Dataset Information

Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature.

ABSTRACT: Introduction:Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease. Presentation of Case:A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma. Discussion:The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery. Conclusion:Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.

SUBMITTER: Adams L

PROVIDER: S-EPMC6178180 | biostudies-other | 2018

REPOSITORIES: biostudies-other

ACCESS DATA

Publications

Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature.

Adams Laurie L Friedman Theodore M TM Shaver Timothy R TR Younan George G

Case reports in surgery 20180926

<h4>Introduction</h4>Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease.<h4>Presentation of case</h4>A 49-year-old Caucasian female, who presented with an upper gastr ...[more]

PMID: 30356398

Similar Datasets

Project description:Context: Metastatic pheochromocytoma/paraganglioma (MPP) therapy mainly involves radionuclide therapy, chemotherapy, and targeted therapy. In recent years, temozolomide (TMZ) showed great promise in some MMP patients, especially those with SDHB germline mutation. We reported a patient with MPP who did not have any known germline genetic change and responded remarkably well to TMZ monotherapy. Case presentation: The patient was a 41-year-old woman with local and distant recurrence (soft tissues and bone metastases) of retroperitoneal paraganglioma. She suffered from dizziness, palpitation, sweating, weight loss and constipation, with the blood pressure fluctuating substantially from 130/100 mmHg to 190/120 mmHg, although she was on phenoxybenzamine and metoprolol medication. The patient showed clinical and radiological response after 3-cycle TMZ therapy. Upon 15 cycles of TMZ therapy, her symptoms were dramatically alleviated, urinary norepinephrine excretion decreased from 1,840 μg/24 h to 206 μg/24 h, and CT showed that the lesions further shrank. Molecular profiling of the tumor tissue of the patient revealed hypermethylation of the O6-methylguanine-DNA-methyltransferase (MGMT) promoter and a negative immunostaining for MGMT. Globally, only 26 cases of MPP treated with TMZ have been described so far. TMZ is effective, especially in patients with SDHB mutation, which can be explained by the silencing of MGMT expression as a consequence of MGMT promoter hypermethylation in SDHB-mutated tumors. Although, in general, patients with SDHB mutation or MGMT promoter hypermethylation have better response to TMZ, there are also exceptions. Severe side effects are uncommon, with only 17.4% patients experiencing Grade 3 toxicities, including lymphopenia, and hypertension. Conclusions: TMZ is effective and safe in MPP patients, and, it may work better on patients with SDHB-related MPP. Measurement of MGMT expression might help assess the tumor sensitivity to TMZ but this needs further systematic investigation.

Dataset Information

Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature.

Publications

Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets