Appendix Cancer

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An anatomical diagram showing the appendix and peritoneum, both relevant to abdominal cancers.

What is Appendix Cancer?

Appendix cancer is a rare malignancy that originates in the appendix, a small tubular organ connected to the beginning of the colon. There are numerous types of appendix cancer, but most cases present as pseudomyxoma peritonei (PMP), characterized by pools of mucin that can spread throughout the abdominal cavity.

The Basics

Diagnosis and Prognosis

Because appendix cancer is rare, it is often discovered during an appendectomy for suspected appendicitis. However, other symptoms include abdominal bloating, pelvic pain, unexplained weight changes, and "Jelly Belly"—the accumulation of mucinous material in the abdomen. Since these symptoms overlap with other conditions, specialized evaluation with imaging and tumor markers is essential for an accurate diagnosis.

The standard of care for advanced appendix cancer is a combination of Cytoreductive Surgery (CRS) and Heated Intraperitoneal Chemotherapy (HIPEC). CRS involves the surgical removal of all visible tumors, while HIPEC delivers heated chemotherapy directly into the abdominal cavity to target remaining microscopic cancer cells. Survival outcomes are highest when a complete removal of all visible disease is achieved by a specialized surgical team.

The prognosis for appendix cancer is highly dependent on the specific tumor grade and the ability to achieve a complete surgical removal of the disease.

Low-Grade Tumors (DPAM/LGMCP): These have the most favorable outlook, with 10-year survival rates between 75% and 82% and a median overall survival of more than 15 years after successful treatment.

High-Grade Tumors (PMCA/HGMCP): These are more invasive, with a median overall survival of 8 to 10 years following CRS/HIPEC.

Aggressive Subtypes: For Signet ring cell carcinoma, the median overall survival is approximately 3 years, though this may improve to 6 years if the lymph nodes are negative.

Key Success Factor: Regardless of the subtype, the best survival outcomes are achieved when a specialized surgical team performs a complete removal of all visible tumors via Cytoreductive Surgery (CRS) and HIPEC. Without this intervention, survival for advanced cases significantly shortens to 2–5 years.

Frequently Asked Questions

The Basics

Appendix cancer, also known as appendiceal cancer, is a rare malignancy that originates in the appendix, a small tubular organ connected to the beginning of the colon occurs in less than 1% of all appendectomies. [see References: 1] The exact function of the appendix is unknown, but more recent studies suggest that it may house certain bacteria that aid in digestion and help replenish the digestive tract after a gastrointestinal illness.[2] It originates from a malignant, mucinous growth in the appendix, the small tubular organ connected to the colon. Because of its location and often subtle early presentation, these tumors cannot always be detected with a colonoscopy. The exact function of the appendix is unknown, but more recent studies suggest that it may house certain bacteria that aid in digestion and help replenish the digestive tract after a gastrointestinal illness.[2] Appendix cancers are very diverse and include several distinct types of tumors, ranging from mucinous neoplasms to goblet cell carcinomas and neuroendocrine tumors.

In addition to histologic tumor subtype, appendix cancers are also classified by “grade,” which describes how abnormal the cancer cells appear under a microscope and reflects how aggressive the tumor is. Low-grade tumors tend to grow more slowly, while high-grade tumors are more aggressive.

Appendix cancer most commonly presents as pseudomyxoma peritonei (PMP), a clinical syndrome characterized by accumulation of mucin and widespread peritoneal disease within the abdominal cavity. Mucin is a jelly-like substance that the body normally produces to protect the lining of the stomach, intestines, and the appendix. In some appendix cancers, tumor cells produce excess mucin which can cause the appendix to rupture, and lead to abdominal spread. PMP is classified as either a low-grade, less aggressive form, known as disseminated peritoneal adenomucinosis (DPAM) or low-grade mucinous carcinoma peritonei (LGMCP), or a more invasive form known as peritoneal mucinous carcinomatosis (PMCA) or high-grade peritoneal carcinoma peritonei (HGMCP).[3] Overall 10-year survival for PMP is approximately 32%,[4, 5] though outcomes vary significantly by tumor grade and completeness of surgical removal.

When detected early, many types of appendix cancer are highly treatable. Cytoreductive surgery and heated intraperitoneal chemotherapy (CRS/HIPEC) is considered the standard of care treatment for all types of advanced appendix cancers. CRS refers to the aggressive surgical removal of all visible tumors. HIPEC is the perfusion of heated chemotherapy throughout the abdominal cavity and is performed immediately following cytoreductive surgery.

Risk Factors

Age: Average age of diagnosis is 40 years
Gender: Occurs more frequently in females
Smoking tobacco
Family history of appendix cancer (or other gastrointestinal tumors)
Certain medical conditions that affect the stomach’s ability to make acid, such as atrophic gastritis or Zollinger-Ellison syndrome.

Person sitting with head resting on knees, illustrating signs and symptoms of appendix cancer, a type of abdominal cancer.

Signs & Symptoms

Appendicitis: Most cases are found during appendectomy for appendicitis
Abdominal bloating
Abdominal or pelvic pain/tenderness
Unexplained weight gain/loss
Changes in bowel function
Increase in waist size / fluid buildup in your abdomen, known as“Jelly Belly” - a nickname for the accumulation of mucinous, jelly-like material in the abdomen.

Evaluation

Physical exam
Imaging studies: CT scan, MRI or PET/CT scan
Blood work to include tumor markers (CA 19-9, CEA, CA 125, CRP)
Diagnostic laparoscopy may be recommended to assess the amount and location of the tumor or to determine if the tumor can be completely removed by CRS/HIPEC.

Key Facts

Rarity

Appendix cancer is rare and often initially misdiagnosed, making it important that specialized peritoneal surface malignancy surgeons and pathologists carefully review your case.

Survival Outcomes
Survival outcomes vary significantly by tumor subtype. Low-grade mucinous tumors are associated with the longest survival, while high-grade tumors, especially those with signet ring cells or goblet cells, tend to behave more aggressively.

Complete CRS/HIPEC
In appropriately selected patients with peritoneal disease, regardless of tumor subtype, the best survival is achieved with complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). In other words, the complete removal of all visible disease.

Chemotherapy

Given the lack of clear evidence, systemic chemotherapy should be reserved for patients who are not candidates for CRS/HIPEC, have high-risk pathology to help decrease recurrence after complete CRS/HIPEC, or are in a clinical trial.

Finding Specialized Care

Finding a care provider who is knowledgeable with appendix cancer and how to treat it is critically important. To search for providers who specialize in performing CRS/HIPEC and providers who specialize in appendix cancer, navigate to our Find a Specialist page.

Find a Specialist

please note: Our goal is to provide information to help you find a doctor closest to your home that can provide the best quality of care for your diagnosis or your anticipated CRS/HIPEC procedure. The Abdominal Cancers Alliance does not endorse any care provider or medical center over another.

Treatment

When detected early, many types of appendix cancer are highly treatable. Because appendix cancers vary widely in their biology and aggressiveness, treatment options depend on the tumor subtype, stage, and extent of disease. Surgery, particularly CRS/HIPEC for advanced disease, is the cornerstone treatment for most patients.

Surgery:

Almost all appendix cancers will be treated with surgery. The extent of surgery depends on the tumor type, grade, size, and whether or how much it has spread beyond the appendix.

For low-grade tumors confined to the appendix, a simple appendectomy (the surgical removal of the appendix) may be the only treatment needed. If the tumor extends into the cecum, an extended cecectomy (the surgical removal of the cecum, the first part of the large intestines) will be needed to remove all cancerous tissues.

For high-grade tumors that have not developed a hole in (or perforated/pushed through) the appendix wall, a right hemicolectomy (the surgical removal of part of the colon) will be needed to remove the portion of the colon surrounding the appendix along with nearby blood vessels and lymph nodes. This allows for accurate staging, assessment of lymph nodes are not involved and to make sure that all tumors are completely removed.[6]

If the tumor has perforated the appendix and spread throughout the abdominal cavity, then a more extensive cytoreductive surgery will be performed. This procedure involves the removal of all visible tumor deposits and any involved organs or structures.[7] Longer survival is associated with less amount of disease remaining after surgery.[8, 9] Because appendix cancer is rare and treatment can be complex, it is important to have a surgeon who is experienced with appendix cancers perform your surgery.

Chemotherapy:

In some cases, appendix cancer may be treated with systemic chemotherapy, commonly known as intravenous (or IV) chemotherapy. For patients with extensive disease, chemotherapy may be recommended prior to surgery (neoadjuvant therapy) to reduce tumor burden and increase the likelihood of a successful operation with complete tumor removal.[7, 10] Depending on surgical results and tumor type, postoperative chemotherapy may be recommended. The most commonly used agents/regimens are adopted from treatments used for colon cancer, including FOLFOX, FOLFIRI, with or without bevacizumab (Avastin). Sometimes, XELODA may be used as maintenance therapy.[11, 12]

However, it is important to note that the role of systemic chemotherapy in appendix cancer is poorly defined and the benefit is not well established. To date, there are no randomized clinical trials studying the effectiveness of systemic chemotherapy in appendix cancer. As a result, treatment recommendations are extrapolated from colon cancer studies, even though there is evidence that these tumors behave differently and are distinct at the molecular level.[13] An experienced medical oncologist should review your specific case for chemotherapy recommendations.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC):

Numerous studies have shown a improved survival with the use of extensive cytoreduction (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC).[7, 8] Cytoreductive surgery is the aggressive surgical removal or destruction of all visible cancer. It is performed through an incision down the midline of the abdomen and may include removal of parts of or the entirety of organs such as: the spleen, gallbladder, liver, peritoneum (lining of the abdominal cavity), uterus, fallopian tubes/ovaries, and bowel. Following CRS, heated chemotherapy is circulated directly into the abdominal cavity to kill any remaining microscopic cancer cells.

CRS/HIPEC is the recommended treatment when the appendix has ruptured and there is evidence of disease in the abdominal cavity (peritoneal spread). Patients who undergo complete CRS, with no residual disease (CC-0/1), immediately followed by HIPEC have the best chance at long-term survival.[8, 9] Today, CRS/HIPEC is widely regarded as the standard of care treatment for all subtypes of advanced appendix cancers.

Cytoreductive surgery and hyperthermic intrathoracic chemotherapy (CRS/HITHOC):

In rare cases, appendix cancer can spread beyond the abdominal cavity into the chest (thoracic) cavity (pleural space), most commonly through direct extension across the diaphragm. Historically, pleural involvement was considered a sign of advanced disease with limited (mostly palliative) treatment options. However, recent studies have demonstrated that select patients may benefit from CRS combined with hyperthermic intrathoracic chemotherapy (HITHOC).[33]

CRS/HITHOC may be performed via an abdominal incision through the diaphragm (if abdominal disease is found to be penetrating through the diaphragm to the chest) or between the ribs (if treating isolated pleural disease). CRS involves removing all visible tumor deposits within the chest cavity and may include resection of portions of the pleura (lining of the lung and chest wall), diaphragm, pericardium, or lung if involved by tumor. Similar to CRS/HIPEC, the goal is to achieve complete removal of all visible disease. Following CRS, heated chemotherapy is circulated throughout the chest cavity to destroy microscopic cancer cells that may remain after surgery, called HITHOC. Similar to HIPEC, HITHOC allows for high concentrations of chemotherapy to be delivered directly to the affected cavity while minimizing systemic exposure and better outcomes are observed with a complete cytoreduction is achieved.

Because pleural spread from appendix cancer is uncommon, evidence is limited to retrospective studies and series from specialized centers. Thus, CRS/HITHOC is generally reserved for carefully selected patients with pleural metastases from appendix cancer who have no or limited disease outside the chest and are healthy enough to undergo a major operation. Nevertheless, in carefully selected patients, CRS/HITHOC may offer meaningful disease control and prolonged survival compared to systemic chemotherapy alone. Outcomes are strongly influenced by tumor biology, disease burden, and completeness of cytoreduction.[34, 35] Evaluation by a multidisciplinary team experienced in appendix cancer and regional chemotherapy techniques is essential when considering this treatment approach.

Pressurized intraperitoneal aerosol chemotherapy (PIPAC)

Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is an emerging palliative treatment option for selected patients with advanced appendix cancer that has spread throughout the abdominal cancer. Unlike CRS/HIPEC, which aim to remove all visible disease, PIPAC is mainly used for patients with tumors that cannot be completely removed surgically or who are not candidates for major surgeries.

PIPAC is performed using minimally invasive laparoscopic surgery, during which, chemotherapy is delivered as a fine mist to the abdominal cavity. The procedure can be repeated multiple times at scheduled intervals. The goal is to stabilize disease, reduce tumor burden, improve symptoms, and maintain quality of life. In some cases, patients initially considered unresectable may experience sufficient disease response to become candidates for CRS. Though data is still limited and the role of PIPAC in appendix cancer continues to be investigated.[36]

At present, PIPAC is considered an investigational or specialized treatment only available at a limited number of centers, commonly through a clinical trial. Patients interested in PIPAC should be evaluated by a specialized multidisciplinary team

Diagnosis and Prognosis

Although the recommended treatment for all advanced appendiceal cancers is CRS/HIPEC, prognosis and outcomes are highly dependent on the type of cancer and extent of disease. Therefore, accurately diagnosing these tumors is especially important for treatment and surveillance decisions. This diagnosis can be challenging as appendix tumors encompass a spectrum of related but distinct entities with overlapping features, and researchers are still working to determine the best classification system [31]. Furthermore, many pathologists are unfamiliar with this rare disease. Do not be surprised if your CRS/HIPEC surgeon requests that your pathology slides be reviewed to confirm or refine the diagnosis.

Carcinoid/Neuroendocrine Tumors

Appendix neuroendocrine tumors (NETs), historically referred to as carcinoid tumors, are commonly located at the tip of the appendix and are usually discovered incidentally at an early stage, without widespread abdominal disease. Although less common than with other gastrointestinal NETs, these tumors can occasionally secrete serotonin and other substances that cause carcinoid syndrome, characterized by diarrhea, skin flushing, and cardiac issues [14].

Treatment and prognosis depends primarily on tumor size and risk features. For an early stage diagnosis, small, localized tumors are often treated with appendectomy alone, while larger tumors or those with high-risk features may warrant a right hemicolectomy. Early-stage appendix NETs have an excellent prognosis, with survival of greater than 30 years.[15]

In the rare event that they have spread beyond the appendix to the peritoneal cavity (<12% of cases), complete surgical resection with or without HIPEC is recommended, with a 5-year survival ranging from 32-78% depending on the lymph node status, tumor grade, and presence of distant metastases[15]. Although the role of cytoreductive surgery is accepted, the role of HIPEC in appendix NETs is not well established and data remains limited. For unresectable disease, somatostatin analogs, such as octreotide, can help manage symptoms of carcinoid syndrome and may slow disease progression in some patients.

DPAM/LGMCP

The most common appendix cancer subtype is low-grade mucinous carcinoma peritonei (LGMCP), which may also be referred to as DPAM or LAMN. This is a non-invasive subtype that is characterized by large mucin pools and few bland epithelial cells. It originates in a low or high-grade appendiceal mucinous neoplasm (LAMN/HAMN) and progresses to LGMCP/DPAM once it has spread beyond the appendix into the abdominal cavity.[3] While the disease can disseminate extensively within the peritoneal cavity, it rarely spreads outside of the abdomen or to lymph nodes and is not known to respond to systemic chemotherapy.[16]

LGMCP is the subtype associated with the longest survival and generally favorable prognosis, particularly when complete cytoreduction is achieved. Following complete CRS/HIPEC, median overall survival exceeds 15 years in many series, and 10-year survival rates are commonly reported in the 75-82% range.[8, 17] Although less common than in other subtypes, recurrence can also occur in up to 30% of patients and median progression-free survival is 7-10 years after complete CRS/HIPEC.[18] Emerging evidence demonstrates that the number of epithelial cells present in the mucin is directly correlated to recurrence and overall survival, with acellular mucin associated with the best outcomes.[19, 20]

Without complete CRS/HIPEC, survival shortens to under 5 years. The role of systemic chemotherapy remains limited. Because these tumors illicit a large immune response, some oral chemotherapy agents with an anti-inflammatory component, such as capecitabine (Xeloda), may be offered in unresectable cases, though data is limited.[21] The role of targeted or inflammatory-modulating strategies remain investigational.[21]

PMCA/HGMCP

High-grade mucinous carcinoma peritonei (HGMCP), historically referred to as PMCA, is an invasive mucinous adenocarcinoma with biologically more aggressive behavior. Prognosis varies based on tumor grade (well, moderately, or poorly differentiated) and the presence of lymph node involvement. Patients with well-differentiated tumors and negative lymph nodes tend to have more favorable survival outcomes compared to those with poorly differentiated or node-positive disease.[3]

Complete CRS/HIPEC represents the primary treatment approach and offers the best chance for long-term disease control for all HGMCP, with median overall survival ranging from 8-10 years.[8, 9, 17] Recurrence is more common compared to LGMCP, occurring in up to 45% of patients around 4-5 years after CRS/HIPEC.[17, 18] Without complete CRS/HIPEC, survival shortens to approximately 2-3 years. Systemic chemotherapy with colon-type regimens (FOLFOX, FOLFIRI) may be recommended for patients with unresectable disease or as “preventative” treatment (adjuvant therapy) after complete CRS/HIPEC in patients who are at high risk for recurrence (i.e. positive lymph nodes or poorly differentiated).[11, 22] However, its benefit after complete CRS/HIPEC remains variable and, with limited evidence, is generally tailored to individual risk factors.

PMCA-S/HGMCP-S

Signet ring cell carcinoma or PMCA-S is a type of high-grade mucinous carcinoma peritonei that is highly invasive and aggressive. The term “signet ring” describes the shape of the cancer cells, in which the nucleus is pushed to the periphery of the cell, creating a characteristic ring-like appearance. This subtype is associated with a higher likelihood for lymph node involvement and distant (extra-abdominal) metastases compared to other mucinous appendix tumors.[3]

The prognosis largely depends on the amount of tumor, measured using the PCI score, and the extent of the disease, including lymph node status and feasibility of achieving complete cytoreduction. Patients with lower tumor burden (lower PCI score) and no extra-abdominal or lymph node metastases tend to have better survival outcomes.

Treatment decisions can vary across centers. Some centers employ a “PCI cutoff” for signet ring cell tumors and will not perform CRS/HIPEC on patients above that cutoff point. However, some studies have shown that long-term survival can be achieved with CRS/HIPEC, even in patients with a lot of disease (high PCI), if all the visible tumors can be removed. After complete CRS/HIPEC, median overall survival is approximately 3 years, which improves to around 6 years in lymph node-negative patients.[17, 23, 24]

Even after complete CRS/HIPEC, recurrence is common, occurring in up to 65% of patients and most commonly within the first few years after CRS/HIPEC [32]. Median progression-free survival after CRS/HIPEC is approximately 2 years, reflecting the aggressive nature of this subtype.

Most patients with signet ring cells will be recommended to undergo systemic chemotherapy, before and/or after CRS/HIPEC.[18] Treatment regimens typically are extrapolated from colorectal cancer guidelines.

PMCA-G/HGMCP-G

Goblet cell carcinomas or PMCA-G is one of the rarest types of high-grade mucinous carcinoma peritonei that is characterized by mixed glandular (often signet ring cell) and neuroendocrine features. Because of its mixed histologic features and low incidence, it is commonly misdiagnosed as a less aggressive neuroendocrine/carcinoid tumor.[25] However, biologically it behaves more like a carcinoma than a typical NET.

Prognosis is influenced by the stage of disease at diagnosis, tumor grade, and the presence of adverse features, such as signet ring cells.[26] Once this cancer has spread beyond the appendix, the role of CRS/HIPEC is controversial. However, no other available treatment options can provide the same chance of long-term survival. Some single-center studies have reported a median overall survival of over 4 years, which includes some long-term survivors beyond 5 years, and a median progression-free survival of around 2 years.[18, 27, 37]

Colon-type systemic chemotherapy is commonly used in advanced or unresectable disease, though responses are variable and evidence is limited.

Disease Recurrence

There are limited treatment options beyond CRS/HIPEC for appendix cancers. Thus, if possible, a second repeated (iterative) CRS/HIPEC should be considered for disease recurrence as it has shown the highest survival benefit.[28, 29] The possibility of undergoing an additional CRS/HIPEC procedure will depend on the location of disease recurrence, extent of disease, and physical fitness. Luckily, the majority of recurrences occur within the abdominal cavity.[18] Some localized abdominal recurrences may only require surgery without HIPEC.

If you are not a candidate for a repeated CRS/HIPEC, systemic chemotherapy, PIPAC, and/or a clinical trial should be explored. Depending on the pathology and tumor aggressiveness, your clinicians may recommend observation and reserving systemic therapies once symptoms occur. [32]

Research

Clinical Trials

Clinical trials may provide access to promising new treatment options. To view the latest open clinical trials, visit our partner organization ACPMP's Clinical Trials webpage, where you can find regularly updated information on currently recruiting studies.

Clinical trials for appendix cancer are listed on www.ClinicalTrials.gov. Your medical and surgical oncologists can also help you review clinical trial options and recommendations.

There are few clinical trials for appendix cancers because of challenges with recruiting and funding. However, some clinical trials for colon cancers or certain genetic mutations also include appendix cancer patients and may be open to additional alternative treatment options.

A full list of all active clinical trials and their current recruitment status is registered on www.ClinicalTrials.gov.

Other Resources

More Information on Appendix Cancer

Patient Support

PMP Pals

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Appendix Cancer Connection

appendix-cancer.org

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References

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Appendix Cancer

What is Appendix Cancer?

What are the common symptoms of appendix cancer?

How is appendix cancer (appendiceal cancer) treated?