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cells of the pancreas, whereas NETs of other sites such as the lungs or gastrointestinal tract are often referred to as carcinoid tumors ( Kulke et al . 2012 ). NETs can be further classified according to histological subtype. A recent classification
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Quality of life is multi-dimensional, including issues relating to symptoms from the disease but also social, emotional, functional and financial domains. Debate remains on the true definition of quality of life and its measurement. Quality of life measurements are best done by patients themselves, although, in some situations a proxy such as carer or relative can be substituted. Healthcare workers can over- or underestimate overall quality of life. Currently used devices for measuring quality of life in cancer include the European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30, which is a generic tool for all cancers and which requires the use of add-on modules for specific cancers. We are developing a separate module for carcinoid/neuroendocrine tumours, in accordance with the EORTC guidelines on module development, which will be translated into five languages and will be available for use throughout Europe.
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The rationale underlying an aggressive approach in the management of some carcinoid patients is explained and illustrated by the presented case of a middle-aged man with advanced classic typical midgut carcinoid. The patient exhibited somatostatin receptor scintigraphy-positive massive liver metastases, carcinoid syndrome, severe tricuspid and pulmonic cardiac valve disease with congestive heart failure, ascites and malnutrition. He had been treated for several years with supportive medications and biotherapy including octreotide and alpha interferon but his tumor eventually progressed and his overall condition was markedly deteriorated when he first sought more aggressive treatment. This consisted of prompt replacement of both tricuspid and pulmonic valves, followed by hepatic artery chemoembolus (HACE) injection and then surgical tumor debulking including excision of the primary tumor in the small intestine. In addition, radiofrequency ablation was utilized to reduce the volume of metastases in the liver. Prophylactic cholecystectomy was also performed and a biopsy of tumor was submitted for cell culture drug resistance testing. This was followed by systemic chemotherapy utilizing the drug (docetaxel) which the in vitro studies suggested as most likely to be effective. His excellent response to this succession of treatments exemplifies the successful application of aggressive sequential multi-modality therapy.
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spread to regional lymph nodes and liver at the time of diagnosis. The tumors can produce hormones, e.g. serotonin and tachykinins, which can give rise to the carcinoid syndrome causing flushing, bronchoconstriction, and heart disease ( de Herder 2007
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Introduction Neuroendocrine neoplasms (NENs), previously named ‘carcinoid’ tumours, most frequently occur in the gastrointestinal (GI) tract (54.5%) and have been an area of ongoing interest in the field of many different disciplines including
Department of Cardiology, Department of Obesity and Endocrinology, Clinical Sciences Centre, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK
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Introduction Neuroendocrine tumours (NETs) are a varied group of tumours that arise from neuroendocrine precursor cells. They are rare occurring in 1.2–2.1/100 000 of the general population ( Modlin & Sandor 1997 ). Carcinoid syndrome, which is
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Lundberg Laboratory for Cancer Research, Department of Zoology, Department of Neuroscience and Physiology, Lundberg Laboratory for Cancer Research, Department of Pathology and Cytology, Sahlgrenska Academy, University of Göteborg, Gula Stråket 8, SE-413 45 Göteborg, Sweden
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Introduction Metastases, i.e. growth of tumour cells in lymph nodes and distant organs, represent the most serious consequence of malignancy, and account for more than 90% of tumour-related deaths. Most patients with ileal carcinoids have metastatic
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Bronchial carcinoids Bronchial carcinoid (BC), the second most common carcinoid tumor after gastrointestinal carcinoids, accounts for 20–30 percent of all cases of well-differentiated neuroendocrine tumors. With an incidence rate of 1.35 per 1 00
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Maimonides Institute for Biomedical Research of Cordoba (IMIBIC), Córdoba, Spain
Endocrinology and Nutrition Service, Reina Sofia University Hospital, Córdoba, Spain
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NETs is the carcinoid syndrome (CS). First described in 1931 ( Scholte 1931 ), this syndrome is characterized by the occurrence of increased bowel movements, vasoactive flushes and bronchospasms as well as long-term fibrotic changes in the mesentery and
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Many clinicians prefer to avoid surgery in patients with carcinoid neoplasia, because of its slow growth and relatively favourable prognosis. Nevertheless, the commonest cause of death in patients with carcinoid is advanced metastatic disease, and both clinical and epidemiological data indicate that the more effectively the disease is ablated, the more long-lasting the benefit. Multidisciplinary management of patients with carcinoid must consider inherited risk, possible multiple carcinoids and/or synchronous non-carcinoid cancer, and the use of a range of investigations that also evaluate the 10% of patients with carcinoid syndrome with or without valvular heart disease. Although primary size is correlated with the presence of nodal with or without liver metastases, carcinoid tumours <1 cm in diameter may be metastatic at presentation, particularly those arising within the small intestine. In the jejunum and ileum, resection of all sizes of carcinoid with local and regional nodes is preferred, to prevent nodal dissemination causing mesenteric ischaemia with or without infarction. Resection of nodal metastases should be undertaken in those with persistent or recurrent nodal disease if possible. Appendiceal and right colonic carcinoids are most effectively treated by right hemicolectomy with local and regional nodal clearance, as for adenocarcinoma. However, for most appendiceal carcinoids which are <1 cm in diameter and non-invasive, appendicectomy alone is sufficient. For appendiceal carcinoids 1-2 cm in diameter, histopathological assessment helps to determine the need for hemicolectomy. Liver resection has been followed by prolonged 5 year survival in several series and is recommended in appropriate patients to attempt cure or to debulk metastatic disease. Liver transplantation has had only qualified success in highly selected patients without extra-hepatic disease in whom other therapies have failed.
