Patients with pseudomyxoma peritonei present with symptoms that are intimately related to the deposition of mucus in the abdomen and the pelvis. The associated increase in abdominal size is commonly referred to as "jelly belly". Prominent symptoms include abdominal pain but there is frequently an absence of tenderness on palpation of the abdomen. Male patients can also present with inguinal hernia, defined as a partial protrusion of the bowels through a defect in the abdominal musculature. Women, on the other hand, develop large ovaries as the tumor cells settle and grow rapidly there.

Tumor cells accumulate particularly over the greater omentum covering the bowels, underneath the diaphragm and in the pelvis. Most cases do not initially involve the intestines but, in the absence of treatment, bowel obstruction may develop leading to a loss of intestinal function and progression into poor food intake, inadequate nutrition and, ultimately, complications that may prove life threatening.

A range of disorders can present similarly to pseudomyxoma peritonei, and it is critical for the physician to accurately differentiate among them.

Unlike pseudomyxoma peritonei, ovarian tumors usually consist of a single, expanding solid mass. They do not go into the abdomen but patients frequently complain of abdominal and lower back pain. Mucinous cancers of the ovary, stomach, gallbladder and colon are similar to pseudomyxoma peritonei and are chiefly characterized by a an excess of mucous production in the abdominal cavity, but they are usually more aggressive than mucinous tumors arising in the appendix.

Mesotheliomas are tumors of the protective layers that envelop and cover organs in the body. Mesotheliomas most commonly target the pleura, the membranes covering the lungs. Other variants can also involve the stomach, small and large intestines and the pericardium, the lining of the heart. They present very similarly to pseudomyxoma peritonei and it is usually very difficult to distinguish between the two conditions. Associated symptoms depend on the location, stage and type of the tumors. Asbestos is a major risk factor for mesotheliomas and is responsible for approximately 80% of all the cases. The disease can start almost 50 years after the exposure to asbestos but, once it develops, progression is very rapid and the disease is frequently fatal.

Appendicitis, an acute inflammation of the appendix, is also present on the differential diagnosis. The first sign is usually a slight discomfort around the navel area, which progresses into severe pain and localizes into the right lower quadrant of the abdomen. Prominent signs and symptoms include nausea, vomiting, fever and tenderness upon palpation of the right lower quadrant.

• Pain

  A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. [ncbi.nlm.nih.gov]

  In less advanced stages females are admitted to hospitals with lower abdominal pain, pelvic or ovarian masses, infertility and menstruation disturbances [17]. [termedia.pl]

  They do not go into the abdomen but patients frequently complain of abdominal and lower back pain. [symptoma.com]

  Abdominal pain may result from this. Inflammation of the large intestine is accompanied by redness, protrusion, and pain in the skin. [news-medical.net]

• Inguinal Hernia

  The intraabdominal mucus can accumulate in the inguinal canal and by physical examination be indistinguishable from the usual inguinal hernia. [ncbi.nlm.nih.gov]

  Diagnosis may follow discovery of an ovarian mass in women or recent development of inguinal hernia, appendicitis or intestinal occlusion. [orpha.net]

  hernia (25%) Next most common in females are bilateral or unilateral ovarian masses Imaging Findings On CT scan, mucinous material has similar density to fat and appears heterogeneous. [learningradiology.com]

• Splenectomy

  Cyst rupture was noted on computed tomography, and splenectomy was performed. The abdominal cavity was filled with a large amount of gelatinous ascites, with the appearance of PMP. [ncbi.nlm.nih.gov]

  Splenectomy. Removal of your spleen. Your spleen is part of your immune system. After removing it, you’ll have to take antibiotics for the rest of your life to help prevent infections. Cholecystectomy. Removal of your gallbladder. [my.clevelandclinic.org]

  • Combined Modality Treatment – Introduced in the 1990s by Sugarbaker – Aims at achieving cure or at least long-term remission – Aggressive cytoreductive surgery • Parietal peritonectomy – Greater omentectomy – Splenectomy – Stripping of the left diaphragm [de.slideshare.net]

• Falling

  One French study found that using specialised and expert services mortality falls to approximately 4% when malignancies do not develop and many sufferers are finding that successful surgery allows them to return to a close to normal life in most cases [blogs.scientificamerican.com]

  This past fall the incredible Dr. Temple retired; he will always have an indelible mark in my family's life and all our joys, so I hope he gets to enjoy a wonderful retirement. [beatingjellybelly.blogspot.com]

  An intermediate grade tumour has also been described with a prognosis that falls between DPAM and PMCA. 1 More recently similar two and three category systems have been proposed by Bradley et al 5 and Misdraji et al. 6 Pseudomyxoma peritonei is a rare [nzma.org.nz]

  My only surviving aunt, aged 95, had had a serious fall and had died. [camsoftpartners.co.uk]

• Weight Gain

  When symptoms occur, they may include: slow increase in waist size hernia (a swelling on the abdomen) loss of appetite unexplained weight gain abdominal or pelvic pain changes in bowel habits appendicitis. [christie.nhs.uk]

  But some of the following may appear over time: Belly pain Changes in your bowel habits Enlarged ovary in women Hernia (a bulge near your groin) Poor appetite Swollen belly Weight gain or a bigger waist size Diagnosis It can be hard to diagnose PMP because [webmd.com]

• Dyspnea

  She presented with progressive dyspnea and abdominal distention. Abdominal computed tomography revealed moderately dense ascites without an obvious mass. Chest computed tomography revealed a large, solitary right lower-lobe lung mass. [ncbi.nlm.nih.gov]

  Other less common signs include abdominal pain, weight loss, urinary symptoms, constipation, vomiting, and dyspnea. [orpha.net]

  Case presentation: We report the case of a 76-year-old woman who presented with increased abdominal girth and dyspnea for 2 weeks. She was diagnosed as a case of pseudomyxoma peritonei. [dovepress.com]

• Abdominal Pain

  A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. [ncbi.nlm.nih.gov]

  In less advanced stages females are admitted to hospitals with lower abdominal pain, pelvic or ovarian masses, infertility and menstruation disturbances [17]. [termedia.pl]

• Abdominal Distension

  […] produces abdominal distension Characterized by re-accumulation after debulking Ultimately leads to intestinal obstruction No metastatic spread to nodes or organ parenchyma other than ovary Pleural involvement rare Due to communications across the diaphragm [surgpathcriteria.stanford.edu]

  We report a case of a 30-year-old woman who had complained of lower abdominal distension. She was noted to have a history of primary mucinous tumor of the left ovary (13.2 × 9.9 × 10.4 cm) that was removed surgically. [ncbi.nlm.nih.gov]

• Nausea

  A 55-year-old man presented with a five-month history of increasing abdominal girth, a weight loss of 4.5 kg (10 lb), and occasional episodes of anorexia and nausea. [nejm.org]

  A 46-year-old female with a past medical history notable for depression, asthma, and uterine leiomyomas presented to an urgent care with 5 days of progressive abdominal pain, bloating, nausea, and subjective fevers. [ncbi.nlm.nih.gov]

  Nausea and vomiting Nausea and vomiting may be symptoms of many medical conditions. Both symptoms are related, but each one may occur separately. In PMP, these symptoms arise due to gastrointestinal disorders such as infectious gastroenteritis. [news-medical.net]

  Compression in your digestive system may cause nausea or loss of appetite. Constipation. Abdominal swelling may cause an intestinal blockage. Hernias. Compression may push one of your organs through a gap in your muscle wall. [my.clevelandclinic.org]

• Abdominal Mass

  Although rare, neural compression caused by PMP and intra-abdominal masses needs to be considered in the differential diagnosis of sciatica. [ncbi.nlm.nih.gov]

  There is a female:male predominance of 4:1 and the mean age at presentation is about 55 years. 1,2 Clinical features include pain in the right lower quadrant, a palpable abdominal mass, and intermittent colicky pain.Approximately 25% of mucoceles are [appliedradiology.com]

• Constipation

  Other less common signs include abdominal pain, weight loss, urinary symptoms, constipation, vomiting, and dyspnea. [orpha.net]

  Constipation Defecation difficulties or blockage that leads to swelling or pain in the abdomen is another symptom of pseudomyxoma peritonie. Nausea and vomiting Nausea and vomiting may be symptoms of many medical conditions. [news-medical.net]

  Constipation. Abdominal swelling may cause an intestinal blockage. Hernias. Compression may push one of your organs through a gap in your muscle wall. Inguinal hernia is one of the most common findings in people assigned male at birth. [my.clevelandclinic.org]

• Aggressive Behavior

  In addition to the benign subtype, a malignant subtype resembling colon cancer has been described that has a more aggressive behavior. This subtype is sometimes referred to as “high grade PMP” and also peritoneal mucinous adenocarcinoma (PMCA). [hipec.com]

• Irritability

  The symptoms include: Abnormality of the abdomen, the abdominal wall, and the peritoneum: Swelling of the abdomen Abdominal pain Irritation Redness Increased size of the abdomen is the main symptom associated with PMP, which occurs because of conditions [news-medical.net]

  Clinical conditions showed no peritoneal irritation, normal peristalsis and dyarrhea in the last three days. No vomiting or fever were reported. Blood exams showed a WBC count of 11,250/mm3 with CRP value of 11 mg/l and CEA of 38,9 UI/ml. [journals.lww.com]

  The symptoms can be non-specific and are often misdiagnosed as irritable bowel and ovarian neoplasia in women. How is it diagnosed? The diagnosis of PMP can be difficult. [christie.nhs.uk]

  Physical examination (abdominal distention with so-called “jelly belly” ascites, and less often fever and peritoneal irritation) and laboratory tests (leukocytosis, anemia) are also not discriminative 33. [current-oncology.com]

  It has been suggested that this might arise because of mucinous metaplasia due to chronic irritation from ascitic fluid 7. Despite this controversy, the appendix is still the alleged dominant origin associated with PMP 10. [ispub.com]

• Cesarean Section

  A review of the medical literature revealed no cases of PMP diagnosed at time of cesarean section. A previously healthy gravida 5, para 3 underwent cesarean section. [ncbi.nlm.nih.gov]

Diagnostic workup of pseudomyxoma peritonei is initiated by a high index of suspicion and confirmed with imaging tests through computerized tomography and histological examination of the tumor cells [13]. The use of ultrasound has also proven to be effective in providing additional useful information for the diagnosis of the disease [14]. Furthermore, some reports show abnormal radionuclide uptake, although more extensive studies need to be performed for confirmation [15].

• Ovarian Mass

  Typically, the disease presents as suspected acute appendicitis, ovarian mass, abdominal distension, or ventral hernia. Our case represents a very rare presentation of superinfected PMP. [ncbi.nlm.nih.gov]

  Diagnosis may follow discovery of an ovarian mass in women or recent development of inguinal hernia, appendicitis or intestinal occlusion. [orpha.net]

  masses Imaging Findings On CT scan, mucinous material has similar density to fat and appears heterogeneous. [learningradiology.com]

The Washington Hospital center has pioneered the cytoreductive approach, consisting of a peritonectomy supplemented with preoperative chemotherapy [7]. The peritonectomy is a procedure in which the parietal peritoneum is fully stripped and the visceral peritoneum is subsequently resected. Sugerbaker et al. [7] recommend the extensive cytoreduction for grade I mucinous adenocarcinoma in combination with intra and postoperative chemotherapy, and other studies report success of this procedure even in patients with a very poor general condition [7] [17]. Recent innovations include intraperitoneal chemotherapy with implantable port systems, although success rates have been mixed [16] [17]. 

Pseudomyxoma peritonei has a poor prognosis. With repeated surgeries, the median survival rate is approximately two years with a small proportion surviving to five years [7]. Patients who undergo extensive chemotherapy in addition to repeated surgeries generally fare better, but a full cure should not be expected.

The cause of pseudomyxoma peritonei remains unknown, and no genetic, familial or environmental factors have been identified. The development of abnormal growth in the appendix in the form of a polyp results in the perforation of the appendix and the subsequent spreading of tumor cells in characteristic locations. They can be found especially in the greater omentum (the fatty tissue that covers the intestines), pelvis, diaphragm and ovaries (in women). The tumor cells continue their growth in their new locations.

Pseudomyxoma peritonei is a very rare disorder with an incidence of 1 case over 100,000 people per year. It peaks around 50 years of age and is slightly less common in men than in women.

Pseudomyxoma peritonei was thought previously to arise from a variety of tumors but it is now established that the majority of cases are related to tumors in the appendix [6] [7]. A variant termed mucinous peritoneal carcinomatosis has a poor prognosis and is characterized by signet ring cells. It can occur in the pancreas, gallbladder, colon or stomach and then implants randomly in the abdominal cavity. There is commonly a significant involvement of the small bowel. 

The redistribution phenomenon is a distinguishing characteristic of pseudomyxoma peritonei when no prior surgery has occurred [8] [9] [10] [11] [12]. The tumor spreads frequently to the greater omentum, the fat that is present on top of the bowels, and to the diaphragm, the muscular structure that separates the abdominal cavity from the thorax. The greater omentum and the diaphragm are both sites of absorption. Due to the effect of gravity, pseudomyxoma peritonei tumors can also settle in the dependent portions of the abdominal cavity. The pattern of redistribution generally spares the visceral peritoneum due to the intense and frequent peristaltic movements that occur in the gastrointestinal tract.

Tumor cells involved in pseudomyxoma peritonei can be distinguished due to their lack of adherence molecules, enabling them to move freely in the abdomen and avoid sticking to a particular structure. Histological examination of cancer tissue shows "dissecting mucous", which should not be confused with malignant transformation.

Preventive measures consist of aggressive surgery in combination with intraperitoneal lavage with water and dextrose, thus preventing the continued accumulation of tumors cells in the abdomen. This method has been shown to be particularly effective for pseudomyxoma peritonei associated with ovarian mucinous cystadenoma, in the presence of multiple peritoneal implants [18].

Pseudomyxoma peritonei is a type of cancerous growth characterized by the production of large quantities of mucin in the abdomen [1]. The tumor cells spread throughout the abdomen and pelvis and subsequently damage the function of the large and small intestines, the liver, the ovaries, the uterus, the ovaries or other organs. Prognosis has improved but severe complications such as obstruction of the intestines and cachexia can lead to death if the disease is left untreated [2].

Although the tumor most commonly arises from an infiltrating polyp in the appendix, some tumors that secrete mucin can also occur in the stomach, pancreas, colon and rectum [3] [4]. In addition, primary ovarian tumors can secrete mucin and may display very similar characteristics to pseudomyxoma peritonei, although it remains unknown whether these tumors originally arise in the appendix and then metastasize to the ovaries [5].

Treatment is variable and depends on symptom severity and complications, but the most successful procedure is the Sugarbaker technique, in which the surgeon performes a peritonectomy, in which the parietal peritoneum is stripped and the visceral peritoneum is resected. Surgery is accompanied by the administration of chemotherapeutic agents within the abdomen (intraoperative chemotherapy) to rid the body of any remaining tumor cells and decrease the rate of recurrence. Some cases of pseudomyxoma peritonei develop very slowly and require only observation and frequent monitoring.

Pseudomyxoma peritonei is a very rare disease characterized by tumor growth in various parts of the abdominal cavity. It arises most frequently from growth of tissue in the appendix (polyp) and then spreads along the lining of the abdomen and the diaphragm, the muscle that separates the abdomen from the chest cavity (the thorax). The tumor cells characteristically produce a jelly like substance called mucin, which progressively collects and leads to symptoms and complications. Pseudomyxoma peritonei can also arise from tumors in the intestines, bladder or ovaries. The primary cause of the disease is still unknown but multiple factors are thought to be involved, depending on the type of cancer.

Unlike other cancers, pseudomyxoma peritonei does not spread through the lymphatic system or blood stream. Because it lacks molecules that would otherwise permit it to stick in particular locations, it can roam free in the abdomen and tumor cells can be present throughout the abdominal cavity. Pseudomyxoma peritonei develops very slowly and symptoms can appear years after the initial development of the disease. 

Patients with pseudomyxoma peritonei develop symptoms that are mostly related to the accumulation of mucin in the abdomen. These symptoms can manifest with pain in abdomen and pelvis in addition to swelling, bloating, infertility and diarrhea or constipation. In some cases, however, patients will not develop these characteristic symptoms and diagnosis can become extremely difficult. In particular, the disease can be difficult to distinguish from variants of ovarian cancer that produce mucin and can also result in swelling and pain in the abdomen and pelvis. Most often, the diagnosis can only be conclusively determined after an operation permitting the exploration of the abdomen (exploratory laparatomy).

Treatment of pseudomyxoma peritonei is variable and is dependent on tumor size and overall health. Any of the following treatment types can be recommended: chemotherapy, debulking surgery, surgery complimented with chemotherapy or observation and periodic assessment.

The most common approach consists of surgery in combination with chemotherapy. The procedure was developed by Sugarbaker and is named after him. It consists of the surgical removal of the tumor in the abdomen by stripping the membrane that covers abdominal organs (peritoneum) and the subsequent removal of any tissue that has been seeded with tumor cells. Various organs can be involved and they include the small and large intestines, uterus, ovaries, liver and spleen. The surgical treatment is followed by the administration of chemotherapy directly within the abdomen (intraperitoneal chemotherapy). This helps in ridding the body of any tumor cells that may still be present and decreases the risk of recurrence. The whole operation is intensive, takes about 10 hours and requires an extended period of recovery, depending on the type and number of organs affected. The patient may remain in the hospital for up to 3 weeks and complications can develop in approximately 30% of patients. In addition, 20% of patients may require a stoma, which is an opening made on the surface of the abdomen to divert the flow of feces and urine. Around half of the patients will only need the stoma for 4 to 6 months. Furthermore, many cases necessitate repeated surgical operations separated by several months. Recommendations from the National Institute for Health and Care Excellence (NICE) advise the performance of this procedure only in very specialized centers, after discussion of the risks and benefits associated with it with the patient.

In some cases, the doctor may opt to perform a debulking surgical operation but without chemotherapy. This will help in reducing symptoms but will not lead to a cure. The surgery might involve the removal of the uterus and ovaries or the resection of bowel. Frequently, many debulking surgeries will have to be performed due to recurrence.

Patients who are not susceptible to surgery may only receive chemotherapy in the form of mitomycin C injections or tablets of capecitabine. These two drugs have a benign side effect profile, although frequent monitoring with blood tests and CT is necessary.

Finally, the doctor may opt to simply monitor and observe if the tumor is slowly growing. Surgery or chemotherapy might become necessary in case symptoms progress and complications develop.

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