Duodenal gangliocytic paragangliomas are rare neuroendocrine tumors primarily localized in the periampullary area. Though mostly asymptomatic, they can present with various symptoms, most often jaundice, anemia and abdominal pain. The present paper is a case series report, describing our personal experience with patients presenting to the Emergency Unit with different symptoms due to duodenal gangliocytic paraganglioma. Endoscopic resection is safe and indicated in most of the cases, being also associated with lower medical costs. EUS plays a central role in the pre-resection management and in surveillance, and immunostaining is decisive to ascertain the tumor histologic origin. In addition to reporting our experience, we researched the literature regarding these rare tumors and performed a comprehensive review.
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http://dx.doi.org/10.3390/life13030597 | DOI Listing |
Clin Nucl Med
December 2024
Department of Nuclear Medicine, Chongqing University Cancer Hospital, Chongqing, China.
We present the imaging findings of a 48-year-old woman that metastasized to multiple bones with a history of duodenal gangliocytic paraganglioma. 68Ga-DOTATATE PET/CT showed multiple osteolytic bone destruction with intense uptake. Multiple bone metastases originating from duodenal gangliocytic paraganglioma confirmed histopathological results of a biopsy on the chest-back bone.
View Article and Find Full Text PDFDiseases
October 2024
Department of Surgery, San Giovanni Addolorata Hospital, 00184 Rome, Italy.
Background: Duodenal neuroendocrine tumors (NETs) are uncommon, accounting for less than 4% of all gastrointestinal neoplasms. Prognosis is related to tumoral staging and grading, as well as to the specific subtype. In this article, we retrospectively describe the clinical presentation and surgical treatment of two rare large duodenal NETs: a high-grade G3 NET and a Gangliocytic Paraganglioma (GP).
View Article and Find Full Text PDFExp Ther Med
December 2024
Department of Gastroenterology, The Second Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China.
Gangliocytic paraganglioma (GP) is a rare neuroendocrine tumor primarily found in the duodenum, most commonly in the second and third sections of the duodenum. Diagnosis of GP is based on its distinctive histopathological characteristics, which include three types of tumor cells in varying proportions: i) Epithelioid, ii) spindle-like and iii) ganglion-shaped cells. The distribution of the three tumor cell components varies from case to case and a patient may be easily misdiagnosed if one of the components is predominant.
View Article and Find Full Text PDFEndocrinol Diabetes Nutr (Engl Ed)
August 2024
Servicio de Endocrinología y Nutrición, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.
Paragangliomas (PGLs) are rare and encapsulated neuroendocrine tumors (NET), located in the adrenal gland or the extra-adrenal paraganglia. Extra-adrenal PGLs may develop a gangliocytic component with ganglion cells which are called gangliocytic paragangliomas (GPs). The most common location is the duodenum, and they appear with digestive symptoms or as an incidental finding.
View Article and Find Full Text PDFMedicine (Baltimore)
December 2023
Department of Gastroenterology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian, P.R. China.
Rationale: Gangliocytic paraganglioma is a rare tumor that can occur in several organs throughout the body. Gangliocytic paraganglioma of the main duodenal papilla is even rarer. This study analyzes and discusses the endoscopic management of a case of gangliocytic paraganglioma of the main duodenal papilla and reviews the relevant literature.
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