Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Goblet cell carcinoid


Other Names for this Disease

  • Goblet cell carcinoma
  • Mucinous carcinoid
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My wife has been diagnosed with goblet cell carcinoma of the appendix. I am very concerned and can't find much information. Can you help? How can I find a specialist?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is goblet cell carcinoid?

Goblet cell carcinoid (GCC)is a rare type of neuroendocrine tumor that involves the appendix almost exclusively. It typically comes to attention in the fifth to sixth decade of life. Signs and symptoms may include acute appendicitis, followed by abdominal pain and a mass. In about fifty percent of affected females, the tumor has metastasized to the ovary by the time of diagnosis. In general, approximately 76% of individuals with a GCC are alive five years after the diagnosis. Prognosis largely depends on the stage of the disease (i.e. the size of the tumor and whether it has spread to other parts of the body). Treatment often includes appendectomy and right hemicolectomy, followed by adjuvant chemotherapy in some cases. However, there is some debate about the extent of surgical treatment for these tumors.[1]
Last updated: 6/25/2013

What are goblet cells?

Goblet cells are cells that line internal organs (such as the small intestine and lungs) and make mucus to cover and protect these organs.
Last updated: 3/22/2012

What is a carcinoid tumor?

Carcinoid tumor refers to a non-cancerous or cancerous (carcinoma) tumor that begins in the skin or in tissues that line or cover internal organs. Carcinoid tumors are a type of neuroendocrine tumor. They are most commonly located in the gastrointestinal tract, such as the stomach or the appendix.[2] They are also found in the lungs.[3]
Last updated: 1/19/2009

What are the most common gastrointestinal carcinoid tumors?

Carcinoid tumors of the appendix are among the most common types of gastrointestinal carcinoid tumors. They are often discovered accidentally during surgery and identified after additional testing of a tumor sample.[2][4]
Last updated: 1/19/2009

What are the signs and symptoms of goblet cell carcinoid?

The most common first signs in individuals with goblet cell carcinoid are acute appendicitis, abdominal pain and/or a lower abdominal palpable mass.[1] Symptoms of appendicitis may include pain and/or swelling in the abdomen; loss of appetite; nausea and vomiting; constipation or diarrhea; inability to pass gas; and/or a low fever.[5][4]

Other signs and symptoms may include bowel obstruction, intussusception, gastrointestinal bleeding, and chronic intermittent lower abdominal pain. Rare presentations have reportedly included mesenteric adenitis, and iron deficiency anemia due to cecal ulceration.[1]
Last updated: 6/25/2013

What is the long-term outlook for individuals with goblet cell carcinoid?

In general, 76% of individuals who are diagnosed with goblet cell carcinoid live at least five years after being diagnosed.  Important factors to consider when determining prognosis include the size and location of the tumor, and whether or not the cancer has spread to other parts of the body.[5][6]  If cancer cells have spread to distant parts of the body, the chances of survival are decreased.
Last updated: 6/25/2013

How might goblet cell carcinoid be treated?

Surgical resection (removing the abnormal tissue) is the primary treatment for goblet cell carcinoid (GCC). However, due to the condition's rarity, there is a lack of ample evidence or general consensus regarding the extent to which resection should be performed for different stages of this condition.[1]

Because of the typical course of the condition and the malignant nature of the tumors, treatment recommendations are more similar to that of adenocarcinomas rather than most carcinoids. Stage I tumors may be treated with appendectomy alone. However, in higher stages, a right hemicolectomy (RH) is the most commonly recommended surgical option despite controversy in the medical community. The justification for RH is to do adequate nodal sampling (when samples are taken to check for more cancer) because metastasis is common. Some researches have reported a lack of benefit from extensive surgery provided there is no nodal involvement. It has also been reported by some researchers that in patients studied, the 5-year survival rates were not significantly different between those treated with appendectomy and those who underwent RH.[1] Careful follow-up after surgery is highly recommended and may include periodic physical examinations, blood testing, and imaging studies.[6] In some cases, adjuvant chemotherapy is also recommended.[1]

We are unable to give advice to individuals regarding the best course of treatment. We recommend speaking with your health care provider to discuss treatment options.
Last updated: 6/25/2013

How can I learn more about the treatment of goblet cell carcinoid?

You can find articles that discuss the treatment of goblet cell carcinoid in PubMed, a searchable database of medical literature. Some articles are available as a complete document, while information on other studies is available as a summary.  To obtain the full article, contact a medical, university, or local library, or order it through the PubMed web site: http://pubmed.gov/. You can contact the NLM toll-free at 888-346-3656 to locate libraries in your area.
Last updated: 6/25/2013

How can I find local specialist and specialty centers?

The National Cancer Institute (NCI) has a resource page that lists cancer centers and comprehensive cancer centers. Click here to learn more about what it means to be designated a 'cancer center' or 'comprehensive cancer center.' To search for designated NCI cancer centers visit the link below.
https://cissecure.nci.nih.gov/factsheet/FactsheetSearch.aspx?FSType=1.2

In addition, the following support organizations provide information and supportive resources for people with carcinoid tumors and their families. Support organizations are often able to help people locate local specialist and specialty centers. We recommend that you call the groups directly to see if they can assist you with this request.

The Carcinoid Cancer Foundation, Inc.
333 Mamaroneck Avenue
Suite 492
White Plains, NY 10605
Toll-free: 888-722-3132
Telephone: 914-683-1001
Fax: 914-683-5919
Web site: http://www.carcinoid.org/  

Caring for Carcinoid Foundation
One Kendall Square
PMB 180
Cambridge, MA 02139
Telephone: 857-222-5492
E-mail: info@caringforcarcinoid.org
Web site: http://www.caringforcarcinoid.org/

American Cancer Society
Toll-free: 800-ACS-2345
Web site: http://www.cancer.org/
Last updated: 1/20/2009

How can I learn about research involving goblet cell carcinoid?

The National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. While no studies involving goblet cell carcinoid specifically are listed at this time, there are hundreds of studies enrolling patients with neuroendocrine tumors in general. To find these trials, click on the link above and use 'neuroendocrine' as your search term. After you click on a study, review its 'eligibility' criteria to determine its appropriateness. Use the study’s contact information to learn more.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if your wife is eligible for any clinical trials.

Patient Recruitment and Public Liaison Office (PRPL)
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
E-mail: prpl@mail.cc.nih.gov  
Web site: http://clinicalcenter.nih.gov/

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand

A tutorial about clinical trials that can also help answer questions and can be found at the following link from the National Library of Medicine. 
http://www.nlm.nih.gov/medlineplus/clinicaltrials.html

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases (ORD), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8
Last updated: 6/25/2013

References
  • Paromita Roy and Runjan Chetty. Goblet cell carcinoid tumors of the appendix: An overview. World J Gastrointest Oncol. June 15, 2010; 2(6):251–258. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2998842/. Accessed 6/25/2013.
  • Modlin IM, Kidd M, Latich I, Zikusoka MN, Eick GN, Mane SM, Camp RL. Genetic differentiation of appendiceal tumor malignancy: a guide for the perplexed. Ann Surg. 2006 Jul;
  • Gordon R, Burns K, Friedlich M. Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma. Neuroendocrinology. 2008;
  • Byrn JC, Wang JL, Divino CM, Nguyen SQ, Warner RR. Management of goblet cell carcinoid. J Surg Oncol. 2006 Oct 1;
  • Plöckinger U, Couvelard A, Falconi M, Sundin A, Salazar R, Christ E, de Herder WW, Gross D, Knapp WH, Knigge UP, Kulke MH, Pape UF; Frascati. Consensus Conference participants. Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma. Neuroendocrinology. Epub 2007 Oct 11 -7;
  • Roy P, Chetty R. Goblet cell carcinoid tumors of the appendix: An overview. World Journal of Gastrointestinal Oncology. 2010; 2:251-258. http://www.ncbi.nlm.nih.gov/pubmed/21160637. Accessed 3/21/2012.
Other Names for this Disease
  • Goblet cell carcinoma
  • Mucinous carcinoid
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.