Year: 2024
Type: article
Study design: Case Report
Abstract: M.D. Chandra: None. Introduction: Neuroendocrine tumors (NETs) are rare tumors usually originating in the GI tract and lung. An ovarian neuroendocrine tumor is a rare entity, accounting for 0.5% of all carcinoid tumors and 0.1% of all ovarian. Case Presentation: A 75-year-old female presented with five days of abdominal pain. The evaluation showed normal labs and an essentially normal CT scan of the abdomen and pelvis, except for an incidental finding of a 7.8 cm right ovarian solid heterogeneously enhancing mass. MRI revealed that this mass had solid and cystic components. Later, the patient revealed an 18-pound unintentional weight loss in the past six months, with recurrent episodes of nausea, loose stools, and flushing. Evaluation for carcinoid syndrome showed an elevated 24-hour urinary 5-hydroxyindoleacetic acid [5-HIAA] at 160.8 mg, elevated serum serotonin, and chromogranin levels along with a gallium-68 dotatate scan revealing a right adnexal mass consistent with neuroendocrine malignancy. Further imaging was negative for metastasis, and upper and lower GI endoscopies were normal. She underwent tumor resection and bilateral salpingo-oophorectomy. During her exploratory laparotomy, one lesion was noted in the proximal small bowel. The ovarian tumor measured 9.1 x 9.3 X 7.4 cm, with pathology confirming its origin from the GI tract. Histology was notable for a well-differentiated neuroendocrine tumor without perineural invasion, positive for chromogranin A, immunoreactive for synaptophysin, and chromogranin. Hence, this was a metastatic small bowel neuroendocrine tumor with an isolated focus of a well-differentiated tumor in the right ovary. Four months after surgery, the patient's labs, including 24-hour 5-HIAA, were normal. The follow-up gallium scan did not show any uptake and her symptoms had resolved. Discussion: Gastrointestinal NETs, formerly called carcinoid tumors, arise from endocrine cells of the digestive tract. Well-differentiated NETs exhibit sluggish growth, and most NETs do not produce functioning hormones. Still, approximately 25% make hormones, such as serotonin, where hormone-related symptoms [diarrhea, facial flushing] are often both the source of presentation and morbidity. They can be encountered throughout the GI tract and may present symptomatically or be found incidentally. The best initial test is the 24-hour urinary excretion of 5-HIAA. Urinary and plasma serotonin and chromogranin concentrations can also be measured. The tumor must be localized and staged with the help of imaging techniques like CT or MRI. Upper and lower endoscopy must be performed for evaluation of metastatic tumors with an unknown primary site. Whole-body imaging with somatostatin receptor imaging like Octreoscan or gallium 68 dotatate is preferred, the latter being a better choice. Site-specific surgical removal helps in controlling the carcinoid syndrome symptoms. Monday, June 3, 2024
Source: Journal of the Endocrine Society
Author: Medhavani D Chandra
Institution: Marshfield Clinic
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,418
OpenAlex ID: W4409965507