Peritoneal Cancer Clinical Presentation
- Author: Wissam Bleibel, MD; Chief Editor: N Joseph Espat, MD, MS, FACS more...
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- Primary peritoneal carcinoma usually manifests with abdominal distention and diffuse nonspecific abdominal pain secondary to ascites. This tumor is described almost exclusively in women. Atypical presentations of primary peritoneal carcinoma have been described, including a case of severe glandular dysplasia on a screening Papanicolaou test (Pap smear).
- Malignant peritoneal mesothelioma usually manifests with symptoms and signs of advanced disease, including pain, ascites, weight loss, or an abdominal mass.
- These tumors tend to manifest with diffuse involvement of the peritoneal cavity, including omental caking and diaphragmatic and pelvic tumor deposits.
- Thrombocytosis is common and is associated with a poor prognosis.
- Other common clotting abnormalities include phlebitis, emboli, hemolytic anemia, and disseminated intravascular coagulation.
- Esophageal achalasia, secondary amyloidosis, and dermatomyositis have been reported.
- Most patients die without metastasis or involvement of the chest.
- Desmoplastic small round cell tumors occur typically in young patients and manifest with extensive involvement of the peritoneal surfaces. Rapid multifocal growth and hematogenous metastasis to the liver, lungs, and lymph nodes are common.
- Leiomyomatosis peritonealis disseminata is found most commonly in women of reproductive age who are pregnant; these patients are usually asymptomatic, have a long-term history of oral contraceptive use, or have uterine leiomyomas at the time of diagnosis. All cases of this disease have been discovered intraoperatively during obstetric and gynecologic surgical procedures.
- Peritoneal hemangiomas are usually associated with hemangiomas of the GI tract. They are rare and can manifest with ascites, anemia (from chronic blood loss), thrombocytopenia, and coagulopathy.
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- A chromosomal translocation, which results in the fusion of the Ewing sarcoma gene with the Wilms tumor gene, has been identified and implicated in desmoplastic small round cell tumors.
- Hereditary predisposition may play a role in primary peritoneal carcinoma; patients with the BRCA1 mutation have an increased risk.
- Susceptibility to mesothelioma may be influenced by genetic makeup.
- Although conventional wisdom dictates that asbestos is the environmental factor most commonly associated with mesothelioma, asbestos does not transform human mesothelial cells in tissue culture. This suggests that additional carcinogens act in concert with asbestos to cause mesothelioma. Simian virus 40 has been proposed as an etiologic agent; however, the evidence for this hypothesis is weakening.
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