Tricuspid valve replacement and liver transplantation in a 51-year-old female patient with grade 1 ileum NET and refractory carcinoid syndrome

#4235

Introduction: Refractory carcinoid syndrome (RCSy) could lead to severe complications.

Aim(s): Present the role of liver transplantation in the management of RCSy.

Materials and methods: A 51-year-old woman was referred with flushing and diarrhoea in 2018. Routine lab tests were unremarkable. Abdominal computed tomographic scan showed multiple liver lesions and a solid 5cm mesenteric mass. Biopsy from a liver lesion confirmed that it was a metastasis of grade 1, well-differentiated neuroendocrine tumor (NET). All visible tumors exhibited moderate tracer uptake on somatostatin receptor scintigraphy. As the primary tumor was identified in the small intestine, the patient underwent ileal resection and removal of metastatic mesenteric lymph nodes. The histology report confirmed the presence of multiple carcinoid tumors in the ileum, classified as NET G1. Despite telotristat and an increased dose of SSA, the patient developed severe carcinoid heart disease two years later, resulting in significant tricuspid regurgitation that necessitated tricuspid valve replacement. In 2021, an 18F-DOPA PET/CT scan confirmed that all tumors were localized in the liver. However, interventional local therapy or surgical resection was not possible due to the high tumor burden and PRRT was not recommended because of low SSR expression. In 2022, liver transplantation was suggested and subsequently performed. The intraoperatively found 1 cm mass in the pancreas head and a small portal lymph node were histologically diagnosed as metastases.

Conference:

Presenting Author: Tőke J

Authors: Tőke J, Kövesdi A, Szolnoky J, Huszty G, Piros L,

Keywords: refractory carcinoid syndrome, liver transplantation, ileum neuroendocrine tumor,