Tumors of the Peritoneum

Pathology

The peritoneum is a thin serous membrane that lines the inner surface of the abdominal cavity and envelops many abdominal organs, including the stomach, intestines, liver, and uterus. It serves to protect and support these organs, while also facilitating their smooth movement within the abdomen. Tumors of the peritoneum comprise a rare group of neoplasms that may arise as primary lesions or develop secondary to metastatic disease.

Types

Primary forms arise directly in the peritoneum and are less common. Among these, the most frequent are:

  • Peritoneal mesothelioma, which develops from mesothelial cells in the peritoneum;
  • Primary carcinoma of the peritoneum, a malignancy with histologic features similar to serous ovarian carcinoma, but developing independently of the ovaries.

Far more common are secondary forms, also referred to as peritoneal carcinosis, which result from the dissemination of malignant cells from primary tumors in other abdominal organs. The most frequent primary sites include the colorectum, ovaries, and stomach, with less common origins in the pancreas or liver.

Previously regarded as a terminal condition manageable only with palliative care, peritoneal carcinosis is now understood as a locoregional disease that, in carefully selected patients, may be amenable to multimodal treatment with curative intent. In this context, cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has emerged as one of the most innovative and promising therapeutic strategies.

The Numbers in Italy

According to data from the AIRTUM (Italian Association of Cancer Registries), primary tumors of the peritoneum are rare, representing less than 1% of all malignancies, whereas secondary involvement of the peritoneum is considerably more common, particularly in patients with advanced-stage abdominal cancers.

Risk Factors

Peritoneal tumors, whether primary or secondary, may be associated with a range of factors that increase the likelihood of involvement of the peritoneal membrane. The main recognized risk factors are summarized below:

  • Previous advanced abdominal malignancy: In particular, colorectal cancer, stomach cancer, ovarian cancer, or appendix cancer with a propensity for local spread are associated with a higher risk of developing peritoneal carcinosis.
  • Asbestos exposure: In cases of peritoneal mesothelioma, occupational or environmental exposure to asbestos is the principal known risk factor for this primary malignancy of the peritoneal lining.
  • Advanced age and female sex: Certain primary peritoneal tumors, particularly those histologically similar to ovarian carcinoma, occur more frequently in women over 60 years of age.
  • Genetic predisposition and family history: Germline mutations (e.g., in BRCA1, BRCA2, or genes associated with Lynch syndrome), as well as a family history of ovarian, fallopian tube, or peritoneal cancers, are associated with an increased risk of peritoneal neoplasms.
  • Obesity and hormonal factors: For some primary peritoneal tumors, obesity and a history of hormone replacement therapy have also been identified as associated risk factors.

Symptoms

In the early stages, tumors of the peritoneum may not show obvious signs of disease. In fact, symptoms often appear at a more advanced stage, when the tumor has spread within the peritoneal cavity.

The most common clinical manifestations include:

  • Ascites, defined as the accumulation of fluid within the abdominal cavity, leading to abdominal distension, bloating, and a sensation of tightness;
  • Progressive increase in abdominal circumference, not attributable to changes in diet or body weight;
  • Diffuse or persistent abdominal pain, often described as a feeling of heaviness or constant discomfort;
  • Alterations in bowel habits (alvus), such as constipation, diarrhea, or irregular bowel movements;
  • Dyspnea, resulting from diaphragmatic compression caused by the accumulation of ascitic fluid;
  • Anorexia and early satiety, related to compression of intra-abdominal organs.

Because these symptoms may also occur in a range of benign conditions, they should not be a cause for undue alarm. However, it is important to seek prompt medical evaluation if they are persistent or progressively worsening. A thorough clinical assessment enables the physician to determine whether further diagnostic investigations are warranted and, if a diagnosis is confirmed, to initiate an individualized treatment plan.

Diagnosis and Examination

The diagnosis of peritoneal tumors requires a careful, multidisciplinary approach, as clinical manifestations are often non-specific and may overlap with those of other abdominal conditions. The primary objectives are to detect the presence of disease at an early stage, evaluate its extent, and determine the most appropriate therapeutic strategy.

Level 1 examinations

    • The diagnostic work-up typically begins with a specialist clinical evaluation followed by non-invasive imaging studies, which are essential for detecting the presence of ascites or abdominal masses:Abdominal ultrasound

    • CT scan of abdomen and pelvis with contrast medium

    • Magnetic resonance imaging (MRI)

    • PET/TC

Second level examinations

When radiological examinations suggest the presence of peritoneal disease, it is necessary to obtain histological confirmation, that is, a microscopic diagnosis of the tumor cells.
This is done through:

    • diagnostic paracentesis

    • percutaneous or laparoscopic biopsy

Diagnostic laparoscopy

In many cases, diagnostic laparoscopy is a key step.
This is a minimally invasive procedure that allows direct observation of the peritoneal cavity, evaluation of the extent of disease, and taking samples for histologic examination. This examination also helps determine the resectability of the tumor, that is, whether the disease can be treated surgically.

Multidisciplinary Assessment

All diagnostic findings are reviewed within the Interdisciplinary Care Group (GIC) or Multidisciplinary Team (MDT). This multidisciplinary approach enables the development of a personalized treatment strategy, taking into account the biological characteristics of the tumor, the extent of disease, and the patient’s overall clinical condition.

Therapies

Once the diagnosis has been confirmed, the multidisciplinary team evaluates several factors in order to define an individualized treatment plan. Alongside the tumor type, size, and extent of disease spread, patient-related factors such as age, overall health status, and medical history are also taken into account. The proposed therapeutic strategy is then discussed with the patient, including alternative options when comparable efficacy is available.


For selected patients with particularly aggressive disease in whom standard treatments have not been effective, access to experimental therapies within clinical trials conducted by the Institute may also be considered. When deemed appropriate by the multidisciplinary team, this option is presented and carefully explained to the patient, with whom a shared decision-making process is undertaken.
Surgery

Surgery

The success of treatment for peritoneal carcinosis depends on careful patient selection, advanced surgical expertise, and appropriate postoperative management. These factors can be reliably ensured only in specialized referral centers dedicated to the treatment of this disease, such as our Institute.

Peritoneal carcinosis

Over the past 20 years, the treatment of peritoneal carcinosis has made important advances through the development of innovative surgical techniques and therapies. Today, this condition-once considered a terminal phase of the disease-can be addressed in a targeted manner and, in some cases, with curative intent.

The most effective and internationally recognized approach is the combined approach, which combines:

  • cytoreductive surgery, aimed at complete or nearly complete removal of visible disease;

  • intraperitoneal chemohyperthermia (HIPEC), a procedure that allows direct treatment of the abdominal cavity with heated chemotherapy drugs.

Cytoreductive surgery

The goal of surgery is to remove all macroscopic tumor implants from the peritoneum.
Because chemotherapy drugs penetrate only a few millimeters into the tissue, physical removal of the tumor is a critical step.

The intervention may include:

  • The removal of the primary tumor and nearby lymph nodes;

  • removal of organs involved in the disease, such as spleen, gallbladder, portions of the stomach or intestines, uterus and adnexa, depending on the spread.

When the disease affects the inner lining of the abdomen, peritonectomy, or partial or total removal of the peritoneum (pelvic, lateral, central, or diaphragmatic), is performed according to now standardized and internationally recognized techniques.
This surgery is performed only when a surgical field free of visible disease can be obtained.

Intraperitoneal chemoperthermia (HIPEC)

After the surgical phase is finished, HIPEC is performed in the same operating session.
During this procedure, the abdominal cavity is “washed” for about 60-90 minutes with a solution of chemotherapy drugs heated to 41-42°C.

Heat enhances the effectiveness of drugs and acts directly on residual cancer cells, damaging their internal structures and reducing the likelihood of recurrence.
Intraperitoneal administration also allows for very high drug concentrations (up to 1000 times higher than in blood) without increasing systemic toxicity.

HIPEC is particularly indicated for tumors that tend to remain confined to the abdominal cavity, such as ovarian, appendicular, and peritoneal tumors.

Pseudomyxoma Peritonei (PMP)

Pseudomyxoma Peritonei (PMP) is a rare form of mucinous neoplasm that originates in most cases from theappendix and, more rarely, from other organs such as the ovary, colon, or small intestine. It is characterized by the production and accumulation of mucin (mucus) within the abdominal cavity, which can result in a progressive increase in the volume of the abdomen and, in advanced cases, alterations in the function of internal organs.

In the past, the treatment of PMP relied on repeated debulking surgeries (i.e., partial removal of mucinous material and tumor masses), which unfortunately offered limited results: recurrences were very frequent and long-term survival was low, with 5-year survival rates around 6% and perioperative mortality of about 2.7%.

Thanks to advances in research and surgical techniques, today the combination of cytoreductive surgery and intraperitoneal chemohyperthermia (HIPEC) is the gold standard of treatment for this condition.
This approach, introduced and developed by U.S. surgeon Paul Sugarbaker, yields significantly better results: in specialized centers, 10-year survival rates can be as high as80 percent in selected cases.

Treatment of PMP requires a high degree of multidisciplinary expertise and must be performed in referral centers with the necessary skills and technologies to ensure maximum effectiveness and patient safety.

Peritoneal mesothelioma

Diffuse malignant peritoneal mesothelioma (DMPM) is a rare form of cancer that originates from the cells of the mesothelium, the thin lining that internally borders the abdominal cavity (peritoneum).
It is a disease mainly related to exposure to asbestos (asbestos) and, in recent years, its incidence has been steadily increasing, parallel to pleural mesothelioma.

DMPM is generally an unresponsive neoplasm to traditional systemic chemotherapy. However, in some cases, this type of treatment can help reduce the volume of disease, making the patient later a candidate for more radical surgery.

As early as 2006, the Consensus Conference of the Peritoneal Surface Oncology Group International (PSOGI) indicated the combination of cytoreductive surgery (CRS) and intraperitoneal chemoperthermia (HIPEC), possibly combined with systemic chemotherapy, as the standard of care for peritoneal mesothelioma.

The clinical progress achieved with this approach is significant: while the median survival with systemic chemotherapy alone was about 12 months, with combined CRS + HIPEC + systemic chemotherapy treatment, the results have more than quadrupled, reaching median survivals of more than 50 months in specialized centers.

Peritoneal carcinosis of colorectal origin

Peritoneal carcinosis of colorectal origin is a condition that can arise as an evolution of colon or rectal cancer, when cancer cells spread within the peritoneal cavity.

Thanks to new systemic treatment regimens and the possibility of combination treatments, the median survival of patients with this form is now significantly improved, reaching about 24 months. These data confirm that peritoneal spread represents a biologically distinct entity compared with other forms of colorectal cancer metastasis.

In carefully selected patients, the combination of cytoreductive surgery (CRS) and intraperitoneal chemohyperthermia (HIPEC) yielded even more encouraging results: a median survival of nearly 30 months and a disease-free interval of more than one year.

In light of this evidence, the Italian Association of Medical Oncology (AIOM) has included, already in the 2016 guidelines, the possibility of employing the combined CRS + HIPEC approach in patients with isolated peritoneal carcinosis, provided they are treated in specialized high-volume centers, with teams experienced in the management of this complex pathology.

Peritoneal carcinosis of ovarian origin

Epithelial ovarian carcinoma represents the most frequent and aggressive form among gynecologic malignancies. It is often referred to as a “silent tumor,” as in many cases it manifests with few initial symptoms and is diagnosed at an advanced stage (III or IV), when the disease has already spread to the peritoneum.

The standard treatment of advanced ovarian cancer is based on two basic pillars:

  • cytoreductive surgery, which aims to remove as much visible disease as possible;

  • systemic chemotherapy, to act on residual cancer cells.

The tendency of ovarian carcinoma to spread primarily in the peritoneal cavity has made this disease an ideal model for the use of locoregional treatments that can act directly on the site of disease.

In this context, the combination of cytoreductive surgery (CRS) and intraperitoneal chemohyperthermia (HIPEC) has shown promising results. Initially used mainly in cases of relapse, this strategy is now considered effective even at the time of initial diagnosis, as shown in recent international studies (Van Driel et al., N Engl J Med, 2018).

The combined approach allows, in selected patients treated in highly specialized centers, to significantly improve both overall survival and quality of life, offering new therapeutic perspectives even in the most advanced stages of the disease.

Chemotherapy

The treatment of peritoneal carcinosis is increasingly based on a multimodal approach that combines different therapeutic strategies to optimize patient outcomes.

Within this framework, systemic chemotherapy may be administered:

  • Before surgery (neoadjuvant setting), to reduce tumor burden and increase the likelihood of achieving complete cytoreductive surgery;
  • After surgery (adjuvant setting), to reduce the risk of recurrence and limit disease dissemination beyond the peritoneal cavity.

In recent years, the development of more targeted and effective agents has contributed to improved treatment outcomes and has enabled a more personalized therapeutic approach. However, standardized protocols regarding the number of cycles, timing, and drug combinations are still lacking, as management must be tailored to the individual patient.

For this reason, it is essential that each patient be managed within an Interdisciplinary Care Group (ICG) dedicated to this disease. This multidisciplinary team, composed of oncologists, surgeons, radiation oncologists, and other specialists, collectively evaluates the clinical situation, defines an individualized treatment plan, and accompanies the patient throughout the entire therapeutic pathway.

Supportive Therapies

Patients with peritoneal carcinosis receive comprehensive support throughout their diagnostic and therapeutic journey from specialists in palliative care, nutrition, and psycho-oncology.

Ongoing Support

At the Candiolo Cancer Institute, physicians and nurses within the multidisciplinary team are available to provide patients with comprehensive support in managing the various side effects that may arise during treatment.

Direct line to specialists

The cancer patient is often a fragile patient, who needs help and support in his or her disease journey: when he or she experiences an ailment, whether related to the disease or a side effect of therapy, he or she should be able to receive a specialist’s opinion quickly, through a “fast track.”

For this reason, at the Candiolo Institute there is an assistance service every day, Monday through Friday from 8 a.m. to 5 p.m.: just call the secretary of the Oncology Day Hospital (011.993.3775 ) reporting the need for an urgent consultation, and the patient is quickly contacted by his or her specialist doctor.

Continuing care and palliative care

At the Candiolo Institute, specialists from different disciplines are available at Candiolo for patients who need or request them to offer:

    • nutritional support

    • psychological support

    • physiotherapy

    • dressing of venous access devices

    • pain therapy

    • Management of other coexisting conditions.

Social work

Social work

The Social Service Department of the Candiolo Institute conducts information and orientation interviews for patients and their families on how to access services in the area and how to obtain welfare and social security benefits provided by law (disability, benefits for aids and prostheses, work leave, etc.).

The service operates on Wednesdays and Fridays from 9 a.m. to 1 p.m. – Phone: 011.993.30

Follow up

With the conclusion of the course of treatment, the follow-up period begins during which, through a series of examinations and visits, the side effects of the therapies performed, their effectiveness, and the patient’s functional recovery are monitored.

Follow-up examinations are especially important to intercept any recurrences early so that appropriate therapy can be intervened. For the patient, they are also a valuable opportunity for dialogue with their medical specialist.

It is the same specialist physician who schedules follow-up visits, in which the patient’s health condition is assessed and the required test reports are viewed.

Checkups are performed at scheduled intervals for the duration of 5-10 years and include examination, blood chemistry tests, CEA marker assay, Ca19.9, and CT chest and abdomen with contrast medium.

They have a shorter cadence at first (three to six months), then gradually thin out over time (once a year). The frequency and type of examinations provided depend on the stage of the tumor and the treatments given.

Interdisciplinary Group

Every cancer requires, at all stages of its management, a multidisciplinary approach, which at the Candiolo Cancer Institute is ensured by a team of specialists from the various clinical and surgical departments. This team is referred to as the GIC (Interdisciplinary Care Group).

The GIC is responsible for accompanying each patient throughout the entire diagnostic and therapeutic pathway, including the prescription and scheduling of examinations, as well as communication with the patient and their family members. It defines and shares a personalized care plan for each individual, based not only on the type and stage of the tumor but also on patient-specific characteristics. The objective is to achieve the best possible oncological and functional outcomes while maintaining an optimal quality of life.

The Group also collaborates closely with the Institute’s research teams to ensure timely access to the most recent innovations in screening, diagnosis, and treatment arising from ongoing scientific research.

Clinical Divisions

The diagnostic and therapeutic pathway for peritoneal tumors at the Candiolo Cancer Institute involves several clinical divisions, including:

Why Choose Us

At the Candiolo Cancer Institute, each patient with pancreatic cancer is managed through a highly specialized approach, supported by the coordinated work of a dedicated Interdisciplinary Care Group (GIC).

Clinical experience and tailored approach

Due to the high number of cases treated each year, the Candiolo Institute is a national reference for taking pancreatic cancers. Our experience enables us to deal with even the most complex situations, always with a personalized approach built on the clinical and personal profile of each patient.

Imaging technologies and advanced diagnostics

Establishing the treatment plan always starts with an accurate and timely diagnosis. Patients have access to state-of-the-art imaging technologies that allow accurate assessment of the extent of the disease.

In addition, the Institute offers advanced and sophisticated laboratory investigations, including molecular and genomic analyses, which are critical for identifying biological features of cancer and guiding therapeutic decisions

Minimally invasive surgical techniques and multidisciplinarity

When indicated, surgery is performed with minimally invasive techniques (laparoscopic or thoracoscopic), which reduce operative trauma, promote faster recovery, and improve postoperative quality of life. Every treatment choice is defined within the GIC, ensuring a consistent and integrated approach.

Clinical research and access to trials

As an IRCCS, the Candiolo Institute combines clinical practice with a strong vocation for scientific research. Patients can be evaluated forinclusion in active clinical trials, which represent a real chance to access innovative therapies not yet available in standard practice. Collaboration between care and research is a distinctive value that translates into concrete opportunities for the patient.

Care and support every step of the way

The Interdisciplinary Care Group takes care of the person at every stage: from diagnosis to treatment to follow-up, with attention to nutritional support, psychological health, and reintegration into daily life. The organization of checkups, visits and treatment is designed to ensure continuity and serenity, always valuing the human dimension of care.