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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Goblet cell carcinoid


Other Names for this Disease
  • Goblet cell carcinoma
  • Mucinous carcinoid
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Overview



What is goblet cell carcinoid?

What are the signs and symptoms of goblet cell carcinoid?

How might goblet cell carcinoid be treated?


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 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.[2][3]

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

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.[4] 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

References
  1. 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.
  2. 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;
  3. Byrn JC, Wang JL, Divino CM, Nguyen SQ, Warner RR. Management of goblet cell carcinoid. J Surg Oncol. 2006 Oct 1;
  4. 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.