Peritoneal carcinomatosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: Peritoneal metastases; Peritoneal seeding
Overview
Peritoneal carcinomatosis (also known as peritoneal metastases) is defined as a malignant tumoral seeding of the peritoneum. Peritoneal carcinomatoses are the most common peritoneal malignancy, these commonly arise from ovarian cancer, colon cancer, gastric cancer, and pancreatic cancer. Calcified peritoneal carcinomatosis may occur in serous ovarian adenocarcinoma, colon cancer, and gastric cancer.
Historical Perspective
- Peritoneal carcinomatosis was first described by Swerdlow in 1959.[1]
Classification
- According to the Gilly classification, peritoneal carcinomatosis may be classified according to nodule size and intraperitoneal involvement (localized or diffuse) into 4 categories:[2]
- 0 No macroscopic disease
- 1 Malignant granulations less than 5 mm in diameter localized in one part of the abdomen
- 2 Malignant granulations less than 5 mm in diameter diffuse to the whole abdomen
- 3 Localized or diffuse malignant granulations 5–20 mm in diameter
- 4 Localized or diffuse large malignant masses (more than 2 cm in diameter)
Pathophysiology
- The pathogenesis of peritoneal carcinomatosis is characterized by the malignant seeding of a tumor in the peritoneal cavity.[3]
- Peritoneal carcinogenesis arises from the celomic epithelium lining of the abdominal cavity (peritoneum) in response to an oncogenic stimulus.[3]
- The mutation on BRCA1/BRCA2 has been associated with the development of peritoneal carcinomatosis.
- On gross pathology, characteristic findings of peritoneal carcinomatosis, include:[4]
- Multilocular thin-walled cysts containing serous fluid
- Occasionally unilocular
- May be up to 15 cm
- Adherent to the surface
- On microscopic histopathological analysis, characteristic findings of peritoneal carcinomatosis, include:[4]
- Thin-walled, irregular-shaped cysts
- Mesothelial lining
- Squamous metaplasia
- Eosinophilic fluid
Causes
- Common causes of peritoneal carcinomatosis, include:[4]
- Peritoneal mesothelioma
- Colon cancer
- Gastric cancer
- Pancreatic cancer
- Peritoneal carcinomatosis may also be caused by a mutation in the BCRA1 or BCRA2 genes.
Differentiating Peritoneal Carcinomatosis from Other Diseases
- Peritoneal carcinomatosis must be differentiated from other diseases that cause abdominal pain, ascites, and weight loss, such as:[4]
Epidemiology and Demographics
- The prevalence of peritoneal carcinomatosis is approximately 0.03 per 100,000 individuals worldwide.[5]
Age
- Peritoneal carcinomatosis is more commonly observed among patients aged 50 - 70 years.[6]
- Peritoneal carcinomatosis is more commonly observed among adults.[7]
Gender
- Females are more commonly affected with peritoneal carcinomatosis than males.[7]
Race
- There is no racial predilection for peritoneal carcinomatosis.[7]
Risk Factors
- The most important risk factor in the development of peritoneal carcinomatosis is the presence of ovarian cancer, colon cancer, gastric cancer, and pancreatic cancer.
Natural History, Complications and Prognosis
- The majority of patients with peritoneal carcinomatosis may be initially asymptomatic.
- Early clinical features include abdominal pain, abdominal distension, and nausea.
- If left untreated, the majority of patients with peritoneal carcinomatosis may progress to develop portal hypertension, pulmonary edema, and death.
- Common complications of peritoneal carcinomatosis include intestinal obstruction and pulmonary thromboembolism.
- Prognosis is generally poor, and the 5 year survival rate of patients with peritoneal carcinomatosis is approximately.
Diagnosis
Diagnostic Criteria
- The diagnosis of peritoneal carcinomatosis is made when at least the following diagnostic criteria are met:[6]
- Imaging findings compatible with peritoneal carcinomatosis (see below)
- Elevated protein concentration (more than 4.0 g/dL)
- Abnormal serum-ascites albumin gradient (less than 1.1 g/dL)
- High cell count (lymphocyte predominance)
Symptoms
- Peritoneal carcinomatosis is usually asymptomatic.
- Symptoms of peritoneal carcinomatosis may include the following:
Physical Examination
- Patients with peritoneal carcinomatosis usually appear pale and lethargic.
- Physical examination may be remarkable for:
Inspection
- Enlarged abdomen
- Abdominal distension
Palpation
- Bulging of the flanks or shifting dullness
- Fluid thrill or fluid wave
- Other physical examination findings may include:
Laboratory Findings
- Laboratory findings consistent with the diagnosis of peritoneal carcinomatosis, include:
- Elevated carcinoembryonic antigen (unspecific)
- Elevated cancer antigen 125 (unspecific)
- Elevated cancer antigen 19-9 (unspecific)
Imaging Findings
- Enhanced CT scan is the imaging modality of choice for peritoneal carcinomatosis.[6]
- On CT, peritoneal carcinomatosis is characterized by the following findings:[4]
- Smooth or nodular peritoneal thickening and enhancement.
- Implants on the liver and the splenic surfaces are frequently seen and result in scalloping of the surface by the masses.
- Sites of tumor implantation are the intersegmental fissure, superior recess of the lesser sac, subphrenic space, and Morison pouch.
- Usually there is large ascites, which is often loculated.
- The images below demonstrate a case of peritoneal carcinomatosis.
Other Diagnostic Studies
- Peritoneal carcinomatosis may also be diagnosed using abdominal paracentesis.
- Findings on paracentesis may include:
- Opalescent appearance
- Elevated protein concentration (more than 4.0 g/dL)
- Abnormal serum-ascites albumin gradient ( less than 1.1 g/dL )
- Other diagnostic studies, include: positron emission tomography (PET)/PET-CT, diagnostic laparoscopy, ultrasonography, magnetic resonance imaging (MRI)
Treatment
Medical Therapy
- The mainstay medical therapy for peritoneal carcinomatosis is hyperthermic intraperitoneal chemotherapy (HIPEC).[8]
- Common chemotherapy agents, include:[8]
- Mitomycin
- Cisplatin
- Oxaliplatin
- Doxorubicin
- Mitoxantrone
Surgery
- Cytoreductive surgery is the mainstay of therapy for peritoneal carcinomatosis.[8]
- Laparotomy in conjunction with cytology testing is the most common approach to the treatment of peritoneal carcinomatosis.
- Cytoreductive surgery is composed of three steps: exploration of the abdominal cavity, debulking and chemoperfusion.
Prevention
- There are no primary preventive measures available for peritoneal carcinomatosis.
- Once diagnosed and successfully treated, patients with peritoneal carcinomatosis are followed-up every 1, 3 or 6 months.
- Follow-up testing includes ultrasound, paracentesis, and abdominal examination.[6]
References
- ↑ Swerdlow M: Mesothelioma of the pelvic peritoneum resembling papillary cystadenocarcinoma of the ovary: Case report. Am J Obstet Gynecol 77:200, 1959.
- ↑ Kianmanesh R, Ruszniewski P, Rindi G, Kwekkeboom D, Pape UF, Kulke M, Sevilla Garcia I, Scoazec JY, Nilsson O, Fazio N, Lesurtel M, Chen YJ, Eriksson B, Cioppi F, O'Toole D (2010). "ENETS consensus guidelines for the management of peritoneal carcinomatosis from neuroendocrine tumors". Neuroendocrinology. 91 (4): 333–40. doi:10.1159/000286700. PMID 20424420.
- ↑ 3.0 3.1 Deraco M, Santoro N, Carraro O, Inglese MG, Rebuffoni G, Guadagni S, Somers DC, Vaglini M (1999). "Peritoneal carcinomatosis: feature of dissemination. A review". Tumori. 85 (1): 1–5. PMID 10228488.
- ↑ 4.0 4.1 4.2 4.3 4.4 Peritoneum. Libre patholgy. https://librepathology.org/wiki/Peritoneum Accessed on April 7, 2016
- ↑ Segelman J, Granath F, Holm T, Machado M, Mahteme H, Martling A (2012). "Incidence, prevalence and risk factors for peritoneal carcinomatosis from colorectal cancer". Br J Surg. 99 (5): 699–705. doi:10.1002/bjs.8679. PMID 22287157.
- ↑ 6.0 6.1 6.2 6.3 Villanueva A, Pérez C, Sabaté JM, Llauger J, Giménez A, Sanchis E, García T, Moreno A (1995). "[Peritoneal carcinomatosis. Review of CT findings in 107 cases]". Rev Esp Enferm Dig (in Spanish; Castilian). 87 (10): 707–14. PMID 8519536.
- ↑ 7.0 7.1 7.2 Kusamura S, Baratti D, Zaffaroni N, Villa R, Laterza B, Balestra MR, Deraco M (2010). "Pathophysiology and biology of peritoneal carcinomatosis". World J Gastrointest Oncol. 2 (1): 12–8. doi:10.4251/wjgo.v2.i1.12. PMC 2999153. PMID 21160812.
- ↑ 8.0 8.1 8.2 Glockzin G, Schlitt HJ, Piso P (2009). "Peritoneal carcinomatosis: patients selection, perioperative complications and quality of life related to cytoreductive surgery and hyperthermic intraperitoneal chemotherapy". World J Surg Oncol. 7: 5. doi:10.1186/1477-7819-7-5. PMC 2639355. PMID 19133112.