Peritoneal cancer, also known as primary peritoneal cancer, is a relatively rare cancer affecting the peritoneum. The peritoneum is an extremely permeable membrane that borders the inside of the abdomen and protects the organs within, including the digestive tract. Peritoneal cancer develops in the peritoneum’s cells, but it may also spread from other cancers that started in neighbouring organs, such as the ovaries, fallopian tubes, or colon.
It is considered primary cancer when peritoneal cancer develops from the peritoneal cells. However, distinguishing between primary peritoneal and advanced ovarian cancers is often difficult because they have similar symptoms and treatments. These include a family history of ovarian, breast, or colorectal cancer, a personal history of hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome, and specific genetic mutations, such as BRCA1 and BRCA2 mutations.
Epidemiology
Peritoneal cancer is rare, and its prevalence and incidence rates vary across different populations.
Incidence: The incidence of peritoneal cancer is relatively low compared to other types of cancer. Peritoneal cancer is estimated to account for approximately 10% of all primary cancers originating in the female reproductive system.
Age and Gender: Men and women may both get peritoneal cancer, although women are more likely to have it. Most cases are found in postmenopausal women, typically between 50 and 70. However, peritoneal cancer can also occur in younger women and, rarely, men.
Risk Factors: Several risk factors have been associated with an increased likelihood of developing peritoneal cancer. These include a family history of ovarian, breast, or colorectal cancer, a history of hereditary nonpolyposis colorectal cancer (HNPCC), and genetic mutations such as BRCA1 and BRCA2. Other factors, such as increasing age, obesity, and a history of endometriosis, may also contribute to the development of peritoneal cancer.
Geographic Variations: Studies have shown some geographic variations in the incidence of peritoneal cancer. For instance, higher rates have been reported in developed countries compared to developing nations.
Survival Rates: Survival rates for peritoneal cancer depend on various factors, including the cancer stage at diagnosis, the individual’s overall health, and the response to treatment. Unfortunately, peritoneal cancer is often diagnosed at an advanced stage, negatively impacting survival outcomes. However, with advancements in treatment approaches, including cytoreductive surgery and heated intraperitoneal chemotherapy (HIPEC), survival rates have improved for selected patients.
Anatomy
Pathophysiology
PATHOPHYSIOLOGY
Genetic and Molecular Alterations: Like other types of cancer, peritoneal cancer involves genetic and molecular alterations in the affected cells. These alterations can disrupt the normal regulation of cell growth, division, and death. Mutations in genes such as TP53, BRCA1, BRCA2, and KRAS have been associated with the development of peritoneal cancer.
Metastasis: Peritoneal cancer can occur through direct extension from neighboring organs or metastasis from distant sites. Cancer cells can invade the peritoneum by breaking through the basement membrane and infiltrating the surrounding tissues. The cancer cells can then spread within the peritoneal fluid, allowing them to reach different peritoneum areas and form new tumors.
Peritoneal Fluid and Microenvironment: Peritoneal fluid, a natural lubricant in the abdominal cavity, plays a role in the pathophysiology of peritoneal cancer. The fluid contains various molecules, growth factors, and immune cells that can influence the growth and spread of cancer cells. Changes in the composition and characteristics of the peritoneal fluid may promote tumor growth and metastasis.
Inflammation and Angiogenesis: Chronic inflammation within the peritoneum can contribute to the development and progression of peritoneal cancer. Inflammatory processes can release cytokines, growth factors, and chemokines that promote cell proliferation, angiogenesis (formation of new blood vessels), and tissue remodeling. These processes create a supportive environment for tumor growth.
Ascites Formation: Peritoneal cancer can cause the accumulation of fluid within the peritoneal cavity, leading to a condition called ascites. Ascites can develop due to increased vascular permeability, lymphatic obstruction, or impaired absorption of peritoneal fluid. The presence of ascites can further promote the spread of cancer cells within the peritoneum.
Etiology
Family History: A family history of certain cancers, such as ovarian, breast, or colorectal, can increase the risk of developing peritoneal cancer. This suggests that there may be genetic factors involved in its development. Mutations in genes, such as BRCA1 and BRCA2, are associated with an increased risk of breast and ovarian cancers.
Hereditary Conditions: Certain hereditary conditions can predispose individuals to peritoneal cancer. For example, individuals with hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, have an increased risk of developing various types of cancer, including peritoneal cancer.
Genetic Mutations: Specific genetic mutations have been associated with an increased risk of peritoneal cancer. Mutations in genes involved in DNA repair mechanisms, such as BRCA1 and BRCA2, can increase the risk of peritoneal cancer. These mutations can be inherited or occur spontaneously.
Gender: Peritoneal cancer is more commonly diagnosed in women than in men. This may be partly because peritoneal cancer is often associated with ovarian cancer, and women have a higher incidence of ovarian cancer.
Age: The risk of peritoneal cancer increases with age. It is more commonly diagnosed in postmenopausal women, typically between 50 and 70.
Endometriosis: Endometriosis, a condition in which the tissue that usually lines the uterus grows outside the uterus, has been associated with an increased risk of developing peritoneal cancer. The exact relationship between endometriosis and peritoneal cancer is not fully understood, but chronic inflammation and hormonal factors may play a role.
Genetics
Prognostic Factors
Stage of Cancer: At diagnosis, the peritoneal stage is an important prognostic factor. The extent of cancer spread, including the size of the tumor, involvement of nearby organs, and presence of distant metastasis, influences the prognosis. Peritoneal cancer is typically staged using the FIGO (International Federation of Gynecology and Obstetrics) staging system.
Histopathological Features: The histopathological characteristics of the tumor can provide valuable prognostic information. Factors such as the histological type of cancer (e.g., serous, mucinous, endometrioid), tumor grade (degree of cellular abnormality and aggressiveness), and presence of specific molecular markers can impact prognosis. For example, a higher tumor grade is generally associated with a worse prognosis.
Completeness of Surgical Resection: The extent to which the tumor can be surgically removed, also known as the completeness of surgical resection or cytoreduction, is a crucial prognostic factor for peritoneal cancer. Achieving complete cytoreduction (removing all visible tumors) improves survival outcomes. This often involves a complex surgical procedure called cytoreductive surgery, sometimes combined with heated intraperitoneal chemotherapy (HIPEC).
Performance Status and Overall, Health: An individual’s overall health and functional status, as assessed by factors like performance status and comorbidities, can impact prognosis. Better overall health and performance status are generally associated with improved outcomes.
Response to Treatment: The response to treatment, including chemotherapy and targeted therapies, is an important prognostic factor. Individuals who have an excellent response to treatment and achieve disease control are more likely to have a favorable prognosis.
Genetic and Molecular Markers: Certain genetic and molecular markers can provide prognostic information for peritoneal cancer. For example, genetic mutations like BRCA1 and BRCA2 may impact prognosis and guide treatment decisions.
Clinical History
CLINICAL HISTORY
Age Group: Peritoneal cancer can occur in individuals of various age groups, but it is more commonly diagnosed in postmenopausal women, typically between 50 and 70. However, it can also affect younger women and, rarely, men. The symptoms and presentation may be similar across age groups, but the possibility of peritoneal cancer should be considered in postmenopausal women presenting with abdominal symptoms.
Associated Comorbidities or Activity Level: Comorbidities or specific activities can influence the clinical presentation of peritoneal cancer. For example, individuals with a history of endometriosis may be at higher risk for developing peritoneal cancer, and the presence of symptoms related to endometriosis along with abdominal pain or discomfort should raise suspicion. Additionally, individuals with obesity or other chronic conditions may have different symptom profiles or presentations due to overlapping symptoms or complications.
Acuity of Presentation: Peritoneal cancer can have a variable acuity of presentation. In some cases, symptoms may develop gradually and progress over time, while in other cases, symptoms may appear suddenly and be more severe. Acute presentations may occur when there is a rapid accumulation of fluid within the peritoneal cavity (ascites) or if there is bowel obstruction due to tumor growth.
Common clinical manifestations of peritoneal cancer may include:
Abdominal pain or discomfort: One of the most common symptoms can range from mild to severe. The pain may be constant or intermittent and may be localized or diffuse.
Abdominal distension or bloating: Ascites or tumor growth can increase abdominal size or bloating sensation.
Changes in bowel habits: This can include constipation, diarrhea, or alterations in bowel movements.
Loss of appetite and weight loss: Individuals with peritoneal cancer may experience a decreased appetite and unintentional weight loss.
Nausea and vomiting: These symptoms may occur due to obstruction or irritation of the gastrointestinal tract.
Fatigue and weakness: Generalized fatigue and weakness can be associated with advanced stages of peritoneal cancer or because of the systemic effects of the disease.
Physical Examination
Abdominal Inspection: The healthcare provider may visually inspect the abdomen for any visible abnormalities, such as distension, asymmetry, or prominent veins. They may also observe for any signs of surgical scars or evidence of previous interventions.
Abdominal Palpation: The provider will use their hands to feel and palpate the abdomen, assessing for any areas of tenderness, masses, or organ enlargement. They may systematically palpate different abdomen regions, including the upper quadrants, lower quadrants, and pelvis.
Fluid Wave Test: The presence of ascites (fluid accumulation in the peritoneal cavity) may be assessed using the fluid wave test. The provider will tap one side of the abdomen while simultaneously palpating the opposite side. A positive fluid wave test occurs when the provider can feel a fluid wave, suggesting the presence of ascites.
Pelvic Examination: In women, a pelvic examination may be performed to assess the reproductive organs. The provider will visually inspect the external genitalia and then perform a manual examination of the uterus, ovaries, and surrounding structures, feeling for any masses, tenderness, or abnormal findings.
Lymph Node Examination: The healthcare provider may also assess for enlarged lymph nodes in the abdomen, groin, or other regions. Palpation of lymph nodes may help determine the presence of metastasis or spread of cancer.
Auscultation: The provider may use a stethoscope to listen for bowel sounds, which can provide information about the function and movement of the intestines. Absent or abnormal bowel sounds may suggest bowel obstruction or other gastrointestinal issues.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
DIFFERENTIAL DIAGNOSIS
Ovarian Cancer: Ovarian cancer can cause abdominal pain, bloating, and distension, like peritoneal cancer. The two conditions can coexist or be difficult to differentiate as they may originate from the same tissue.
Gastrointestinal Cancers: Cancers affecting the gastrointestinal tract, such as colorectal cancer, gastric cancer, or pancreatic cancer, can manifest with abdominal pain, weight loss, changes in bowel habits, and abdominal distension.
Endometriosis: Endometriosis is a condition in which the tissue that usually lines the uterus grows outside the uterus. It can cause chronic pelvic pain, menstrual irregularities, and pelvic masses, which may mimic peritoneal cancer. Endometriosis and peritoneal cancer can coexist or share similar symptoms.
Inflammatory Bowel Disease (IBD): Conditions such as Crohn’s disease or ulcerative colitis, collectively known as inflammatory bowel disease, can cause abdominal pain, diarrhea, weight loss, and inflammation within the abdominal cavity.
Appendicitis: Acute appendicitis is characterized by sudden-onset abdominal pain, often localized to the right lower quadrant. While typically acute and self-limited, a perforated appendix or complications may present with more chronic symptoms and could be mistaken for peritoneal cancer.
Abdominal Tuberculosis: Tuberculosis (TB) can affect the peritoneum, leading to peritoneal tuberculosis. It can cause chronic abdominal pain, weight loss, and ascites, which can mimic peritoneal cancer.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors that can occur in the gastrointestinal tract, including the stomach and intestines. They can present with abdominal pain, bleeding, and abdominal masses, like peritoneal cancer.
Ascites of Non-Malignant Origin: Conditions such as liver cirrhosis, congestive heart failure, or infections can lead to fluid accumulation within the peritoneal cavity (ascites), which can cause abdominal distension and discomfort.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
TREATMENT PARADIGM
The management of peritoneal cancer can be divided into several phases, including initial treatment, consolidation or maintenance therapy, and palliative care. Here is a general overview:
Modification of Environment:
Surgical Intervention: Cytoreductive surgery (CRS) is a critical component of treatment for peritoneal cancer. It involves the surgical removal of visible tumors within the peritoneal cavity. The goal is to achieve complete cytoreduction, removing all visible tumor nodules to the extent feasible. CRS is often performed in combination with other procedures.
Hyperthermic Intraperitoneal Chemotherapy (HIPEC): HIPEC is performed during cytoreductive surgery. After removing visible tumors, heated chemotherapy drugs are administered directly into the peritoneal cavity. The high temperature improves drug penetration and enhances its anticancer effects.
Peritoneal Access Devices: Peritoneal access devices, such as ports or catheters, may be implanted during surgery to allow repeated intraperitoneal chemotherapy administrations.
Administration of Pharmaceutical Agents:
Chemotherapy: Systemic chemotherapy may be used in addition to cytoreductive surgery and HIPEC. It involves administering anticancer drugs through intravenous infusion to target cancer cells throughout the body.
Targeted Therapies: Some peritoneal cancers may have specific molecular characteristics or genetic mutations that can be targeted with specialized drugs. These targeted therapies inhibit specific signaling pathways or molecular targets in cancer growth.
Intervention with Procedures:
Paracentesis: Paracentesis is a procedure in which a needle or catheter is inserted into the peritoneal cavity to drain excessive fluid (ascites). It can relieve symptoms such as abdominal distension and discomfort caused by ascites.
Pleurodesis: In cases where peritoneal cancer has spread to the pleural cavity, pleurodesis may be performed. It involves the introduction of a sclerosing agent into the pleural space to create adhesions and prevent fluid accumulation.
Phases of Management:
Initial Treatment: This phase involves the primary treatment approach, which often includes cytoreductive surgery, HIPEC, and systemic chemotherapy. The goal is to achieve optimal tumor reduction and disease control.
Consolidation/Maintenance Therapy: After initial treatment, consolidation or maintenance therapy may be recommended to control the disease further. This can involve additional cycles of systemic chemotherapy or intraperitoneal chemotherapy.
Palliative Care: In cases where curative treatment is not feasible or when cancer has advanced, palliative care aims to manage symptoms, provide pain relief, and improve the quality of life. Palliative care can involve pain management, nutritional and psychological support for the patient and their caregivers.
Continue the treatment till the disease progression or unacceptable toxicity occurs
Note:
Applicable for maintenance therapy, I.e., fallopian tube, recurrent epithelial ovarian, and peritoneal cancer who are in complete/partial response to platinum-based chemotherapy
Future Trends
Media Gallary
References
Peritoneal cancer:ncbi.nlm.nih.gov
Peritoneal Metastasis:ncbi.nlm.nih.gov
Primary peritoneal serous:ncbi.nlm.nih.gov
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Peritoneal cancer, also known as primary peritoneal cancer, is a relatively rare cancer affecting the peritoneum. The peritoneum is an extremely permeable membrane that borders the inside of the abdomen and protects the organs within, including the digestive tract. Peritoneal cancer develops in the peritoneum’s cells, but it may also spread from other cancers that started in neighbouring organs, such as the ovaries, fallopian tubes, or colon.
It is considered primary cancer when peritoneal cancer develops from the peritoneal cells. However, distinguishing between primary peritoneal and advanced ovarian cancers is often difficult because they have similar symptoms and treatments. These include a family history of ovarian, breast, or colorectal cancer, a personal history of hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome, and specific genetic mutations, such as BRCA1 and BRCA2 mutations.
Peritoneal cancer is rare, and its prevalence and incidence rates vary across different populations.
Incidence: The incidence of peritoneal cancer is relatively low compared to other types of cancer. Peritoneal cancer is estimated to account for approximately 10% of all primary cancers originating in the female reproductive system.
Age and Gender: Men and women may both get peritoneal cancer, although women are more likely to have it. Most cases are found in postmenopausal women, typically between 50 and 70. However, peritoneal cancer can also occur in younger women and, rarely, men.
Risk Factors: Several risk factors have been associated with an increased likelihood of developing peritoneal cancer. These include a family history of ovarian, breast, or colorectal cancer, a history of hereditary nonpolyposis colorectal cancer (HNPCC), and genetic mutations such as BRCA1 and BRCA2. Other factors, such as increasing age, obesity, and a history of endometriosis, may also contribute to the development of peritoneal cancer.
Geographic Variations: Studies have shown some geographic variations in the incidence of peritoneal cancer. For instance, higher rates have been reported in developed countries compared to developing nations.
Survival Rates: Survival rates for peritoneal cancer depend on various factors, including the cancer stage at diagnosis, the individual’s overall health, and the response to treatment. Unfortunately, peritoneal cancer is often diagnosed at an advanced stage, negatively impacting survival outcomes. However, with advancements in treatment approaches, including cytoreductive surgery and heated intraperitoneal chemotherapy (HIPEC), survival rates have improved for selected patients.
PATHOPHYSIOLOGY
Genetic and Molecular Alterations: Like other types of cancer, peritoneal cancer involves genetic and molecular alterations in the affected cells. These alterations can disrupt the normal regulation of cell growth, division, and death. Mutations in genes such as TP53, BRCA1, BRCA2, and KRAS have been associated with the development of peritoneal cancer.
Metastasis: Peritoneal cancer can occur through direct extension from neighboring organs or metastasis from distant sites. Cancer cells can invade the peritoneum by breaking through the basement membrane and infiltrating the surrounding tissues. The cancer cells can then spread within the peritoneal fluid, allowing them to reach different peritoneum areas and form new tumors.
Peritoneal Fluid and Microenvironment: Peritoneal fluid, a natural lubricant in the abdominal cavity, plays a role in the pathophysiology of peritoneal cancer. The fluid contains various molecules, growth factors, and immune cells that can influence the growth and spread of cancer cells. Changes in the composition and characteristics of the peritoneal fluid may promote tumor growth and metastasis.
Inflammation and Angiogenesis: Chronic inflammation within the peritoneum can contribute to the development and progression of peritoneal cancer. Inflammatory processes can release cytokines, growth factors, and chemokines that promote cell proliferation, angiogenesis (formation of new blood vessels), and tissue remodeling. These processes create a supportive environment for tumor growth.
Ascites Formation: Peritoneal cancer can cause the accumulation of fluid within the peritoneal cavity, leading to a condition called ascites. Ascites can develop due to increased vascular permeability, lymphatic obstruction, or impaired absorption of peritoneal fluid. The presence of ascites can further promote the spread of cancer cells within the peritoneum.
Family History: A family history of certain cancers, such as ovarian, breast, or colorectal, can increase the risk of developing peritoneal cancer. This suggests that there may be genetic factors involved in its development. Mutations in genes, such as BRCA1 and BRCA2, are associated with an increased risk of breast and ovarian cancers.
Hereditary Conditions: Certain hereditary conditions can predispose individuals to peritoneal cancer. For example, individuals with hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, have an increased risk of developing various types of cancer, including peritoneal cancer.
Genetic Mutations: Specific genetic mutations have been associated with an increased risk of peritoneal cancer. Mutations in genes involved in DNA repair mechanisms, such as BRCA1 and BRCA2, can increase the risk of peritoneal cancer. These mutations can be inherited or occur spontaneously.
Gender: Peritoneal cancer is more commonly diagnosed in women than in men. This may be partly because peritoneal cancer is often associated with ovarian cancer, and women have a higher incidence of ovarian cancer.
Age: The risk of peritoneal cancer increases with age. It is more commonly diagnosed in postmenopausal women, typically between 50 and 70.
Endometriosis: Endometriosis, a condition in which the tissue that usually lines the uterus grows outside the uterus, has been associated with an increased risk of developing peritoneal cancer. The exact relationship between endometriosis and peritoneal cancer is not fully understood, but chronic inflammation and hormonal factors may play a role.
Stage of Cancer: At diagnosis, the peritoneal stage is an important prognostic factor. The extent of cancer spread, including the size of the tumor, involvement of nearby organs, and presence of distant metastasis, influences the prognosis. Peritoneal cancer is typically staged using the FIGO (International Federation of Gynecology and Obstetrics) staging system.
Histopathological Features: The histopathological characteristics of the tumor can provide valuable prognostic information. Factors such as the histological type of cancer (e.g., serous, mucinous, endometrioid), tumor grade (degree of cellular abnormality and aggressiveness), and presence of specific molecular markers can impact prognosis. For example, a higher tumor grade is generally associated with a worse prognosis.
Completeness of Surgical Resection: The extent to which the tumor can be surgically removed, also known as the completeness of surgical resection or cytoreduction, is a crucial prognostic factor for peritoneal cancer. Achieving complete cytoreduction (removing all visible tumors) improves survival outcomes. This often involves a complex surgical procedure called cytoreductive surgery, sometimes combined with heated intraperitoneal chemotherapy (HIPEC).
Performance Status and Overall, Health: An individual’s overall health and functional status, as assessed by factors like performance status and comorbidities, can impact prognosis. Better overall health and performance status are generally associated with improved outcomes.
Response to Treatment: The response to treatment, including chemotherapy and targeted therapies, is an important prognostic factor. Individuals who have an excellent response to treatment and achieve disease control are more likely to have a favorable prognosis.
Genetic and Molecular Markers: Certain genetic and molecular markers can provide prognostic information for peritoneal cancer. For example, genetic mutations like BRCA1 and BRCA2 may impact prognosis and guide treatment decisions.
CLINICAL HISTORY
Age Group: Peritoneal cancer can occur in individuals of various age groups, but it is more commonly diagnosed in postmenopausal women, typically between 50 and 70. However, it can also affect younger women and, rarely, men. The symptoms and presentation may be similar across age groups, but the possibility of peritoneal cancer should be considered in postmenopausal women presenting with abdominal symptoms.
Associated Comorbidities or Activity Level: Comorbidities or specific activities can influence the clinical presentation of peritoneal cancer. For example, individuals with a history of endometriosis may be at higher risk for developing peritoneal cancer, and the presence of symptoms related to endometriosis along with abdominal pain or discomfort should raise suspicion. Additionally, individuals with obesity or other chronic conditions may have different symptom profiles or presentations due to overlapping symptoms or complications.
Acuity of Presentation: Peritoneal cancer can have a variable acuity of presentation. In some cases, symptoms may develop gradually and progress over time, while in other cases, symptoms may appear suddenly and be more severe. Acute presentations may occur when there is a rapid accumulation of fluid within the peritoneal cavity (ascites) or if there is bowel obstruction due to tumor growth.
Common clinical manifestations of peritoneal cancer may include:
Abdominal pain or discomfort: One of the most common symptoms can range from mild to severe. The pain may be constant or intermittent and may be localized or diffuse.
Abdominal distension or bloating: Ascites or tumor growth can increase abdominal size or bloating sensation.
Changes in bowel habits: This can include constipation, diarrhea, or alterations in bowel movements.
Loss of appetite and weight loss: Individuals with peritoneal cancer may experience a decreased appetite and unintentional weight loss.
Nausea and vomiting: These symptoms may occur due to obstruction or irritation of the gastrointestinal tract.
Fatigue and weakness: Generalized fatigue and weakness can be associated with advanced stages of peritoneal cancer or because of the systemic effects of the disease.
Abdominal Inspection: The healthcare provider may visually inspect the abdomen for any visible abnormalities, such as distension, asymmetry, or prominent veins. They may also observe for any signs of surgical scars or evidence of previous interventions.
Abdominal Palpation: The provider will use their hands to feel and palpate the abdomen, assessing for any areas of tenderness, masses, or organ enlargement. They may systematically palpate different abdomen regions, including the upper quadrants, lower quadrants, and pelvis.
Fluid Wave Test: The presence of ascites (fluid accumulation in the peritoneal cavity) may be assessed using the fluid wave test. The provider will tap one side of the abdomen while simultaneously palpating the opposite side. A positive fluid wave test occurs when the provider can feel a fluid wave, suggesting the presence of ascites.
Pelvic Examination: In women, a pelvic examination may be performed to assess the reproductive organs. The provider will visually inspect the external genitalia and then perform a manual examination of the uterus, ovaries, and surrounding structures, feeling for any masses, tenderness, or abnormal findings.
Lymph Node Examination: The healthcare provider may also assess for enlarged lymph nodes in the abdomen, groin, or other regions. Palpation of lymph nodes may help determine the presence of metastasis or spread of cancer.
Auscultation: The provider may use a stethoscope to listen for bowel sounds, which can provide information about the function and movement of the intestines. Absent or abnormal bowel sounds may suggest bowel obstruction or other gastrointestinal issues.
DIFFERENTIAL DIAGNOSIS
Ovarian Cancer: Ovarian cancer can cause abdominal pain, bloating, and distension, like peritoneal cancer. The two conditions can coexist or be difficult to differentiate as they may originate from the same tissue.
Gastrointestinal Cancers: Cancers affecting the gastrointestinal tract, such as colorectal cancer, gastric cancer, or pancreatic cancer, can manifest with abdominal pain, weight loss, changes in bowel habits, and abdominal distension.
Endometriosis: Endometriosis is a condition in which the tissue that usually lines the uterus grows outside the uterus. It can cause chronic pelvic pain, menstrual irregularities, and pelvic masses, which may mimic peritoneal cancer. Endometriosis and peritoneal cancer can coexist or share similar symptoms.
Inflammatory Bowel Disease (IBD): Conditions such as Crohn’s disease or ulcerative colitis, collectively known as inflammatory bowel disease, can cause abdominal pain, diarrhea, weight loss, and inflammation within the abdominal cavity.
Appendicitis: Acute appendicitis is characterized by sudden-onset abdominal pain, often localized to the right lower quadrant. While typically acute and self-limited, a perforated appendix or complications may present with more chronic symptoms and could be mistaken for peritoneal cancer.
Abdominal Tuberculosis: Tuberculosis (TB) can affect the peritoneum, leading to peritoneal tuberculosis. It can cause chronic abdominal pain, weight loss, and ascites, which can mimic peritoneal cancer.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors that can occur in the gastrointestinal tract, including the stomach and intestines. They can present with abdominal pain, bleeding, and abdominal masses, like peritoneal cancer.
Ascites of Non-Malignant Origin: Conditions such as liver cirrhosis, congestive heart failure, or infections can lead to fluid accumulation within the peritoneal cavity (ascites), which can cause abdominal distension and discomfort.
TREATMENT PARADIGM
The management of peritoneal cancer can be divided into several phases, including initial treatment, consolidation or maintenance therapy, and palliative care. Here is a general overview:
Modification of Environment:
Surgical Intervention: Cytoreductive surgery (CRS) is a critical component of treatment for peritoneal cancer. It involves the surgical removal of visible tumors within the peritoneal cavity. The goal is to achieve complete cytoreduction, removing all visible tumor nodules to the extent feasible. CRS is often performed in combination with other procedures.
Hyperthermic Intraperitoneal Chemotherapy (HIPEC): HIPEC is performed during cytoreductive surgery. After removing visible tumors, heated chemotherapy drugs are administered directly into the peritoneal cavity. The high temperature improves drug penetration and enhances its anticancer effects.
Peritoneal Access Devices: Peritoneal access devices, such as ports or catheters, may be implanted during surgery to allow repeated intraperitoneal chemotherapy administrations.
Administration of Pharmaceutical Agents:
Chemotherapy: Systemic chemotherapy may be used in addition to cytoreductive surgery and HIPEC. It involves administering anticancer drugs through intravenous infusion to target cancer cells throughout the body.
Targeted Therapies: Some peritoneal cancers may have specific molecular characteristics or genetic mutations that can be targeted with specialized drugs. These targeted therapies inhibit specific signaling pathways or molecular targets in cancer growth.
Intervention with Procedures:
Paracentesis: Paracentesis is a procedure in which a needle or catheter is inserted into the peritoneal cavity to drain excessive fluid (ascites). It can relieve symptoms such as abdominal distension and discomfort caused by ascites.
Pleurodesis: In cases where peritoneal cancer has spread to the pleural cavity, pleurodesis may be performed. It involves the introduction of a sclerosing agent into the pleural space to create adhesions and prevent fluid accumulation.
Phases of Management:
Initial Treatment: This phase involves the primary treatment approach, which often includes cytoreductive surgery, HIPEC, and systemic chemotherapy. The goal is to achieve optimal tumor reduction and disease control.
Consolidation/Maintenance Therapy: After initial treatment, consolidation or maintenance therapy may be recommended to control the disease further. This can involve additional cycles of systemic chemotherapy or intraperitoneal chemotherapy.
Palliative Care: In cases where curative treatment is not feasible or when cancer has advanced, palliative care aims to manage symptoms, provide pain relief, and improve the quality of life. Palliative care can involve pain management, nutritional and psychological support for the patient and their caregivers.
Continue the treatment till the disease progression or unacceptable toxicity occurs
Note:
Applicable for maintenance therapy, I.e., fallopian tube, recurrent epithelial ovarian, and peritoneal cancer who are in complete/partial response to platinum-based chemotherapy
Peritoneal cancer:ncbi.nlm.nih.gov
Peritoneal Metastasis:ncbi.nlm.nih.gov
Primary peritoneal serous:ncbi.nlm.nih.gov
medtigo
Peritoneal Cancer
Updated :
May 29, 2023
Peritoneal cancer, also known as primary peritoneal cancer, is a relatively rare cancer affecting the peritoneum. The peritoneum is an extremely permeable membrane that borders the inside of the abdomen and protects the organs within, including the digestive tract. Peritoneal cancer develops in the peritoneum’s cells, but it may also spread from other cancers that started in neighbouring organs, such as the ovaries, fallopian tubes, or colon.
It is considered primary cancer when peritoneal cancer develops from the peritoneal cells. However, distinguishing between primary peritoneal and advanced ovarian cancers is often difficult because they have similar symptoms and treatments. These include a family history of ovarian, breast, or colorectal cancer, a personal history of hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome, and specific genetic mutations, such as BRCA1 and BRCA2 mutations.
Peritoneal cancer is rare, and its prevalence and incidence rates vary across different populations.
Incidence: The incidence of peritoneal cancer is relatively low compared to other types of cancer. Peritoneal cancer is estimated to account for approximately 10% of all primary cancers originating in the female reproductive system.
Age and Gender: Men and women may both get peritoneal cancer, although women are more likely to have it. Most cases are found in postmenopausal women, typically between 50 and 70. However, peritoneal cancer can also occur in younger women and, rarely, men.
Risk Factors: Several risk factors have been associated with an increased likelihood of developing peritoneal cancer. These include a family history of ovarian, breast, or colorectal cancer, a history of hereditary nonpolyposis colorectal cancer (HNPCC), and genetic mutations such as BRCA1 and BRCA2. Other factors, such as increasing age, obesity, and a history of endometriosis, may also contribute to the development of peritoneal cancer.
Geographic Variations: Studies have shown some geographic variations in the incidence of peritoneal cancer. For instance, higher rates have been reported in developed countries compared to developing nations.
Survival Rates: Survival rates for peritoneal cancer depend on various factors, including the cancer stage at diagnosis, the individual’s overall health, and the response to treatment. Unfortunately, peritoneal cancer is often diagnosed at an advanced stage, negatively impacting survival outcomes. However, with advancements in treatment approaches, including cytoreductive surgery and heated intraperitoneal chemotherapy (HIPEC), survival rates have improved for selected patients.
PATHOPHYSIOLOGY
Genetic and Molecular Alterations: Like other types of cancer, peritoneal cancer involves genetic and molecular alterations in the affected cells. These alterations can disrupt the normal regulation of cell growth, division, and death. Mutations in genes such as TP53, BRCA1, BRCA2, and KRAS have been associated with the development of peritoneal cancer.
Metastasis: Peritoneal cancer can occur through direct extension from neighboring organs or metastasis from distant sites. Cancer cells can invade the peritoneum by breaking through the basement membrane and infiltrating the surrounding tissues. The cancer cells can then spread within the peritoneal fluid, allowing them to reach different peritoneum areas and form new tumors.
Peritoneal Fluid and Microenvironment: Peritoneal fluid, a natural lubricant in the abdominal cavity, plays a role in the pathophysiology of peritoneal cancer. The fluid contains various molecules, growth factors, and immune cells that can influence the growth and spread of cancer cells. Changes in the composition and characteristics of the peritoneal fluid may promote tumor growth and metastasis.
Inflammation and Angiogenesis: Chronic inflammation within the peritoneum can contribute to the development and progression of peritoneal cancer. Inflammatory processes can release cytokines, growth factors, and chemokines that promote cell proliferation, angiogenesis (formation of new blood vessels), and tissue remodeling. These processes create a supportive environment for tumor growth.
Ascites Formation: Peritoneal cancer can cause the accumulation of fluid within the peritoneal cavity, leading to a condition called ascites. Ascites can develop due to increased vascular permeability, lymphatic obstruction, or impaired absorption of peritoneal fluid. The presence of ascites can further promote the spread of cancer cells within the peritoneum.
Family History: A family history of certain cancers, such as ovarian, breast, or colorectal, can increase the risk of developing peritoneal cancer. This suggests that there may be genetic factors involved in its development. Mutations in genes, such as BRCA1 and BRCA2, are associated with an increased risk of breast and ovarian cancers.
Hereditary Conditions: Certain hereditary conditions can predispose individuals to peritoneal cancer. For example, individuals with hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, have an increased risk of developing various types of cancer, including peritoneal cancer.
Genetic Mutations: Specific genetic mutations have been associated with an increased risk of peritoneal cancer. Mutations in genes involved in DNA repair mechanisms, such as BRCA1 and BRCA2, can increase the risk of peritoneal cancer. These mutations can be inherited or occur spontaneously.
Gender: Peritoneal cancer is more commonly diagnosed in women than in men. This may be partly because peritoneal cancer is often associated with ovarian cancer, and women have a higher incidence of ovarian cancer.
Age: The risk of peritoneal cancer increases with age. It is more commonly diagnosed in postmenopausal women, typically between 50 and 70.
Endometriosis: Endometriosis, a condition in which the tissue that usually lines the uterus grows outside the uterus, has been associated with an increased risk of developing peritoneal cancer. The exact relationship between endometriosis and peritoneal cancer is not fully understood, but chronic inflammation and hormonal factors may play a role.
Stage of Cancer: At diagnosis, the peritoneal stage is an important prognostic factor. The extent of cancer spread, including the size of the tumor, involvement of nearby organs, and presence of distant metastasis, influences the prognosis. Peritoneal cancer is typically staged using the FIGO (International Federation of Gynecology and Obstetrics) staging system.
Histopathological Features: The histopathological characteristics of the tumor can provide valuable prognostic information. Factors such as the histological type of cancer (e.g., serous, mucinous, endometrioid), tumor grade (degree of cellular abnormality and aggressiveness), and presence of specific molecular markers can impact prognosis. For example, a higher tumor grade is generally associated with a worse prognosis.
Completeness of Surgical Resection: The extent to which the tumor can be surgically removed, also known as the completeness of surgical resection or cytoreduction, is a crucial prognostic factor for peritoneal cancer. Achieving complete cytoreduction (removing all visible tumors) improves survival outcomes. This often involves a complex surgical procedure called cytoreductive surgery, sometimes combined with heated intraperitoneal chemotherapy (HIPEC).
Performance Status and Overall, Health: An individual’s overall health and functional status, as assessed by factors like performance status and comorbidities, can impact prognosis. Better overall health and performance status are generally associated with improved outcomes.
Response to Treatment: The response to treatment, including chemotherapy and targeted therapies, is an important prognostic factor. Individuals who have an excellent response to treatment and achieve disease control are more likely to have a favorable prognosis.
Genetic and Molecular Markers: Certain genetic and molecular markers can provide prognostic information for peritoneal cancer. For example, genetic mutations like BRCA1 and BRCA2 may impact prognosis and guide treatment decisions.
CLINICAL HISTORY
Age Group: Peritoneal cancer can occur in individuals of various age groups, but it is more commonly diagnosed in postmenopausal women, typically between 50 and 70. However, it can also affect younger women and, rarely, men. The symptoms and presentation may be similar across age groups, but the possibility of peritoneal cancer should be considered in postmenopausal women presenting with abdominal symptoms.
Associated Comorbidities or Activity Level: Comorbidities or specific activities can influence the clinical presentation of peritoneal cancer. For example, individuals with a history of endometriosis may be at higher risk for developing peritoneal cancer, and the presence of symptoms related to endometriosis along with abdominal pain or discomfort should raise suspicion. Additionally, individuals with obesity or other chronic conditions may have different symptom profiles or presentations due to overlapping symptoms or complications.
Acuity of Presentation: Peritoneal cancer can have a variable acuity of presentation. In some cases, symptoms may develop gradually and progress over time, while in other cases, symptoms may appear suddenly and be more severe. Acute presentations may occur when there is a rapid accumulation of fluid within the peritoneal cavity (ascites) or if there is bowel obstruction due to tumor growth.
Common clinical manifestations of peritoneal cancer may include:
Abdominal pain or discomfort: One of the most common symptoms can range from mild to severe. The pain may be constant or intermittent and may be localized or diffuse.
Abdominal distension or bloating: Ascites or tumor growth can increase abdominal size or bloating sensation.
Changes in bowel habits: This can include constipation, diarrhea, or alterations in bowel movements.
Loss of appetite and weight loss: Individuals with peritoneal cancer may experience a decreased appetite and unintentional weight loss.
Nausea and vomiting: These symptoms may occur due to obstruction or irritation of the gastrointestinal tract.
Fatigue and weakness: Generalized fatigue and weakness can be associated with advanced stages of peritoneal cancer or because of the systemic effects of the disease.
Abdominal Inspection: The healthcare provider may visually inspect the abdomen for any visible abnormalities, such as distension, asymmetry, or prominent veins. They may also observe for any signs of surgical scars or evidence of previous interventions.
Abdominal Palpation: The provider will use their hands to feel and palpate the abdomen, assessing for any areas of tenderness, masses, or organ enlargement. They may systematically palpate different abdomen regions, including the upper quadrants, lower quadrants, and pelvis.
Fluid Wave Test: The presence of ascites (fluid accumulation in the peritoneal cavity) may be assessed using the fluid wave test. The provider will tap one side of the abdomen while simultaneously palpating the opposite side. A positive fluid wave test occurs when the provider can feel a fluid wave, suggesting the presence of ascites.
Pelvic Examination: In women, a pelvic examination may be performed to assess the reproductive organs. The provider will visually inspect the external genitalia and then perform a manual examination of the uterus, ovaries, and surrounding structures, feeling for any masses, tenderness, or abnormal findings.
Lymph Node Examination: The healthcare provider may also assess for enlarged lymph nodes in the abdomen, groin, or other regions. Palpation of lymph nodes may help determine the presence of metastasis or spread of cancer.
Auscultation: The provider may use a stethoscope to listen for bowel sounds, which can provide information about the function and movement of the intestines. Absent or abnormal bowel sounds may suggest bowel obstruction or other gastrointestinal issues.
DIFFERENTIAL DIAGNOSIS
Ovarian Cancer: Ovarian cancer can cause abdominal pain, bloating, and distension, like peritoneal cancer. The two conditions can coexist or be difficult to differentiate as they may originate from the same tissue.
Gastrointestinal Cancers: Cancers affecting the gastrointestinal tract, such as colorectal cancer, gastric cancer, or pancreatic cancer, can manifest with abdominal pain, weight loss, changes in bowel habits, and abdominal distension.
Endometriosis: Endometriosis is a condition in which the tissue that usually lines the uterus grows outside the uterus. It can cause chronic pelvic pain, menstrual irregularities, and pelvic masses, which may mimic peritoneal cancer. Endometriosis and peritoneal cancer can coexist or share similar symptoms.
Inflammatory Bowel Disease (IBD): Conditions such as Crohn’s disease or ulcerative colitis, collectively known as inflammatory bowel disease, can cause abdominal pain, diarrhea, weight loss, and inflammation within the abdominal cavity.
Appendicitis: Acute appendicitis is characterized by sudden-onset abdominal pain, often localized to the right lower quadrant. While typically acute and self-limited, a perforated appendix or complications may present with more chronic symptoms and could be mistaken for peritoneal cancer.
Abdominal Tuberculosis: Tuberculosis (TB) can affect the peritoneum, leading to peritoneal tuberculosis. It can cause chronic abdominal pain, weight loss, and ascites, which can mimic peritoneal cancer.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors that can occur in the gastrointestinal tract, including the stomach and intestines. They can present with abdominal pain, bleeding, and abdominal masses, like peritoneal cancer.
Ascites of Non-Malignant Origin: Conditions such as liver cirrhosis, congestive heart failure, or infections can lead to fluid accumulation within the peritoneal cavity (ascites), which can cause abdominal distension and discomfort.
TREATMENT PARADIGM
The management of peritoneal cancer can be divided into several phases, including initial treatment, consolidation or maintenance therapy, and palliative care. Here is a general overview:
Modification of Environment:
Surgical Intervention: Cytoreductive surgery (CRS) is a critical component of treatment for peritoneal cancer. It involves the surgical removal of visible tumors within the peritoneal cavity. The goal is to achieve complete cytoreduction, removing all visible tumor nodules to the extent feasible. CRS is often performed in combination with other procedures.
Hyperthermic Intraperitoneal Chemotherapy (HIPEC): HIPEC is performed during cytoreductive surgery. After removing visible tumors, heated chemotherapy drugs are administered directly into the peritoneal cavity. The high temperature improves drug penetration and enhances its anticancer effects.
Peritoneal Access Devices: Peritoneal access devices, such as ports or catheters, may be implanted during surgery to allow repeated intraperitoneal chemotherapy administrations.
Administration of Pharmaceutical Agents:
Chemotherapy: Systemic chemotherapy may be used in addition to cytoreductive surgery and HIPEC. It involves administering anticancer drugs through intravenous infusion to target cancer cells throughout the body.
Targeted Therapies: Some peritoneal cancers may have specific molecular characteristics or genetic mutations that can be targeted with specialized drugs. These targeted therapies inhibit specific signaling pathways or molecular targets in cancer growth.
Intervention with Procedures:
Paracentesis: Paracentesis is a procedure in which a needle or catheter is inserted into the peritoneal cavity to drain excessive fluid (ascites). It can relieve symptoms such as abdominal distension and discomfort caused by ascites.
Pleurodesis: In cases where peritoneal cancer has spread to the pleural cavity, pleurodesis may be performed. It involves the introduction of a sclerosing agent into the pleural space to create adhesions and prevent fluid accumulation.
Phases of Management:
Initial Treatment: This phase involves the primary treatment approach, which often includes cytoreductive surgery, HIPEC, and systemic chemotherapy. The goal is to achieve optimal tumor reduction and disease control.
Consolidation/Maintenance Therapy: After initial treatment, consolidation or maintenance therapy may be recommended to control the disease further. This can involve additional cycles of systemic chemotherapy or intraperitoneal chemotherapy.
Palliative Care: In cases where curative treatment is not feasible or when cancer has advanced, palliative care aims to manage symptoms, provide pain relief, and improve the quality of life. Palliative care can involve pain management, nutritional and psychological support for the patient and their caregivers.
Peritoneal cancer:ncbi.nlm.nih.gov
Peritoneal Metastasis:ncbi.nlm.nih.gov
Primary peritoneal serous:ncbi.nlm.nih.gov
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