Your appendix is a narrow tube-shaped sac that connects to your colon near the beginning of your large intestines. The purpose of yourappendix仍然没有明确，但一些科学家认为它可能是免疫系统的一部分。

附录癌有时称为阑尾癌。当健康的细胞变得异常并迅速增长时，会发生它。这些癌细胞成为附录内部的肿块或肿瘤，其在外科清除附录后经常发现。

附录癌被认为是罕见的。在美国，根据 国家癌症研究所 。

In this article, we review the different types and classifications of appendix cancer, along with symptoms, risk factors, and treatment options.

There are several classifications of appendix cancers, but they’re not well defined. That’s due to the rarity of this type of cancer, which limits the amount of research.

附录癌的分类如下：

神经内分泌癌

In this type, sometimes known as typical carcinoid, a tumor forms with certain cells from the wall of the bowel.

它占了约 一半 of all appendix cancers. It can metastasize, or spread, but can be successfully treated with surgery.

Mucinous adenocarcinoma of the appendix

Mucinous adenocarcinoma of the appendix, also called MAA, occurs in females and males equally. The average age of onset is around 60岁 。没有已知的危险因素的发展ent. The cancer cells produce a jelly-like substance called mucin that can spread cancer to other body parts.

Most of the time, MAA is discovered after a rupture of the tumor that leads to the release of mucin.

Colonic-type adenocarcinoma

This type of cancer accounts for10 percentof appendix cancers and acts similar to colorectal cancer. It usually appears in people between the ages of 62 and 65 and is more common in men than women.

Colonic-type adenocarcinoma usually starts in the base of your appendix.

脚耳细胞腺癌

脚杯细胞致癌物质也称为GCC。它在神经内分泌细胞和一种被称为脚尖细胞的上皮细胞中，产生一种称为粘蛋白的果糖状物质。

GCC most often onsets between the ages of50 to 55。Typically, it causes either appendicitis or abdominal pain with a mass.

Signet ring cell adenocarcinoma

Signet ring cell adenocarcinoma is considered a subtype of colonic-type adenocarcinoma or mucinous adenocarcinoma.

While it’s the most aggressive type and most likely to spread to other organs, it’s very rare. This type more commonly occurs in the colon or stomach but can develop in the appendix as well.

More than 一半的人 with appendix cancer don’t have symptoms. It’s usually discovered during surgery or an imaging test for another condition likeappendicitis。

Your doctor may also discover it during a routinecolonoscopy。但是，如果有症状，他们可能包括：

Many of these symptoms may not occur until the cancer is more advanced. In about a 三分之一的案件 ，癌症已经在诊断时已经蔓延到身体的遥远部分。

Doctors often use theTNM分期系统to describe the extent of appendix cancer. TNM stands for

  • 瘤。The extent of the cancer in your appendix.
  • Node.Whether the cancer has reached your lymph nodes.
  • 转移。癌症是否蔓延到胰腺之外。

Each of three aspects of the tumor is staged and plays a role in determining the overall stage of the cancer.

Tumor

The size and location of the appendiceal tumor are given a score from T0 to score T4b:

Stage 描述
T0 没有癌症的证据
Tis Evidence of cancer in situ, or cancer cells in the first layer of your appendix
T1 附录的下一层癌细胞的证据称为粘膜
T2 The cancer has entered a deep layer of your appendix called the muscularis propria
T3 The cancer has spread into a layer of connective tissue called the subserosa or the blood supply of your appendix called the mesoappendix
T4 The cancer has grown into the lining of your abdominal cavity or nearby organs
T4a Evidence of the tumor in the visceral peritoneum, the tissue that covers the outer surface of most organs
T4b Cancer is present in other organs or structures such as your colon

Node

The extent that your cancer has invaded your lymph nodes is given a score from N0 to N2:

Stage 描述
N0 周围淋巴结没有癌症
N1 Cancer is in 1 to 3 lymph nodes in the area
N2 癌症在该地区的4个或更多淋巴结中

Metastasis

The extent that the cancer has spread to other parts of your body is given a score from M0 to M1c:

Stage 描述
M0 Cancer has not spread to other parts of your body
M1 Cancer has spread to other parts of your body
M1a 它已蔓延至a particular region called the intraperitoneal acellular mucin
M1b It has spread within the peritoneum further than stage M1a
M1c 它超越了腹膜

Grade

Doctors assign a grade to your cancer based on how different cancer cells look from healthy cells under a microscope. A higher-grade cancer is expected to spread quicker.

Appendiceal cancer is given a grade from G1 to g4:

Grade 描述
G1. 肿瘤细胞看起来类似于健康的细胞
G2. Tumor cells look fairly different from healthy cells
G3. Tumor cells look very different from healthy cells
G4. The tumor cells look distinct compared to healthy cells

TNM分期系统

医生根据TNM和等级分数为您的癌症分配整体阶段。较高阶段意味着癌症进一步发展并预后更糟糕。

The table below shows staging forappendiceal carcinomas。It’s worth noting that the staging descriptions for other types of appendix cancers — such as附录的神经内分泌肿瘤— are slightly different.

Stage 描述
Stage 0 Tis + N0 + M0
Stage 1 T1 or T2 + N0 + M0
Stage 2A T3 + N0 + M0
Stage 2B T4a+ N0 + M0
Stage 2C T4b+ N0 + M0
第3A阶段 T1 or T2 + N1 + M0
第3B阶段 T3或T4 + N1 + M0
第3C阶段 任何t + n2 + m0
第4A阶段 Any T + N0 + M1aor任何t +任何n + m1b + g1
Stage 4B Any T + any N + M1b + G2 or G3
Stage 4C 任何t +任何n + m1c +任何g

The cause of appendix cancer is largely unknown and no avoidable risk factors have been identified. However, appendix cancer becomes more common with age and is rare in children.

Neuroendocrine tumors are more common inwomen than men。

Although more research is needed, a few potential risk factors have been suggested by experts, including:

  • 恶性贫血, a deficiency of vitamin B12
  • atrophic gastritis, or long-term inflammation of the stomach lining
  • Zollinger-ellison综合症, a condition of the digestive tract
  • a family history of multiple endocrine neoplasia type 1 (MEN1), a disorder that leads to tumors in the glands that produce hormones
  • smoking

The treatment for appendix cancer depends on the:

  • 肿瘤类型
  • 阶段的癌症
  • person’s overall health

一支多学科医疗专业人士团队将通过您的待遇来帮助您。您的团队将包括各种专业人士，可能包括医生，护士从业者，营养师，辅导员等等。一种称为外科肿瘤科医生的医生将在您的癌症上运作，而医疗肿瘤科医生将发展您的化疗计划。

手术

手术is the most common treatment for localized appendix cancer. If the cancer is localized to the appendix only, the treatment is usually to remove the appendix. This is also called anappendectomy。

For some types of appendix cancer, or if the tumor is larger, your doctor may recommend removing one-half of your colon and also some lymph nodes. Surgery to remove half of your colon is called aHemicolectomy.。

If the cancer has spread, your doctor may recommend cytoreductive surgery, also called debulking. In this type of surgery, the surgeon will remove the tumor, surrounding fluid, and possibly any nearby organs that are attached to the tumor.

Chemotherapy

Treatment may include chemotherapy before or after surgery if:

  • 肿瘤大于2厘米
  • the cancer has spread, especially to the lymph nodes
  • 癌症更具侵略性

Types of化疗include:

  • systemic chemotherapy given intravenously or by mouth
  • 将区域化疗直接置于腹部，如腹腔化疗（史诗）或高温腹膜内化疗（高度高温）
  • 系统性和区域化疗的组合

放射治疗

放射治疗is rarely used to treat appendix cancer. However, it may be recommended if your cancer spreads to other body parts.

Imaging tests

After surgery, your doctor will follow up with imaging tests, such as aCT scanorMRI.，确保肿瘤消失了。

Since appendix cancer is such a rare condition, there’s little information available about its recurrence or survival rates.

According to the American Society of Clinical Oncology, , the 5-year survival rate for Grade 1 and Grade 2 neuroendocrine tumors is between67 to 97 percent。已经蔓延到其他身体部位的先进附录癌的存活率较低。

癌症类型之间的存活率也各不相同。神经内分泌肿瘤具有最高的存活机会，而饰物环细胞肿瘤的血液肿瘤最低的生存率最低 27 percent 。

当另外的结肠成分的情况下，5年的存活率增加了一些案例，并且使用了化学疗法。然而，并非所有附录癌症都需要这些额外的治疗方法。

The survival rate and outlook are generally good for most people with early-stage appendix cancer.

In most cases, appendix cancer goes undetected until an appendectomy is already being performed for other reasons.

在任何癌症诊断后，很重要的是与医生定期跟进，以确保癌症没有复发。