Abstract
Background. A 48-year-old man presented with diarrhea, flushing, abdominal pain and weight loss of 10 kg over a 6-month period. He subsequently developed dyspnea on exertion.
Investigations. Physical examination, laboratory tests, CT of the abdomen, liver biopsy, echocardiography, immunohistochemistry staining of the biopsy specimen for neuroendocrine markers including chromogranin A, synaptophysin and protein gene product 9.5, and 111In-pentetreotide scintigraphy (Octreoscan™).
Diagnosis. Carcinoid tumor of midgut origin with large segment 3 liver metastasis. Carcinoid syndrome and carcinoid heart disease.
Management. Symptomatic relief with somatostatin analog therapy and subsequent resection of the segment 3 liver metastasis. Tricuspid and pulmonary valve replacement.
References
Modlin, I. M. et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 9, 61–72 (2008).
Caplin, M. E. et al. Carcinoid tumor. Lancet 352, 799–805 (1998).
Ramage, J. et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours. Gut 54 (Suppl. 4), iv1–16 (2005).
Bhattacharyya, S. Davar, J., Dreyfus, G. & Caplin, M. E. Carcinoid heart disease. Circulation 116, 2860–2865 (2007).
Kaltsas, G. A., Besser, G. M. & Grossman, A. B. The diagnosis and medical management of advanced neuroendocrine tumours. Endocr. Rev. 25, 458–511 (2004).
Sarmiento, J. M. et al. Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J. Am. Coll. Surg. 197, 29–37 (2003).
Steinmüller, T. et al. Consensus guidelines for the management of patients with liver metastases from digestive (neuro)endocrine tumors: foregut, midgut, hindgut, and unknown primary. Neuroendocrinology 87, 47–62 (2008).
Que, F. G., Nagorney, D. M., Batts, K. P., Linz, L. J. & Kvols, L. K. Hepatic resection for metastatic neuroendocrine carcinomas. Am. J. Surg. 169, 36–42 (1995).
Eriksson, B. et al. Consensus guidelines for the management of patients with digestive neuroendocrine tumors—well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology 87, 8–19 (2008).
Connolly, H. M. et al. Outcome of cardiac surgery for carcinoid heart disease. J. Am. Coll. Cardiol. 25, 410–416 (1995).
Møller, J. E. et al. Prognosis of carcinoid heart disease: an analysis of 200 cases over two decades. Circulation 112, 3320–3327 (2005).
Kousnetzoff, M. et al. Sur la resection partielle du foie chez l'homme et chez les animaux [French]. Rev. Chir. 17, 319–331 (1897).
Parks, R. W., Chrysos, E. & Diamond, T. Management of liver trauma. Br. J. Surg. 86, 1121–1135 (1999).
Mays, E. T. The hazards of suturing certain wounds of the liver. Surg. Gyn. Obstet. 143, 201–204 (1976).
Gurusamy, K. S., Kumar, Y., Sharma, D. & Davidson, B. R. Methods of vascular occlusion for elective liver resection. Cochrane Database Syst. Rev. 4, CD006409 (2007).
Acknowledgements
Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing financial interests.
Rights and permissions
About this article
Cite this article
Bhattacharyya, S., Gujral, D., Toumpanakis, C. et al. A stepwise approach to the management of metastatic midgut carcinoid tumor. Nat Rev Clin Oncol 6, 429–433 (2009). https://doi.org/10.1038/nrclinonc.2009.70
Issue Date:
DOI: https://doi.org/10.1038/nrclinonc.2009.70
- Springer Nature Limited