children (30% of cases) than adults (8% of cases). About 10% of pheochromocytoma cases are familial and at least 70% of these tumors are bilateral.
Factors contributing to development and resolution of dysglycemia in patients with pheochromocytomas and catecholamine-secreting paragangliomas
Published in Annals of Medicine, 2023
Lin Zhao, Ting Zhang, Xu Meng, Zenglei Zhang, Yi Zhou, Hua Fan, Yecheng Liu, Xianliang Zhou, Huadong Zhu
The enzyme phenylethanolamine-N-methyl transferase is responsible for conversion of norepinephrine to epinephrine in the adrenal glands. This enzyme is unique to the adrenal gland, brain, and organ of Zuckerkandl. Consequently, the adrenal medulla secretes the catecholamines predominantly as epinephrine [18]. As epinephrine exerts a heavier impact on glucose metabolism when compared to norepinephrine, dysglycemia may be more likely to occur in patients with PHEOs. Therefore, clinicians should screen for dysglycemia in patients with PHEOs. Besides, many of our patients who had dysglycemia did not have elevated BMI, this suggests that if dysglycemia is present in surprisingly lean patients, the presence of PPGLs should be vigilant.
Characteristics and outcome of patients with pheochromocytoma at a tertiary endocrinology clinic in Durban, South Africa over 14 years
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2018
Abdurraouf Esseid Zorgani, Fraser J Pirie, Ayesha A Motala
All the patients presented with intra-abdominal tumours (Table 2). Of these, 24 (68.6%) originated from the adrenal gland, 10 (28.6%) were located extra-adrenally and one (2.9%) was a combined adrenal and extra-adrenal tumour. Of the 25 adrenal tumours, the majority (88%; n = 22) were unilateral and right-sided (68.2%; n = 15) tumours; three (12%) were bilateral adrenal tumours. Of the 11 extra-adrenal tumours, three were located in the urinary bladder, seven in the lower abdomen and one in the Organ of Zuckerkandl.
Primary retroperitoneal paraganglioma mimicking a ureteral tumor: a case report and literature review
Published in Postgraduate Medicine, 2020
Paragangliomas (PGLs) and pheochromocytomas (PHEOs) are rare catecholamine-secreting neuroendocrine tumors often presenting with secondary hypertension [1,2]. PHEOs arise from chromaffin cells of the adrenal medulla with an incidence of 80–85%, whereas PGLs arise from chromaffin cells of extra-adrenal sites along the sympathetic and/or parasympathetic chain with an incidence of 15–20% [1,3]. PGLs can be found at any site in the entire body, from the skull to the pelvic floor, and majority of them occur in the abdomen. The most common location of PGLs in the abdomen is the organ of Zuckerkandl, which is located in the retroperitoneum aortic bifurcation [4]. Retroperitoneal PGLs can be divided into functional and nonfunctional PGLs. Functional tumors are known to originate, synthesize, store, and secrete catecholamines, causing elevated levels catecholamines in the urine/serum and classical clinical symptoms such as episodic headaches, sweating, and palpitations [5,6]. Nonfunctional retroperitoneal forms are often characterized by their asymptomatic profile and normal levels of catecholamines in the urine/serum, which pose a significant challenge in the diagnosis [6,7]. The abdominal computed tomography (CT) scan does not present any unique imaging characteristics for retroperitoneal PGLs. Consequently, these tumors may be misdiagnosed as other more common retroperitoneal tumors such as lymphomas, schwannoma, and neurofibromas [8,9]. When retroperitoneal PGLs produce symptoms of compression or invasion of adjacent retroperitoneal organs, they could be mistaken for the tumor of the adjacent organs, such as pancreatic, renal, or adrenal neoplasms [10–13]. If the tumor grows near the ureter, ureteral obstruction can occur in rare cases. To our knowledge, only three such cases have been reported to date [14,15]. We reported the case of ureteral obstruction caused by extra-adrenal retroperitoneal PGL, which was similar to a ureteral tumor.