Liver transplantation in a patient with small-intestine neuroendocrine tumor (NET) and carcinoid syndrome

Soares Daniela M.; Fernandes Andreia Martins; Silva Leandro Augusto; Sofia Lopes; Elvas Ana Rita; Jacinta Santos; Teresa Martins; Sara Povoa; Nuno Bonito; Joao Correia; Vasco Ribeiro; Martins Raquel G.; Joana Couto; Fernando Rodrigues

doi:10.1530/endoabs.110.P520

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Endocrine Abstracts (2025) 110 P520 | DOI: 10.1530/endoabs.110.P520

ECEESPE2025 Poster Presentations Endocrine Related Cancer (76 abstracts)

Liver transplantation in a patient with small-intestine neuroendocrine tumor (NET) and carcinoid syndrome

Daniela M. Soares ¹ , Andreia Martins Fernandes ² , Leandro Augusto Silva ² , Sofia Lopes ³ , Ana Rita Elvas ² , Jacinta Santos ² , Teresa Martins ² , Sara Póvoa ² , Nuno Bonito ² , João Correia ² , Vasco Ribeiro ⁴ , Raquel G. Martins ² , Joana Couto ² & Fernando Rodrigues ²

Author affiliations

¹Unidade Local de Saúde de Santo António, Porto, Portugal; ²Instituto Português de Oncologia Francisco Gentil de Coimbra, Coimbra, Portugal; ³Unidade Local de Saúde de Coimbra, Coimbra, Portugal; ⁴Curry Cabral Hospital, Unidade Local de Saúde de São José, Lisboa, Portugal

JOINT943

Background/Aims: Liver metastasis (LM) constitute a major prognostic factor in patients with neuroendocrine tumors (NET). Symptoms of carcinoid syndrome (CS) occur when serotonin and other substances (tachykinins, prostaglandin and histamine) are secreted directly into the systemic circulation, generally associated to small-intestine NET with LM. In case of stage IV disease, resection of the primary tumor can be considered, followed by liver-directed therapies or, in very exceptionally selected cases, liver transplantation (LT).

Results: 31-year-old male, ECOG PS 0, with irrelevant medical history, referred for right-upper quadrant abdominal pain, diarrhea and flushing; initial abdominal imaging revealed multiple hepatic lesions. Laboratory evaluation documented elevated chromogranin A (CgA) (176. 0 ng/mL; reference range <102. 0) and slightly raised urinary 5-hydroxyindoleacetic acid (u5-HIAA) (37. 8 mg/24h; reference range <15). Patient underwent ileoncolonoscopy, with inconclusive findings due to inadequate preparation, and a [⁶⁸Ga]Ga-DOTANOC PET-CT, compatible with probable primary NET of the terminal ileum, with mesentery lymph nodes metastasis and extensive LM. After multidisciplinary evaluation, the patient initiated monthly lanreotide therapy and liver biopsy was performed soon after, revealing hepatic involvement by NET G2 (Ki67 5%). Cardiology assessment excluded carcinoid heart disease. He was then submitted to segmental ileal enterectomy and cholecystectomy; pathological examination confirmed ileal NET G1 with lymph node metastasis (Ki67<1%; pT2N1). Subsequent imaging and evaluations demonstrated stable exclusive liver disease and normalization of CgA and u5-HIAA levels. Nevertheless, exuberant CS symptoms persisted subsequently, despite optimized lanreotide therapy (120 mg 3/3 weeks). Concerning patient age, good performance-status and stable exclusive but symptomatic liver disease, LT was considered over additional local or systemic therapies on a multidisciplinary setting. After further experts’ opinion, the patient was considered a suitable candidate. The procedure was recently carried out at an experienced reference center after patient’s consent, approximately three years after the initial diagnosis. The patient is currently CS-related symptoms’ free and [⁶⁸Ga]Ga-DOTANOC PET-CT re-evaluation is being planned.

Discussion: In this case, the patient remained symptomatic despite normal u5-HIAA levels; symptoms’ resolution shortly after LT highlights the role of unmeasured substances in CS. The case also reflects the complexity of managing patients with metastatic NET and the importance of a multidisciplinary approach, moreover in the presence of hormonal secretion. Quality-evidence on LT in this setting is still scarce; therefore, patients should be judiciously selected.