We present a case of non-functioning malignant paraganglioma of the abdomen with cervical lymph node metastases. At laparotomy the tumor was considered unresectable. The patient received palliative radiation therapy and died 3 months later from disseminated disease. We discuss the diagnostic approach and the therapeutic options.
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Cureus
December 2024
Pediatric Radiology, Detroit Medical Center, Wayne State University, Detroit, USA.
Pheochromocytoma is a catecholamine-secreting tumor that arises from the medullary chromaffin cells but can rarely be extra-adrenal in origin. We present a case of a 16-year-old female patient with uncontrolled hypertension, despite being on lisinopril and metoprolol, and associated left-sided chest pain, recurrent headaches, and an unintentional weight loss of 10 pounds in one month. Laboratory work-up showed a markedly elevated plasma metanephrine level of 4463.
View Article and Find Full Text PDFLaryngoscope Investig Otolaryngol
February 2025
Division of Otolaryngology - Head and Neck Surgery, Department of Surgery Dalhousie University Halifax Nova Scotia Canada.
Objective: Carotid body tumors (CBTs) are rare neoplasms of the paraganglia at the carotid bifurcation. While typically benign, CBTs occasionally exhibit malignancy, metastasizing to nearby lymph nodes. Histopathologic analysis alone is insufficient to confirm malignancy, requiring metastases to non-neuroendocrine tissue for a definitive diagnosis.
View Article and Find Full Text PDFMethods Mol Biol
January 2025
Analytic Biochemistry, Calculi and Manual Chemistry, Mass Spectrometry, ARUP Laboratories, Inc., Salt Lake City, UT, USA.
Metanephrines (metanephrine [MN] and normetanephrine [NMN]) are O-methylated metabolites derived from the catecholamines, epinephrine, and norepinephrine, respectively. High concentrations of metanephrines have been observed in individuals with pheochromocytoma, a neuroendocrine tumor. Measurement of metanephrines in urine is used to screen for the tumor.
View Article and Find Full Text PDFEur J Endocrinol
January 2025
Department of Internal Medicine IV, LMU University Hospital, LMU Munich, 80336 Munich, Germany.
Objective: The effects of sex hormones remain largely unexplored in pheochromocytomas and paragangliomas (PPGLs) and gastroenteropancreatic neuroendocrine tumors (GEP-NETs).
Methods: We evaluated the effects of estradiol, progesterone, Dehydroepiandrosterone sulfate (DHEAS), and testosterone on human patient-derived PPGL/GEP-NET primary culture cell viability (n = 38/n = 12), performed next-generation sequencing and immunohistochemical hormone receptor analysis in patient-derived PPGL tumor tissues (n = 36).
Results: In PPGLs, estradiol and progesterone (1 µm) demonstrated overall significant antitumor effects with the strongest efficacy in PPGLs with NF1 (cluster 2) pathogenic variants.
Jpn 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.
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