Objective: Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and to validate the role of laparoscopic surgery in the treatment of these tumors.
Design: This study is a retrospective review. A total of 68 procedures for pheochromocytoma were performed between June 1997 and February 2017. All patients were investigated and operated on using an established departmental protocol. Relevant data were retrieved from the hospital records of 533 patients who underwent 541 adrenalectomies for benign and malignant adrenal tumors in the same period.
Results: Sixty-nine tumors were removed from 67 patients. One patient with/MEN2A underwent bilateral resection of pheochromocytomas in two stages. Tumor size in laparoscopic procedures ranged from 1.2 cm to 11.0 cm (mean 5.87 cm). Thirty-seven patients had benign disease, 31 potentially malignant (based on PASS) and 1 malignant with metastasis. Eight were in the context of a familial syndrome. Forty-nine patients underwent laparoscopic adrenalectomy, 8 patients had open approach from the start for recurrent pheochromocytoma or large benign tumor, 1 patient had open approach due to inoperable malignant pheochromocytoma and 10 patients had conversions from laparoscopic to open procedure. Nine patients received sodium nitroprusside intraoperatively to treat hypertension. One patient developed pulmonary embolism and succumbed 1 month later. There were no recurrences of the benign or potentially malignant tumors during the follow-up period.
Conclusions: Laparoscopic resection of pheochromocytomas, despite its increased level of difficulty compared to that of other adrenal tumors, is a safe and effective procedure.
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http://dx.doi.org/10.14310/horm.2002.1759 | DOI Listing |
JNCI Cancer Spectr
January 2025
Section on Medical Neuroendocrinology National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, 20892, MD, USA.
Head and neck paragangliomas (HNPGLs) are typically slow-growing, hormonally inactive tumors of parasympathetic paraganglia. Inactivation of prolyl-hydroxylase domain-containing 2 protein causing indirect gain-of-function of hypoxia-inducible factor-2α (HIF-2α), encoded by EPAS1, was recently shown to cause carotid body hyperplasia. We previously described a syndrome with multiple sympathetic paragangliomas caused by direct gain-of-function variants in EPAS1 (Pacak-Zhuang syndrome, PZS) and developed a corresponding mouse model.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Division of Thoracic Surgery, Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
A pheochromocytoma is a malignant tumor with metastatic potential. Moreover, the cardiovascular effects of abnormal amounts of catecholamines resulting from pheochromocytoma impact prognosis. Resection of the primary tumor is useful for reducing catecholamine production; however, the significance of resection of metastases remains unclear.
View Article and Find Full Text PDFAndes Pediatr
October 2024
Escuela de Medicina, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Unlabelled: Adrenal tumors in children are frequently neoplastic and malignant, and surgical resection is the first management option. Minimally invasive surgery (MIS) has proven to be a safe management alternative and is suggested as a preferred alternative approach.
Objective: To report the surgical outcomes of patients with adrenal tumors treated by MIS.
Curr Oncol Rep
January 2025
Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece.
Purpose Of Review: The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.
Recent Findings: Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option.
J Clin Endocrinol Metab
January 2025
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH), CH-8091 Zurich, Switzerland.
Context: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.
Objective: To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.
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