Cancer from A to Z

Types of cancer, how to prevent them, diagnosis and treatment.


Appendix Cancer

Tumors that occur in the appendix comprise a large group of both benign and malignant diseases. Appendix cancer is extremely rare. Most patients are diagnosed after undergoing surgery for acute appendicitis or when an abdominal mass is seen during a CT scan for an unrelated condition.

Tumor size is directly related to prognosis (outlook). Appendix tumors less than two centimeters have a low tendency to spread and patients generally have an excellent five-year survival rate, while tumors larger than 2.5 centimeters may require more aggressive treatment and patients have a lower survival rate.

Tumors of the appendix can be divided into two major groups:

Carcinoid Tumors

Carcinoid tumors are the most common form, representing about half of all appendix tumor cases. These tumors are more common in women, and generally occur in the fourth decade of life. About two-thirds of carcinoid tumors are found in the tip of the appendix, where they do not obstruct the opening between the appendix and colon.

In the majority of cases, carcinoid tumors are small (less than two centimeters) and have a very low risk of spread to nearby lymph nodes. Appendectomy (removal of the appendix) is adequate treatment in these cases. Larger tumors have a slightly higher risk of metastasizing to surrounding lymph nodes and the liver. Removal of the right colon and regional lymph nodes is recommended.

More than 85% of patients with carcinoid tumors of the appendix (even with regional metastases) have a five-year survival rate.

Most patients have no symptoms. However, some signs may include:

  • Acute appendicitis: most cases are discovered during surgery for appendicitis
  • Carcinoid syndrome (if spread to liver): flushing, shortness of breath, diarrhea, heart valve disease on the right side

Non-Carcinoid Tumors

Non-carcinoid tumors of the appendix originate from the epithelial cells lining the inside of the appendix. Most of the cells creating these tumors produce a gelatinous material known as mucin. Due to the anatomic structure of the appendix and the mucin production of these tumors, patients with non-carcinoid tumors of the appendix are at risk for spread of tumor cells and mucin within the abdominal cavity. If left untreated, both tumor cells and mucin can accumulate over time and lead to life-threatening bowel obstruction and cachexia(weight loss, muscle atrophy, significant loss of appetite, fatigue). Prognosis and treatment for these tumors depend on a number of patient and tumor-related factors.

Pseudomyxoma peritonei (PMP) is defined by the presence of acellular (i.e., few, if any, tumor cells) mucin within the abdominal cavity. The most common presentation of PMP is a mucin-producing tumor of the appendix whose cells have spread outside the appendix and into the abdominal cavity. The resulting tumor, which looks like orange jelly, can get as large as 20-30 pounds.

There are no PMP-specific symptoms, but some of the more frequent presenting signs include:  

  • Increase in abdomen size/girth
  • Vague abdominal discomfort; persistent discomfort in the lower right abdomen
  • Pelvic discomfort
  • New hernias
  • Bowel obstruction
  • Ovarian masses

Adenocarcinoid tumors, also known as goblet cell carcinomas, have characteristics similar to both carcinoid and adenocarcinoma tumors of the appendix. Most patients are diagnosed in their 50s. The prognosis for these tumors is slightly poorer than that of carcinoid tumors, with an overall five year survival rate of 78%. However, only about 14% of patients with Stage IV disease survive longer than five years. The main presenting symptom is acute or chronic abdominal pain.

Appendix cancer may not cause any specific symptoms until it has progress into advanced stages. However, abdominal discomfort and bloating is sometimes experienced with advanced disease.

The meeting will provide an educational venue in which current topics of relevance to practicing pathologists and other physicians, academic and non-academic, are presented and discussed.
Current practice and controversies in the era of personalized medicine.
Coordinadores:  Dr. Miguel ángel Sanz  Hospital Universitario y Politécnico la Fe. Valencia, España Dr. Adolfo de la Fuente MD Anderson Cancer Center Madrid, España
Clinical trials
Ensayo de fase 1a/2a, abierto y multicéntrico, para investigar la seguridad, tolerabilidad y actividad antitumoral de dosis repetidas de Sym015, una mezcla de anticuerpos monoclonales dirigida frente al receptor MET, en pacientes con tumores malignos sólidos en fase avanzada
Estudio fase IIIB, prospectivo, randomizado, abierto que evalúa la eficacia y seguridad de Heparina/Edoxaban versus Dalteparina en tromboembolismo venoso asociado con cáncer.
Tumores sólidos. Antiemesis Estudio fase III, multicéntrico, aleatorizado, doble ciego, con control activo para evaluar la seguridad y eficacia de Rolapitant en la prevención de náuseas y vómitos por la quimioterapia (NVIQ) en pacientes que reciben quimioterapia altamente emética (QAE). A phase III, multicenter, randomized, double blind, placebo controlled study of the safety and efficacy of Rolapitant for the treatment of Chemotherapy-induced nausea and vomiting in subjects receiving highly Emetogenic Chemotherapy (HEC)
Ensayo clínico en fase I de determinación de dosis del antiangiogénico multidiana Dovitinib (TKI258) más paclitaxel en pacientes con tumores sólidos.

Appendix cancer has no known cause. There are no known lifestyle changes that one can make to lower the risk of developing appendix cancer.

Often, appendix cancer remains undiagnosed until it is unexpectedly found during or after abdominal surgery or when an abdominal mass is seen during a CT scan for an unrelated condition. If appendix cancer is suspected during abdominal surgery, the surgeon would take a tissue sample (biopsy) for a pathologist to review. Otherwise, the cancer may be diagnosed after your abdominal surgery as part of the routine pathology reading. In that case, another surgery would most likely be recommended to make sure all the cancerous tissue is removed.

Treating appendix cancer depends on the type of tumor, its location within the appendix and whether or not it has spread. Because these tumors are relatively rare, particularly the non-carcinoid tumors, most treatment strategies have not been tested in standard clinical trials. In general, patients will undergo surgery to remove the appendix and/or right colon.

For patients with non-carcinoid tumors that have spread within the abdominal cavity, the most effective therapy appears to be a combined approach of cytoreductive (tumor debulking) surgery and hyperthermic intraperitoneal (i.e., in the abdominal cavity) chemotherapy.

Cytoreductive surgery is a major operation to remove all the tumor and mucin present within the abdominal cavity. This can often require removing involved organs such as parts of the intestine, gallbladder, spleen, ovaries, uterus and even the lining of the abdominal cavity (peritoneum). In patients whose tumors can be completely or nearly completely removed by surgery, administration of hyperthermic intraperitoneal chemotherapy has been shown to significantly improve survival.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is performed only if a complete tumor debulking is achieved. During the surgery, the abdominal cavity is filled with a chemotherapy drug, which is heated to over 40 degrees Celsius. The patient's abdomen is then gently rocked back and forth for 90 minutes to ensure that the chemotherapy bathes all areas of the abdominal cavity, killing any remaining tumor cells.

Currently there is no published data supporting the use of systemic (intravenous) chemotherapy for patients with non-carcinoid appendical tumors. However, there may be an evolving role for the systemic therapies in the management of metastatic colorectal cancer. There is no known role for radiation therapy in the treatment of cancers of the appendix.