What is carcinoid tumor of the appendix?

From the Guidelines

Dome cancer of the appendix is a rare type of cancer that typically begins as a carcinoid tumor, mucinous neoplasm, or adenocarcinoma in the appendiceal tissue, and its management depends on the size and characteristics of the tumor, with appendicectomy being sufficient for classical NETs <1 cm in size, while right hemicolectomy is recommended for tumors >2 cm in size or with certain high-risk features 1. When considering the management of dome cancer of the appendix, it is essential to evaluate the size and characteristics of the tumor, as these factors significantly influence treatment decisions.

• For small tumors (<1 cm in size), appendicectomy is often sufficient, as stated in the guidelines for the management of gastroenteropancreatic neuroendocrine tumors (including carcinoid) tumors (NETs) 1.
• However, for larger tumors (>2 cm in size) or those with specific high-risk features such as serosal breach by tumor, cellular atypia, or invasion, right hemicolectomy is the recommended treatment approach 1. Key considerations in the management of dome cancer of the appendix include:
• The location of the tumor, with approximately 75% of NETs located near the tip of the appendix 1
• The type of tumor, with classical NETs, goblet cell tumors, and adenocarcinomas having different management strategies
• The importance of early detection and treatment, as outcomes are significantly improved with prompt medical evaluation and intervention.

From the Research

Definition and Types of Appendiceal Cancer

• Appendiceal cancer, also known as appendix cancer, is a rare type of cancer that occurs in the appendix, a small pouch attached to the large intestine 2.
• There are several subtypes of appendiceal cancer, including colonic-type adenocarcinoma, mucinous neoplasm, goblet cell carcinoma, and neuroendocrine neoplasm 2.
• Carcinoid tumors are a type of neuroendocrine tumor that can occur in the appendix, and are typically slow-growing and benign, but can become malignant in some cases 3, 4.

Symptoms and Diagnosis

• Appendiceal cancer is often found incidentally during surgery for acute appendicitis, and may not cause any symptoms in its early stages 5, 2.
• In some cases, appendiceal cancer may cause symptoms such as abdominal pain, nausea, and vomiting, especially if the tumor has grown large enough to block the appendix or has spread to other parts of the body 5.

Treatment and Management

• The treatment and management of appendiceal cancer depend on the subtype and stage of the disease, as well as the patient's overall health 2.
• Surgery is often the primary treatment for appendiceal cancer, and may involve removing the appendix (appendectomy) or a larger portion of the intestine (right hemicolectomy) 3, 4, 5.
• In some cases, chemotherapy or other treatments may be necessary to manage the disease, especially if it has spread to other parts of the body 6.

Prognosis and Outcome

• The prognosis and outcome for appendiceal cancer vary depending on the subtype and stage of the disease, as well as the patient's overall health 2.
• In general, the 5-year survival rate for appendiceal cancer is around 50-60%, although this can vary depending on the specific subtype and stage of the disease 2.
• Goblet cell carcinoid, a rare subtype of appendiceal cancer, has a poor prognosis, with a 5-year overall survival rate of 14-22% in stage III-IV disease 6.