Management

Management is directed by a specialist.

Local bowel lesions are resected along with local mesenteric nodes. A partial hepatectomy may be considered when liver metastases are present. Alternatively, the metastases are encapsulated and enucleation may be possible.

Very rarely, liver transplantation has been carried out

• this treatment measure has occasionally been performed in patients in whom extrahepatic tumour spread and primary tumour recurrence has been excluded

Medical treatments:

• somatostatin analogue for somatostatin receptor scintigraphy (SRS) positive carcinoid tumours (1,2)
• somatostatin analogs (Octreotide, Sandostatin, Lanreotide) may reduce the symptoms of carcinoid syndrome, including skin flushing and diarrhoea (3)
• alpha interferon for SRS negative carcinoid tumours
  • option for the control of refractory symptoms of the carcinoid syndrome in patients treated with somatostatin analogs is interferon alfa (3)
• other treatment measures include histamine antagonists (H1 and H2), cyproheptadine, nicotinamide
• treatment with radiolabelled, chelated somatostatin analogues may be considered in some cases (at present an experimental treatment modality)

Metastatic disease is not curable by chemotherapy or radiotherapy.

• hepatic embolization of metastases
  • most effective treatment for liver metastases from functioning neuroendocrine tumours producing symptoms
  • more controversially: may be used to reduce tumour load in these patients in order to improve the well being of the patient or to reduce local symptoms (e.g. "dragging" abdominal pain from hepatomegaly)
  • therapy is directed at interrupting the arterial supply to highly vascularized neuroendocrine hepatic metastases. As the normal liver has a dual blood supply with one-third deriving from the portal blood, normal liver tissue is not compromised by ligation of the hepatic arteries
  • ischaemia can be achieved by hepatic artery ligation, alone or in combination with subsequent systemic chemotherapy, or by selective embolization alone or combined with intra-arterial chemotherapy (chemoembolization)
  • may lead to significant reduction in symptoms (40-90%)

Reference:

1. Endocr Relat Cancer. 2004 Mar;11(1):1-18.
2. de Herder WW and Lamberts SWJ. Best Practice & Research Clinical Endocrinology & Metabolism 2004; Volume 18(4): 477-495.
3. Gade AK, Olariu E, Douthit NT. Carcinoid Syndrome: A Review. Cureus. 2020 Mar 5;12(3):e7186. doi: 10.7759/cureus.7186.