Carcinoid syndrome E34.0

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 13.10.2022

Synonym(s)

Apudom; Argentaffinoma; argentaffinoma syndrome; carcinoid cardiac syndromes; Carcinoid syndrome; malignant carcinoid syndrome

History
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Lubarsch, 1888; Oberndorfer, 1907

Definition
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Metastasized tumor, usually localized in the lower ileum, endocrine active, originating from the cells of the APUD system, characterized by a seizure-like flush symptomatology.

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Occurrence/Epidemiology
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Most common endocrine disease of the gastrointestinal tract. Incidence: 0.3-0.5/100.000 diseases/year.

Etiopathogenesis
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Production of serotonin and other biologically active substances (histamine, VIP, prostaglandins, glucagon, gastrin, calcitonin) by the enterochromaffin cells proliferating in the carcinoid.

Manifestation
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Women and men are equally affected, mainly in the 2nd-7th decade.

Clinical features
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Integument: Flushing symptoms associated with itching attacks, provocable by vasoactive procedures and substances (excitement, warm soups, spicy foods, alcohol, salicylic acid, paracetamol, methocarbamol, mephenesin). With recurrent symptoms, planar cyanosis of the face and neck, telangiectasias, periorbital swelling. Pellagroid manifestations with xerosis, hyperkeratosis, and scaling on the neck and forearms occur when endocrine active substances secreted by carcinoid cells, such as serotonin, histamine, kinins, prostaglandins, become systemically active due to abolished hepatic inactivation, usually after liver metastasis.

Extracutaneous manifestations:

Abdomen:Periodic abdominal pain, diarrhea, nausea, vomiting.
Heart: Thickening and shrinkage of the pulmonary and tricuspid valves and of the endocardium = endocarditis fibroplastica; signs of an acquired (mainly right-sided) valvular defect (tricuspid regurgitation and pulmonary stenosis), tachycardia, hypotensive crises, and asthmoid complaints. On the ECG: low voltage, atrial overload on the right, right ventricular hypertrophy and right bundle branch block. During carcinoid flush, coronary spasm with Prinzmetal angina may occur
Pulmonary signs: bronchial asthma. Metabolic signs: including bouts of ravenous hunger with spontaneous hypoglycemia.

Laboratory
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Detection of increased hormone production. Detection of increased excretion of 5-hydroxy-indoleacetic acid in urine in about 30% of patients (more than 20 mg/24 hrs). For insulinoma in particular: 72-hour hunger test with determination of blood sugar, plasma insulin level and C-peptide. With mainly gastrinoma: gastrin test.

Histology
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Tumour from argentophilic cells (argentaffinoma).

Internal therapy
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In case of inoperability or metastases conservative therapy: therapy of first choice: somatostatin derivative octreotide (e.g. Sandostatin). Initial 1-2 times/day 50 µg octreotide s.c. Then dose escalation to 3 times/day 100-200 µg s.c.
Therapy of the 2nd choice: Interferon alfa-2a (e.g. Roferon) 3 times/week 3-6 million IU s.c. or polychemotherapy (Streptozocin, 5-fluorouracil, cyclophosphamide, doxorubicin).
Treatment of the accompanying symptoms:

Flush: Kallikrein secretion: receptor blockers such as phenoxybenzamine (e.g. dibenzyran) 10-60 mg/day p.o.
Histamine secretion: clemastine(e.g. Tavegil) 1-2 mg/day p.o. or trial with H1-blockers (e.g. diphenhydramine) in combination with H2-blockers (e.g. ranitidine or cimetidine) 3 times/day mg p.o.
Serotonin secretion: Cyproheptadine (e.g. Peritol) 6-30 mg/day p.o.
Diarrhoea: Loperamide (e.g. Imodium) 2 times/day 1 bps. or Tinct. opii simplex 3 times/day 10-20 trp. p.o.
Pellagroid symptoms: Nicotinamide (e.g. Nicobion) 200 mg/day p.o. or Nicotinic acid (e.g. Niconacid) 250 mg/day p.o.

Operative therapie
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Surgical removal of the primary tumour (usually partial resection of the small intestine) and the regional lymph nodes as early as possible, limited chances of success with a tumour size of > 2 cm.

Progression/forecast
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Five-year survival rate: 18%. Mean survival time: 2.5 years. Disease progressions of up to 20 years were observed.

Diet/life habits
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Low serotonin diet (avoid bananas, walnuts, tomatoes, pineapple, plums, mirabelle plums, melons, avocados, eggplants, kiwis and alcohol).

Note(s)
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The term carcinoid, although well established in medical terminology, is no longer sufficient to cover the entire morphological and biological spectrum of neoplasias of the disseminated neuroendocrine cell system.

The WHO classification of 2000 therefore uses the superordinate terms neuroendocrine tumour (NET) and neuroendocrine carcinoma (NEK) instead of the term carcinoid.

In detail, a distinction is made between neuroendocrine tumours with benign behaviour, questionable dignity, low-malignant behaviour and high-malignant behaviour in relation to their localisation and based on various morphological and biological criteria.

S.u. Neuroendocrine tumors of the gastroentero-pancreatic system (GEP-NET)

Literature
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Boushey RP et al (2002) Carcinoid tumors. Curr Treat Options Oncol 3: 319-326
Creutzfeldt W, Stöckmann F (1987) Carcinoid and Carcinoid Syndromes. On J Med 82: 4-16
Feldman JM (1987) Carcinoid tumors and syndrome. Semin Oncol 14: 237-249
Goede AC et al (2003) Carcinoid tumour of the appendix. Br J Surg 90: 1317-1322
Kähler HJ, Heilmeyer L (1961) Clinic and pathophysiology of carcinoid and carcinoid syndrome. Results Inn Med Pediatrics 16: 292-559
Lubarsch O (1888) On the primary cancer of the ileum together with remarks on the simultaneous occurrence of cancer and tuberculosis. Virchows Arch path Anat 111: 280-317
Moller JE et al (2003) Factors associated with progression of carcinoid heart disease. N Engl J Med 348: 1005-1015
Oberndorfer S (1907) Carcinoid tumors of the small intestine. Frankf Z Pathol 1: 425-429
Rodriguez J et al (2003) Combined typical carcinoid and acinic cell tumor of the lung: a heretofore unreported occurrence. Hum Pathol 34: 1061-1065