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Primary Peritoneal Carcinoma - Causes, Symptoms, Diagnosis, and Treatment

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Primary peritoneal cancer is rare and originates in the abdominal cavity or peritoneum lining, usually in the pelvic organ-covering area.

Medically reviewed by

Dr. Rajesh Gulati

Published At March 28, 2024
Reviewed AtMarch 28, 2024

Introduction

PPC is an uncommon form of primary peritoneal carcinoma. It initially affects the delicate layer of tissue that lines the abdominal cavity. The peritoneum is the name given to this lung lining. The predominant subtype of ovarian cancer cells is identical to PPC cells. The rationale is that during fetal development, the ovary's surface and the abdomen's lining originate from the same tissue. Thus, PPC is managed similarly to ovarian cancer by medical professionals.

What Is the Peritoneum?

The peritoneum surrounds every organ in the abdomen, including the liver and intestines. It serves as an infection barrier in addition to protecting the organs. It is composed of two layers. The parietal layer is the layer that runs along the wall of the abdomen. The second layer, known as the visceral layer, is what surrounds the organs.

The two layers can slide over one another because of the tiny amount of fluid that keeps them apart. One may move around without producing friction on the layers because of this fluid.

What Is Primary Peritoneal Carcinoma?

Peritoneal carcinoma is a distinct kind of malignancy. Women are primarily affected by PPC. Males rarely experience this condition. Most people are diagnosed after reaching the age of 60. Most of the women diagnosed with ovarian, fallopian, and peritoneal serous malignancies concurrently exhibit a pre-existing condition referred to as pelvic pleural carcinoma (PPC).

The etiology of PPC remains unknown. The majority of malignancies originate from the amalgamation of many factors. A minority of preterm infants (PPCs) may exhibit a potential association with the genetically defective BRCA 1 and BRCA 2 genes. The genes above demonstrate genetic similarities to those linked to an elevated vulnerability to the onset of ovarian cancer and breast cancer.

Which Individuals Are Susceptible to Developing Primary Peritoneal Cancer?

Females exhibit a greater susceptibility to primary peritoneal cancer compared to males. The risk factors for peritoneal cancer are comparable to those for ovarian cancer and encompass:

  • Age: The majority of individuals diagnosed with primary peritoneal carcinoma are typically six years of age or older.

  • Endometriosis: Endometriosis, characterized by the growth of endometrial tissue outside the uterus, is associated with an elevated risk.

  • Genetical: The presence of a first-degree family member (specifically, a mother, sister, or daughter) who has been diagnosed with peritoneal cancer, fallopian tube cancer, or ovarian cancer is associated with an elevated risk in terms of genetics. Women who possess mutations in the BRCA1 or BRCA2 genes are also at an elevated risk. Individuals possessing Lynch syndrome genes, an inherited disorder that increases the susceptibility to several forms of cancer, are also affected.

  • Height: Individuals with greater height exhibit an elevated susceptibility to primary peritoneal cancer.

  • Hormone Replacement Therapy: Postmenopausal women who undergo hormone therapy face an elevated risk.

  • Obesity: Individuals with more fatty tissue are at an elevated risk.

  • Reproductive History: Individuals experiencing infertility and lacking biological offspring may exhibit an elevated susceptibility.

What Symptoms Are Associated With Primary Peritoneal Cancer?

As with ovarian cancer, early phases of the disease are frequently asymptomatic. If so, symptoms are frequently vague. The majority of individuals develop symptoms as cancer progresses. Those who do exhibit symptoms during the initial phases may comprise the following:

  • Pain in the abdomen and pelvis.

  • Unusual vaginal discharge or hemorrhage.

  • A sensation of fullness or bloating in the abdomen or pelvis.

  • Alterations in the bowel (increased flatulence and constipation) or rectal bleeding.

  • Prolonged urination.

  • Symptoms such as indigestion, appetite loss, or feeling replete before finishing a meal.

  • Unintentional weight reduction or gain.

As peritoneal cancer progresses, the following additional symptoms may manifest:

  • Weakness and fatigue.

  • The accumulation of fluid in the abdomen is known as ascites.

  • Experiencing nausea or regurgitation.

  • Dyspnea or breathlessness.

  • Leg swelling may develop.

What Complications Are Associated With Primary Peritoneal Cancer?

Patients diagnosed with advanced peritoneal cancer may experience the subsequent complications:

  • Experiencing abdominal discomfort.

  • Lack of ability to consume food or liquids.

  • Severe vomiting or vertigo.

  • Obstacles of the small intestine, large intestine, or urinary tract.

What Is the Diagnostic Process for Primary Peritoneal Cancer?

The healthcare provider will initiate the process by doing a comprehensive history and physical examination while inquiring about prevailing symptoms. If the symptoms indicate the presence of peritoneal cancer, it is possible to have:

  • Blood tests detect the presence of CA-125, a substance produced by certain cancer cells. Individuals with peritoneal cancer may exhibit elevated levels of this substance. Peritoneal cancer cells may also create elevated quantities of a protein known as HE4, which can be detected through blood tests.

  • Imaging modalities such as ultrasound, MRI, or CT scan are utilized to detect malignancies. However, the detection of peritoneal cancer can be challenging with these techniques.

  • Laparoscopy is a diagnostic procedure that can be conducted by healthcare providers. This treatment entails the creation of several minute incisions in the abdominal region. During the process, a biopsy may be performed to get a specimen of anomalous tissues. Subsequently, a pathologist scrutinizes these tissues using a microscope to ascertain the presence of malignancy.

  • The paracentesis procedure involves the collection of a sample of abdomen fluid by a healthcare provider in cases of ascites. Peritoneal paracentesis is frequently conducted by healthcare providers in the context of laparoscopy.

  • The pelvic examination involves a comprehensive assessment of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum to identify any potential abnormalities or masses.

What Is the Management or Treatment Approach for Primary Peritoneal Cancer?

The management approach for primary peritoneal carcinoma is contingent upon the following:

  • The dimensions and positioning of the tumor.

  • Stage of cancer.

  • Patients' age and general well-being.

Typically, a physician will initiate surgery as the initial course of treatment. The individual may have a medical intervention known as cytoreductive surgery. The objective of this surgical procedure is to excise the cancerous tissue fully. Studies indicate that the results are superior when a gynecologic oncologist conducts the procedure. These physicians have expertise in managing ailments that impact the female reproductive system, such as cancer.

In addition, the surgeon may additionally extract the:

  • Removal of the fallopian tubes and ovaries (oophorectomy).

  • Hysterectomy of the uterus.

  • The omentum, also known as omentectomy, refers to the adipose tissue that surrounds the intestines.

  • Additional anomalous tissue.

To impede the metastasis of cancer cells to distant anatomical sites, the therapy regimen may encompass the following components:

  • Chemotherapy: Conventional chemotherapy for peritoneal cancer is administered intravenously or intraperitoneally, either through the veins or directly into the abdominal cavity. Providers may employ hot chemotherapy medications or hyperthermic intraperitoneal chemotherapy (HIPEC) in specific instances. In the context of cytoreductive surgery, the administration of HIPEC occurs promptly within the abdominal cavity, concurrently with the patient's presence in the operating room and under anesthesia. HIPEC may offer enhanced efficacy in chemotherapy delivery for certain types of cancer.

  • Targeted Therapy: It involves the administration of specialized medications by healthcare providers to selectively target cancer cells while minimizing the impact on healthy cells.

  • Hormonal Therapy: It is occasionally employed in conjunction with chemotherapy by healthcare providers in instances of advanced disease. This therapeutic intervention inhibits the transmission of endogenous hormones to malignant cells, hence impeding their proliferation.

  • Radiation Therapy: It is hardly employed as the initial therapeutic approach for peritoneal cancer by healthcare providers. They may provide radiation therapy to specifically target localized cancerous regions that have resurfaced following initial therapy.

Conclusion

The tumor known as PPC is characterized by its rarity and aggressiveness, primarily affecting females. Its clinical presentation typically poses a diagnostic challenge due to its resemblance to ovarian cancer. Imaging modalities and examining ascitic fluid play a crucial role in differentiating PPC. Thus, thoroughly examining and understanding the comprehensive investigation must assist in prompt diagnosis and efficient treatment.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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