Anaesthesia for removal of pheochromocytomas requires preoperative preparation of patients so as to normalize blood pressure. During operation, hypovolemia linked with the sudden fall of circulating catecholamines as well as hypertension during handling of the tumor are to be managed. A case of a 11 year child with pheochromocytoma is reported. Once the diagnosis had been established, various attempts to normalize blood pressure failed. Despite a precarious condition, the operation could be performed. During surgery, hypertension occurred and three hypotensive drugs failed to correct it. It ceased after removal of the tumor. The postoperative course was uneventful. The authors discuss the role of the type of catecholamines and their concentration in the failure of antihypertensive medication.
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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 PDFCancer Lett
January 2025
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, Maryland, USA. Electronic address:
Neoadjuvant immunotherapy represents a pioneering approach in the preoperative treatment of cancer, offering novel avenues for tumor reduction and improved patient outcomes by modulating the immune response. This study investigated neoadjuvant immunotherapy using intratumoral administration of mannan-BAM, Toll-like receptor ligands, and antiCD40 antibody (MBTA therapy) followed by surgery in murine models of mouse tumor tissue (MTT) pheochromocytoma, B16-F10 melanoma, and 4T1 and E0771.lmb mammary carcinomas.
View Article and Find Full Text PDFJpn J Radiol
January 2025
Department of Diagnostic Radiology, Institute of Science Tokyo, Bunkyo-ku, Tokyo, Japan.
Adrenal diseases pose significant diagnostic challenges due to the wide range of neoplastic and non-neoplastic pathologies. Radiologists have a crucial role in diagnosing and managing these conditions by, leveraging advanced imaging techniques. This review discusses the vital role of computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine in adrenal imaging, and focuses on morphological and functional evaluations.
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.
Eur J Endocrinol
January 2025
Unidade de Adrenal, Laboratório de Endocrinologia Molecular e Celular LIM25, Divisão de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo 01246-903, Brazil.
Objective: Germline and somatic drivers are identified in 30% and 40% of pheochromocytomas and paragangliomas (PPGLs), respectively. In this study, we investigated the genetic landscape of PPGLs in a Brazilian cohort.
Methods: We studied 182 index patients with PPGLs (116 females and 66 males), comprising 118 pheochromocytoma and 70 paraganglioma cases.
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