Malignant Carcinoid Syndrome Follow-up

  • Author: Luigi Santacroce, MD; Chief Editor: Jules E Harris, MD   more...
 
Updated: Jan 27, 2012
 

Further Inpatient Care

A systematic psychotherapeutic intervention may be very helpful in patients with carcinoid.

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Further Outpatient Care

As with inpatients, a systematic psychotherapeutic intervention may be very helpful.

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Inpatient & Outpatient Medications

  • Always assess the color of the urine. Dark-brown or intensely yellow urine usually indicates dehydration, and sometimes fluids must be given intravenously to keep up with the fluids lost in the diarrhea.
  • Also, obtain the patient's weight daily and replace the essential elements and vitamins, either by diet or by commercial supplements.
  • Other clinical problems associated with carcinoid syndrome, such as tachycardia and asthmalike attacks, should be managed by a cardiologist and pulmonologist.
  • Severe flushes may be treated with clonidine hydrochloride.
  • Treatment must include avoidance of conditions or substances that cause flushing (eg, stress, alcohol, epinephrine, epinephrinelike drugs).
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Deterrence/Prevention

Patients must avoid any physical and emotional stress and any food or drug that may trigger a crisis. For example, monamine oxidase inhibitors should be avoided because they can exacerbate the syndrome by inhibiting serotonin degradation.

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Complications

  • Hypotension related to massive vasodilation can increase the risk of falls and subsequent injuries.
  • Increased platelet aggregation causes a high risk for strokes, deep venous thromboses, and similar disorders.
  • Gastrointestinal bleeding is very rare. Bowel obstruction from tumor mass or, most frequently, from mesenteric adhesions is also very rare.
  • Right-sided heart failure is more frequent, as noted above.
  • Analysis of catecholamines and associated metabolites shows consistent elevation of dopamine, norepinephrine, and epinephrine metabolites in more than 30% of midgut carcinoids. This explains some adverse effects (eg, arrhythmias, perioperative carcinoid crisis) in patients in whom octreotide premedication is indicated.
  • Usually, urinary levels of histamine metabolites are less elevated, but increased histamine production can cause specific symptoms (eg, angioedema), which should be treated specifically.
  • Acute renal failure after liver chemoembolization of carcinoid metastases is an infrequent but well-known complication.
  • Risk of metastasis correlates with size of primary tumor, as follows:
    • Tumors ≤ 1 cm metastasize in only 2% of cases
    • Tumors 1-2 cm metastasize in 50% of cases
    • Tumors ≥ 2 cm metastasize in 85% of cases
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Prognosis

  • The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median survival rate after 5 years is 30-67%. Survival for 8.5 years and 23 years has been reported.
  • In patients with malignant carcinoid syndrome who are cured with surgery, follow-up care does not need to be intense. For patients with advanced disease, follow-up care should be scheduled on a regular basis. The frequency of follow-up care depends on the general condition of patients and their disease. In each follow-up session, blood tests, x-ray films, and other studies may need to be performed complementary to patient examination.
  • For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patient’s perception of symptoms and the influence of treatment, which may affect quality of life as well as disease progression.[43, 44]
  • The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people, and the best treatment remains controversial. Lymph nodes and liver are the most common sites of metastasis, and bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
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Patient Education

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Contributor Information and Disclosures
Author

Luigi Santacroce, MD  Assistant Professor, Medical School, State University at Bari, Italy

Disclosure: Nothing to disclose.

Coauthor(s)

Laura Diomede  University of Bari School of Medicine, Italy

Disclosure: Nothing to disclose.

Lodovico Balducci, MD  Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute

Disclosure: Nothing to disclose.

Specialty Editor Board

Sanjiv S Agarwala, MD  Chief of Oncology and Hematology, St Luke's Cancer Center, St Luke's Hospital and Health Network; Professor, Temple University Shool of Medicine

Sanjiv S Agarwala, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Head and Neck Surgery, American Society of Clinical Oncology, Eastern Cooperative Oncology Group, and European Society for Medical Oncology

Disclosure: BMS Honoraria Speaking and teaching; Novartis Consulting fee Consulting; Merck Consulting fee Consulting

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD  Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center

Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research

Disclosure: GlobeImmune Salary Consulting

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A section (on the right) of an intestinal carcinoid mass arising from the mucosa (150 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
A section of a carcinoid mass (350 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
A section of a rare lymph node metastasis from adenocarcinoid tumor (250 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
 
 
 
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