Goblet cell adenocarcinoma is a rare form of appendix cancer that can spread throughout your abdomen without treatment. The first symptoms are usually the same as in appendicitis. Depending on the tumor’s grade and stage, treatments include surgery and chemotherapy.
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Goblet cell adenocarcinoma (GCA) is an extremely rare and aggressive form of appendix cancer. In the past, experts considered it a type of appendix cancer called a neuroendocrine tumor (NET). But now, they classify it as an adenocarcinoma. This type is more likely to spread and harder to treat.
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Without early treatment, tumors can spread to the tissue layer that lines your abdomen (belly) and abdominal organs. This layer is called your peritoneum. When tumors spread there, it’s called peritoneal carcinomatosis. It’s a sign of advanced disease.
Like many early-stage cancers, GCA can be cured when surgery removes all of the tumor. Surgery and chemotherapy can help people with advanced GCA live longer with fewer symptoms.
This cancer has puzzled medical experts because tumors have cells that are like both neuroendocrine tumors (NETs) and adenocarcinomas. Deciding how to classify GCA is important, as these cancers behave differently. Now we know it’s more like an adenocarcinoma.
To better account for how their mixed cell make-up shapes how aggressive they are, the World Health Organization (WHO) also classifies them based on grade. GCA may be:
When GCA spreads, it usually does so within your abdomen (peritoneal carcinomatosis). It can spread to distant organs, but this is rare.
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Symptoms are often like those of acute appendicitis. They include:
The condition happens when the genes inside cells in your appendix change (mutate) and grow out of control. Experts don’t know what causes the change.
This condition doesn’t run in families, so it’s not hereditary. Most people diagnosed are in their 50s. But people as young as 25 to 30 have also been diagnosed with this rare cancer.
Diagnosis may happen during a workup to see what’s causing your symptoms or incidentally during imaging performed for other reasons. But often, healthcare providers discover the cancer cells while delivering treatment for suspected appendicitis.
After appendix removal surgery (the standard treatment for appendicitis), they examine the removed appendix beneath a microscope. Once your healthcare provider tests the tissue and finds the cancer cells, they can diagnose GCA. They’ll also determine its grade.
An important next step is to see if it’s spread. You may need imaging tests, like a CT scan, MRI or PET scan. You may need a laparoscopy. During this minimally invasive procedure, your surgeon will look for tumors in your belly.
Providers stage goblet cell adenocarcinoma to see how advanced it is. They consider the extent of the tumor’s growth and whether it has spread to lymph nodes or distant organs. Then, they assign a number from 1 to 4, with 1 being early-stage and 4 being most advanced.
Just under half of new cases have spread to the peritoneum, liver or ovaries at diagnosis. High-grade tumors are more likely to be diagnosed at advanced stages.
Treatment depends on the tumor stage and grade.
If it wasn’t done as part of your diagnosis, your surgeon will remove your appendix. They may also remove a large portion of your colon (right hemicolectomy). This surgery is important because it removes the lymph nodes in the area where the cancer may have spread. Afterward, they’ll reattach the remaining parts of your colon.
Other treatments may include:
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Your provider will recommend follow-up visits to see how treatment is working. These visits are important even when treatment gets rid of the cancer. Your provider will monitor you for signs that the cancer has come back, so you can get treatment ASAP, if needed.
Early-stage cancers may be curable with surgery. The prognosis (outlook) is worse if tumors have spread to your peritoneum. Treatments like cytoreductive surgery and HIPEC can help manage symptoms. And they can help you live longer.
The five-year survival rate for GCA ranges from 58% to 81%. Tumor grade is a key factor when it comes to life expectancy:
Still, it’s important to remember that these numbers can’t predict your life expectancy. They only highlight one part of a diagnosis: the grade. And they report on other people’s past experiences. They may be very different from yours.
Also, researchers are still learning about the prognosis of GCA. As these tumors are so rare, there’s still a lot to learn.
This is why your healthcare provider is your best resource for helping you understand what to expect.
Appendix cancer comes in different forms, including rare ones like goblet cell adenocarcinoma (GCA). If you have this disease, it’s important to know how the grade and stage will impact your treatment and prognosis. Experts still have lots to learn about this cancer. But your healthcare provider can explain what comes next based on your circumstances and what researchers do know.
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