Peritoneal Cancer

Back

Practice Essentials

Peritoneal neoplasia can originate de novo from the peritoneal tissues (primary) or invade or metastasize into the peritoneum from adjacent or remote organs (secondary). Primary peritoneal cancers, some of which have been implicated in many cases of carcinomas of unknown primary origin, include ovarian cancer arising in women several years after bilateral oophorectomy. Other described primary peritoneal cancers and tumors include the following:

Signs and symptoms

Primary peritoneal carcinoma usually manifests with abdominal distention and diffuse nonspecific abdominal pain secondary to ascites. This tumor is described almost exclusively in women.

Patients with malignant peritoneal mesothelioma usually manifest with symptoms and signs of advanced disease, including the following:

See Presentation for more detail.

Diagnosis

The workup of peritoneal lesions includes peritoneal lavage cytology, as follows:

Laparoscopy or laparotomy

Studies for malignant peritoneal mesothelioma include the following:

Standard imaging tests, including ultrasonography and helical CT scans, are notably insensitive for the detection of peritoneal tumors. Ultrasonography findings that may suggest the presence of peritoneal lesions include the following:

CT scan findings that suggest primary papillary serous carcinoma of the peritoneum include the following:

CT findings in patients with malignant peritoneal mesotheliomas range from peritoneum-based masses (a so-called "dry" appearance) to ascites, irregular or nodular peritoneal thickening, and an omental mass (a so-called "wet" appearance). Scalloping of the peritoneum or direct invasion of adjacent abdominal organs may also be seen.[1]

Radionuclide scan studies can help confirm the diagnosis of peritoneal hemangiomas; the isotope concentrates in the area where platelets are being sequestered. A CT scan and ultrasound also may detect larger hemangiomas. Angiographic evaluation is a more precise, although invasive, procedure that may be considered when radionuclide scans, CT scans, and ultrasound findings are negative.

See Workup for more detail.

Management

Multimodality therapy is currently the most commonly accepted therapeutic approach for peritoneal mesothelioma. This includes using the combination of the following:

Heated chemotherapeutic drugs used intraoperatively include the following:

For patients with unresectable or recurrent malignant mesothelioma, palliative systemic chemotherapy should be considered. Palliative regimens may include the following:

Primary peritoneal carcinoma is treated with tumor debulking followed by chemotherapy with 5-fluorouracil, doxorubicin, or cisplatin. Newer approaches include the following:

Surgical care

See Treatment and Medication for more detail.

Background

The peritoneum is a serous lining of mesothelial cells with a rich vascular and lymphatic capillary network that covers the abdominal and pelvic walls and organs. Peritoneal neoplasia can originate de novo from the peritoneal tissues (primary) or invade or metastasize into the peritoneum from adjacent or remote organs (secondary).

A number of primary cancers have been described to originate from the peritoneum, some of which have been implicated in many cases of carcinomas of unknown primary origin. Ovarian cancer arising in women several years after bilateral oophorectomy is believed to be one of these primary peritoneal cancers. Other described primary peritoneal cancers and tumors include malignant mesothelioma, benign papillary mesothelioma, desmoplastic small round cell tumors, peritoneal angiosarcoma, leiomyomatosis peritonealis disseminata (LPD), and peritoneal hemangiomatosis.

Pathophysiology

The peritoneal cavity, enclosed by visceral and parietal peritonea, is the largest potential space in the body. Any pathologic process involving the peritoneal cavity can easily disseminate throughout this space by means of unrestricted movement of fluid and cells.

Primary malignant diseases arising from the peritoneal cavity include malignant mesothelioma, cystic mesothelioma, primary peritoneal carcinoma, and desmoplastic small round cell tumor.

Malignant mesothelioma

Malignant peritoneal mesothelioma is a rare but aggressive tumor derived from the peritoneal mesothelium. Although most mesotheliomas involve the pleural surface, 20-30% arise from the peritoneum and are associated with asbestos exposure and abdominal therapeutic radiation. Association of malignant peritoneal mesothelioma and asbestos exposure has been reported to be as high as 83%.

Mesotheliomas are composed of strands of connective tissue covered by cells that react positively to periodic acid-Schiff staining in the cytoplasm. These cells grow in multiple layers, forming papillary or tubular formations. Histologically, malignant mesothelioma is classified into epithelial, sarcomatoid, and mixed. Clinical features of malignant mesothelioma include abdominal pain, abdominal or pelvic masses, and thrombocytosis; ascites is the major factor in the disease morbidity and mortality. Rarely, the tumor spreads into the pleural space.

On CT scan, this neoplasm can appear as peritoneum-based masses or abdominal ascites with associated nodular or diffuse peritoneal thickening.

This locally aggressive disease is difficult to treat or palliate. Commonly, treatment regimens combine aggressive cytoreductive surgery with intraperitoneal chemotherapy. Thorough cytoreductive surgery is the cornerstone of current treatment, while hyperthermic intraoperative intraperitoneal chemotherapy (HIIC) is a promising strategy in suitable patients.[2]

Cystic mesothelioma

Cystic mesothelioma is a rare intermediate-grade tumor with a predilection for surfaces of the pelvis.[3] Typically, these lesions consist of multiple grapelike clusters of mesothelium-lined cysts separated by fibrous tissue. The nomenclature for this entity is confusing, and several synonyms (eg, multilocular peritoneal inclusion cyst, cystic mesothelioma) are used interchangeably in the literature.

This rare tumor commonly occurs in young to middle-aged women and typically presents with abdominal pain, tenderness, or distension.

Radiologic tests demonstrate thin-walled cysts containing watery secretions, easily seen on ultrasound, CT scan, and MRI.[4]

Some authors reported effective intraperitoneal chemotherapy, but no clinical study is available about long-term outcome. The short-term prognosis is favorable, although the tumor recurs in 25–50% of cases.

The differential diagnosis includes lymphangioma, mesenteric-omental cysts, ovarian cystadenoma and cystadenocarcinoma, cystic teratoma, pseudomyxoma peritonei, cystic smooth muscle tumors, visceral cysts, and endometriosis.

Primary peritoneal carcinoma

Primary peritoneal carcinoma (ie, serous surface papillary carcinoma) arises primarily from peritoneal cells. The mesothelium of the peritoneum and the germinal epithelium of the ovary arise from the same embryologic origin; therefore, the peritoneum may retain the multipotentiality allowing the development of a primary carcinoma.

This rare malignancy predominantly affects postmenopausal women and typically displays multicentric peritoneal and omental involvement. Pathologically and clinically, it resembles papillary serous ovarian carcinoma. This malignancy is differentiated from its ovarian counterpart by the fact that it involves the extraovarian peritoneum significantly and the ovarian surface minimally or not at all. Extensive calcification or omental caking is present in many cases and is a useful CT finding to exclude mesothelioma. The absence of an ovarian mass is critical for excluding metastatic papillary serous ovarian carcinoma, which otherwise has a similar CT appearance.

Treatment of this malignancy is very similar to that of epithelial ovarian cancer, which includes combination chemotherapy after optimal cytoreductive surgery.

Desmoplastic small round cell tumor

This tumor is a highly aggressive malignancy that has recently been described. It involves the peritoneal cavity in most cases. Unlike the other primary peritoneal neoplasms, desmoplastic small round cell tumor (DSRCT) most often affects young adults. This malignancy extensively and rapidly invades the peritoneal surfaces with hematogenous metastasis to the liver, lungs, and lymph nodes.

Cytologically, DSRCT is a highly cellular tumor composed of small round cells with granular chromatin, nuclear molding, and inconspicuous nucleoli that are arranged singly and in clusters.[5] This tumor exhibits a unique immunohistochemical profile, characterized by coexpression of epithelial (keratin and epithelial membrane antigen), neural (neuron-specific enolase and CD56), mesenchymal (vimentin), and myogenic (desmin) markers. The reciprocal chromosomal translocation t (11; 22)(p13; q12) is also specific for DSRCT.

Radiological investigation shows multiple rounded peritoneal masses with or without ascites. The omentum and paravesical regions are often involved.

The recommended treatment is a combination of multiagent chemotherapy with adjuvant surgery and radiation. The overall survival for people with this disease is poor despite aggressive treatment.

Other neoplasms

Although clear cell carcinoma is often derived from the ovary and associated with endometriosis, cases of peritoneal origin have been reported. Residual tumor volume appears to determine survival in these patients. These tumors are typically resistant to conventional platinum-based chemotherapy but in one case, adjuvant chemotherapy using irinotecan and cisplatin was effective.[6]

In addition to the above-mentioned primary peritoneal malignancies arising from the peritoneal lining, various types of neoplasms may develop from mesenchymal and lymphatic tissues of the abdominal and pelvic cavities. This includes different forms of sarcomas, histiocytoma,[7] gastrointestinal stromal tumors (GIST), and lymphoproliferative malignancies. Moreover, the differential of peritoneal malignancies includes many benign tumors derived from lymphatic, vascular, neuromuscular, or fatty tissues.

Etiology

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 BRCA1/2 mutations have an increased risk.[8]

Susceptibility to mesothelioma may be influenced by genetic makeup.[9]

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.[9]

Epidemiology

All of the primary peritoneal cancers are rare. Primary peritoneal carcinoma is very uncommon. Peritoneal mesotheliomas are also rare, with 2 cases per 1 million population reported each year. However, the incidence appears to be increasing. Based on the prior use of asbestos, more than 8 million people in the United States were exposed and at risk. Benign cystic peritoneal mesotheliomas are rare.

Primary peritoneal carcinoma is a rare tumor that occurs almost exclusively in women. Malignant mesotheliomas show extreme male predominance (93% in one series). Desmoplastic small round cell tumors occur in adolescent persons and young men. Primary peritoneal carcinoma occurs in older patients than do epithelial ovarian cancers. Benign cystic peritoneal mesotheliomas are rare and are found predominantly in younger women.

Most cases of leiomyomatosis peritonealis disseminata have been discovered in reproductive-aged women (mean age 37 y), in young pregnant women, and in women who have hormonal excess for any other reason. In most reported cases, nodules either regress or exhibit growth once the hormonal stimulation has been removed.

Prognosis

Peritoneal cancers traditionally have been associated with significant morbidity and universal mortality, however, significantly improved long-term survival has been reported in patients with resectable disease and complete cytoreduction.[10]

Survival is poor for patients with primary peritoneal carcinoma, with 100% mortality; the median survival reported is 12-25 months, even with extensive surgery and chemotherapy. Benign cystic peritoneal mesotheliomas are associated with prolonged survival despite bulky disease. Desmoplastic small round cell tumors are associated with a reported median survival of 17 months.

In a multi-institutional data registry that included 405 patients with diffuse malignant peritoneal mesothelioma, overall median survival was 53 months (1 to 235 months), and the 5-year survival rate was 47%. Multivariate analysis showed that the following four prognostic factors were independently associated with improved survival[11] :

History

Primary peritoneal carcinoma usually manifests as 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).

Patients with malignant peritoneal mesothelioma usually present with symptoms and signs of advanced disease, including pain, ascites, weight loss, or an abdominal mass. Diffuse involvement of the peritoneal cavity is typical, often 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.

Laboratory Studies

In malignant peritoneal mesothelioma, findings from cytologic examination of ascites can suggest the diagnosis, and findings from percutaneous biopsy of the omentum can help verify the diagnosis. This condition is usually confined to the abdomen at the time of diagnosis.

Imaging Studies

Standard imaging tests, including ultrasonography and helical computed tomography (CT) scans, are notably insensitive for the detection of peritoneal tumors. CT is limited in detecting small peritoneal metastases. CT can depict tumors larger than 1 cm with sensitivity of 85–93% and specificity of 91–96%, but the sensitivity decreases to 25–50% in detecting implants that are 1 cm or smaller.[15]

Ultrasonography is similarly insensitive; rather than relying on solid tumor detection, therefore, it is important to consider findings that may suggest the presence of peritoneal lesions. These include the presence of ascites, fixing together of bowel loops, thickening of mesentery, and omental matting.

CT scan findings are nonspecific in primary papillary serous carcinoma of the peritoneum. Consider this diagnosis when findings include the following:

Malignant peritoneal mesotheliomas produce CT findings that range from peritoneum-based masses (a so-called "dry" appearance) to ascites, irregular or nodular peritoneal thickening, and an omental mass (a so-called "wet" appearance). Scalloping of the peritoneum or direct invasion of adjacent abdominal organs may also be seen.[1]

Some studies show that magnetic resonance imaging (MRI) is superior to helical CT scan for the detection of peritoneal and bowel wall abnormalities.

Preoperative imaging of the abdomen and pelvis plays an important role in determining the extent of peritoneal disease in patients who are being considered for cytoreductive surgery. Delayed gadolinium-enhanced imaging combined with diffusion-weighted MRI has been shown to be a superior imaging modality to predict which patients will be able to undergo complete primary cytoreductive surgery. In one study, MRI more accurately predicted peritoneal cancer index (PCI) preoperatively in patients undergoing evaluation for cytoreductive surgery than CT. PCI measures the size and extent of peritoneal tumor at laparotomy.  For predicting resectability, MRI had sensitivity of 95%, specificity of 70%, and accuracy of 88% compared with 55%, 86%, and 63%, respectively, for CT.[16]

Positron emission tomography (PET) imaging has not been shown to be sensitive for lesions smaller than 1 cm in the abdominal cavity.

Dual-time point imaging after carbonated water may increase the accuracy of 18F-fluorodeoxyglucose (FDG) PET/CT for the imaging of peritoneal cancer in patients affected by colorectal cancer.[17]

Findings from radionuclide scan studies can help confirm the diagnosis of peritoneal hemangiomas; the isotope concentrates in the area where platelets are being sequestered. A CT scan and ultrasound also may detect larger hemangiomas. Angiographic evaluation is a more precise, although invasive, procedure that may be considered when radionuclide scans, CT scan, and ultrasound findings are negative.

Procedures

The workup of peritoneal lesions includes peritoneal lavage cytology. Peritoneal lavage can be performed using a percutaneous closed technique or at the time of laparoscopy or laparotomy. The sensitivity of the test results depends on the ability to completely lavage all regions of the peritoneal cavity and the ability to detect cancer cells being shed into the peritoneal cavity by the tumor.

Direct visualization of the peritoneal surfaces along with palpation of the abdominal contents is by far the most sensitive modality for detecting peritoneal cancer. This can be accomplished with a minimally invasive approach (ie, laparoscopy), which allows for safe, directed peritoneal lavage for cytology, or with open abdominal exploration and palpation of the peritoneal surfaces. Open abdominal exploration and palpation are extremely sensitive for 1- to 2-mm peritoneal nodules.

Histologic Findings

Primary peritoneal carcinoma is histologically indistinguishable from primary epithelial ovarian carcinoma; however, primary ovarian cancer can be excluded when the following criteria are present:

Medical Care

Multimodality therapy is currently the most commonly accepted therapeutic approach to peritoneal mesothelioma. This includes using the combination of surgical cytoreduction, intraperitoneal perioperative chemotherapy, and hyperthermia. Intraperitoneal chemotherapy greatly enhances drug concentrations in the peritoneal cavity and decreases its systemic toxicity. See the videos below.



View Video

Dr. Oliver Zivanovic, MD, PhD, discusses the role of hyperthermic intraperitoneal chemotherapy in ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.



View Video

Dr. Oliver Zivanovic, MD, PhD, demonstrates hyperthermic intraperitoneal chemotherapy for ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.

In addition to the intraoperative use of heated chemotherapeutic drugs such as cisplatin, mitomycin, or doxorubicin, newer techniques include adding immunotherapeutic agents such as interleukins and interferons. While the median survival with traditional therapeutic options ranges from 4 to 12 months, the application of multimodality therapy has shown promising results with increased survival approaching 60 months.[18]

For patients with unresectable or recurrent malignant mesothelioma, palliative systemic chemotherapy (with different regimens such as cisplatin plus pemetrexed) should be considered. Other antineoplastic agents that may be used include cisplatin plus paclitaxel or mitomycin, doxorubicin, and irinotecan.

Primary peritoneal carcinoma is treated similarly to ovarian cancer, with cytoreduction and chemotherapy. Multimodality treatment consisting of tumor debulking followed by chemotherapy regimens based on 5-fluorouracil, doxorubicin, or cisplatin has been shown to have high response rate and improvement of median survival. Furthermore, the use of newer antineoplastic agents such as taxanes, topoisomerase I inhibitors, gemcitabine, and vinorelbine, alone or in combinations (eg, gemcitabine/cisplatin, irinotecan/cisplatin, docetaxel/gemcitabine, gemcitabine/carboplatin) has increased median survival to 8-11 months. In addition to the use of chemotherapeutic agents, recent studies have shown some benefit of antiangiogenic drugs such as bevacizumab and erlotinib.

In November 2014, the FDA approved bevacizumab (Avastin) for platinum-resistant, recurrent, epithelial ovarian cancer in patients who received no more than two prior chemotherapy regimens. It is indicated for use in combination with paclitaxel, pegylated liposomal doxorubicin, or topotecan.[19] In December 2016, bevacizumab’s indication was expanded to include treatment of platinum-sensitive recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer, in combination with carboplatin and either paclitaxel or gemcitabine, followed by bevacizumab alone.[20, 21]

It is generally accepted that all patients with good performance status should be considered for a trial of empiric chemotherapy, preferably with a regimen containing newer drugs. The combination of paclitaxel and carboplatin is a reasonable first-line therapy with the addition of a third agent (etoposide or gemcitabine) providing possible benefit. Supportive measures should be considered in patients with poor performance status.

Surgical Care

Treatment of primary peritoneal carcinoma consists of total abdominal hysterectomy and bilateral salpingo-oophorectomy as needed, with debulking of tumor and follow-up chemotherapy. 

Cytoreductive surgery (CRS) is the removal of macroscopic peritoneal tumors. It may involve multiple peritonectomy and visceral resection procedures. Electrosurgery is used to limit bleeding from the vascular peritoneal surface.[10]

Treatment of malignant peritoneal mesothelioma consists primarily of surgical palliation. Complete surgical resection is rarely, if ever, feasible and has not been shown to afford a survival benefit in the absence of additional therapy. If laparoscopy is used to help make the initial diagnosis, confine port sites to the abdominal midline because port site recurrence has been described, requiring extensive abdominal wall resection.[22]

Benign cystic mesothelioma tends to recur even with aggressive surgical removal; however, among recorded cases, no deaths have been attributable to this disorder.

In patients with desmoplastic small cell tumors, the combination of aggressive surgical debulking and systemic chemotherapy with cyclophosphamide, doxorubicin, and vincristine interspersed with ifosfamide, etoposide, and mesna (P6 protocol) appears to lead to an improved outcome. However, surgical excision is recommended only for nonmetastatic disease.[23]

Treatment of peritoneal and GI hemangiomas has involved surgical removal.

Long-Term Monitoring

Follow up to evaluate patients for complications of the cancer, spread of the cancer, and possible complications of therapy. Screening for known associated cancers and cancer syndromes is essential.

Medication Summary

The goals of pharmacotherapy are to induce remission, to prevent complications, and to reduce morbidity. The current approach favors intraoperative use of heated chemotherapeutic drugs. A variety of chemotherapeutic and antiangiogenic drugs is used.

Cisplatin (Platinol)

Clinical Context:  Inhibits DNA synthesis and thus cell proliferation by causing DNA cross-links and denaturation of double helix.

Doxorubicin (Adriamycin, Rubex)

Clinical Context:  Inhibits topoisomerase II and produces free radicals, which may cause the destruction of DNA. Combination of these events can, in turn, inhibit the growth of neoplastic cells.

Carboplatin (Paraplatin)

Clinical Context:  Analog of cisplatin. Has same efficacy as cisplatin but with better toxicity profile.

Dose is based on following formula:

Total dose (mg) = (target AUC) X (GFR = 25), where AUC (area under plasma concentration-time curve) is expressed in mg/mL/min and GFR (glomerular filtration rate) is expressed in mL/min.

Cyclophosphamide (Neosar, Cytoxan)

Clinical Context:  Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Paclitaxel (Taxol)

Clinical Context:  Mechanisms of action are tubulin polymerization and microtubule stabilization.

Bevacizumab (Avastin)

Clinical Context:  Bevacizumab is a recombinant humanized monoclonal antibody to vascular endothelial growth factor (VEGF) receptors. Blocking the angiogenic VEGF receptor, in turn inhibits tumor angiogenesis, starving tumor of blood and nutrients. It is indicated in combination with paclitaxel, pegylated liposomal doxorubicin, or topotecan for platinum-resistant recurrent epithelial ovarian cancer in patients who received no more than 2 prior chemotherapy regimens. It is also indicated for women with platinum-sensitive recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer either in combination with carboplatin and paclitaxel or in combination with carboplatin and gemcitabine chemotherapy, followed by bevacizumab alone.

Class Summary

These drugs inhibit cell growth and proliferation.

Author

Wissam Bleibel, MD, Staff Physician, Department of Internal Medicine, Caritas Carney Hospital / Tufts University School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Asif Mahmood, MD, Research Associate, Medical College of Virginia Cancer Center

Disclosure: Nothing to disclose.

Olga Kozyreva, MD, Attending Physician, Division of Hematology-Oncology, St Elizabeth's Medical Center; Assistant Professor, Tufts University School of Medicine

Disclosure: Nothing to disclose.

Sarah K May, MD, Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Benjamin Movsas, MD,

Disclosure: Nothing to disclose.

Chief Editor

N Joseph Espat, MD, MS, FACS, Harold J Wanebo Professor of Surgery, Assistant Dean of Clinical Affairs, Boston University School of Medicine; Chairman, Department of Surgery, Director, Adele R Decof Cancer Center, Roger Williams Medical Center

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP, Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Disclosure: Nothing to disclose.

References

  1. Park JY, Kim KW, Kwon HJ, Park MS, Kwon GY, Jun SY, et al. Peritoneal mesotheliomas: clinicopathologic features, CT findings, and differential diagnosis. AJR Am J Roentgenol. 2008 Sep. 191(3):814-25. [View Abstract]
  2. Deraco M, Casali P, Inglese MG, Baratti D, Pennacchioli E, Bertulli R, et al. Peritoneal mesothelioma treated by induction chemotherapy, cytoreductive surgery, and intraperitoneal hyperthermic perfusion. J Surg Oncol. 2003 Jul. 83(3):147-53. [View Abstract]
  3. Szollosi A, Ferenc C, Pinter T, Erenyi A, Nagy A. [Benign cystic mesothelioma, a rare tumor of the peritoneum]. Magy Seb. 2005 Feb. 58(1):35-7. [View Abstract]
  4. Wong WL, Johns TA, Herlihy WG, Martin HL. Best cases from the AFIP: multicystic mesothelioma. Radiographics. 2004 Jan-Feb. 24(1):247-50. [View Abstract]
  5. Chang F. Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features. Arch Pathol Lab Med. 2006 May. 130(5):728-32. [View Abstract]
  6. Takano M, Yoshokawa T, Kato M, Aida S, Goto T, Furuya K, et al. Primary clear cell carcinoma of the peritoneum: report of two cases and a review of the literature. Eur J Gynaecol Oncol. 2009. 30(5):575-8. [View Abstract]
  7. Bodner K, Bodner-Adler B, Mayerhofer S, Grunberger W, Wierrani F, Czerwenka K, et al. Malignant fibrous histiocytoma (MFH) of the mesentery: a case report. Anticancer Res. 2002 Mar-Apr. 22(2B):1169-70. [View Abstract]
  8. Sakamoto I, Hirotsu Y, Nakagomi H, Ouchi H, Ikegami A, Teramoto K, et al. BRCA1 and BRCA2 mutations in Japanese patients with ovarian, fallopian tube, and primary peritoneal cancer. Cancer. 2016 Jan 1. 122 (1):84-90. [View Abstract]
  9. Rudd RM. Malignant mesothelioma. Br Med Bull. 2010 Jan 4. [View Abstract]
  10. Aherne EA, Fenlon HM, Shields CJ, Mulsow JJ, Cronin CG. What the Radiologist Should Know About Treatment of Peritoneal Malignancy. AJR Am J Roentgenol. 2017 Mar. 208 (3):531-543. [View Abstract]
  11. Yan TD, Deraco M, Baratti D, Kusamura S, Elias D, Glehen O, et al. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for malignant peritoneal mesothelioma: multi-institutional experience. J Clin Oncol. 2009 Dec 20. 27(36):6237-42. [View Abstract]
  12. Sebbag G, Shmookler BM, Chang D, Sugarbaker PH. Peritoneal carcinomatosis from an unknown primary site. Management of 15 patients. Tumori. 2001 Mar-Apr. 87(2):67-73. [View Abstract]
  13. Pentheroudakis G, Pavlidis N. Serous papillary peritoneal carcinoma: Unknown primary tumour, ovarian cancer counterpart or a distinct entity? A systematic review. Crit Rev Oncol Hematol. 2009 Nov 6. [View Abstract]
  14. Clement PB. Reactive tumor-like lesions of the peritoneum. Am J Clin Pathol. 1995 Jun. 103(6):673-6. [View Abstract]
  15. Javadi S, Ganeshan DM, Qayyum A, Iyer RB, Bhosale P. Ovarian Cancer, the Revised FIGO Staging System, and the Role of Imaging. AJR Am J Roentgenol. 2016 Jun. 206 (6):1351-60. [View Abstract]
  16. Low RN, Barone RM, Lucero J. Comparison of MRI and CT for predicting the Peritoneal Cancer Index (PCI) preoperatively in patients being considered for cytoreductive surgical procedures. Ann Surg Oncol. 2015 May. 22 (5):1708-15. [View Abstract]
  17. Filippi L, D'Arienzo M, Scopinaro F, Salvatori R, Bagni O. Usefulness of Dual-Time Point Imaging After Carbonated Water for the Fluorodeoxyglucose Positron Emission Imaging of Peritoneal Carcinomatosis in Colon Cancer. Cancer Biother Radiopharm. 2012 Nov 7. [View Abstract]
  18. McConnell YJ, Mack LA, Francis WP, Ho T, Temple WJ. HIPEC + EPIC versus HIPEC-alone: Differences in major complications following cytoreduction surgery for peritoneal malignancy. J Surg Oncol. 2012 Nov 5. [View Abstract]
  19. Pujade-Lauraine E, Hilpert F, Weber B, Reuss A, Poveda A, Kristensen G, et al. Bevacizumab combined with chemotherapy for platinum-resistant recurrent ovarian cancer: The AURELIA open-label randomized phase III trial. J Clin Oncol. 2014 May 1. 32 (13):1302-8. [View Abstract]
  20. Coleman RL, Brady RF, Herzog TJ, et al. A phase III randomized controlled clinical trial of carboplatin and paclitaxel alone or in combination with bevacizumab followed by bevacizumab and secondary cytoreductive surgery in platinum-sensitive, recurrent ovarian, peritoneal primary and fallopian tube cancer. Presented at: Society of Gynecologic Oncology’s Annual Meeting on Women’s Cancer 2015; March 28-31, 2015; Chicago, IL. Abstract 3.
  21. Aghajanian C, Goff B, Nycum LR, Wang YV, Husain A, Blank SV. Final overall survival and safety analysis of OCEANS, a phase 3 trial of chemotherapy with or without bevacizumab in patients with platinum-sensitive recurrent ovarian cancer. Gynecol Oncol. 2015 Oct. 139 (1):10-6. [View Abstract]
  22. Iversen LH, Rasmussen PC, Laurberg S. Value of laparoscopy before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal carcinomatosis. Br J Surg. 2012 Nov 2. [View Abstract]
  23. Stuart-Buttle CE, Smart CJ, Pritchard S, Martin D, Welch IM. Desmoplastic small round cell tumour: a review of literature and treatment options. Surg Oncol. 2008 Aug. 17(2):107-12. [View Abstract]
  24. Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE. Clinical Oncology. 2nd ed. Philadelphia, Pa: Churchill Livingstone; 2000.
  25. Al Balushi Z, Bulduc S, Mulleur C, Lallier M. Desmoplastic small round cell tumor in children: a new therapeutic approach. J Pediatr Surg. 2009 May. 44(5):949-52. [View Abstract]
  26. Bani-Hani KE, Gharaibeh KA. Malignant peritoneal mesothelioma. J Surg Oncol. 2005 Jul 1. 91(1):17-25. [View Abstract]
  27. Barclay L. Benefit From CS/HIPEC in Advanced Abdominal Cancers. Medscape Medical News. Available at http://www.medscape.com/viewarticle/806289. Accessed: July 15, 2013.
  28. Biswas G, Laskar S, Banavali SD, Gujral S, Kurkure PA, Muckaden M, et al. Desmoplastic small round cell tumor: extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer. 2005 Apr-Jun. 42(2):78-84. [View Abstract]
  29. Brigand C, Monneuse O, Mohamed F, Sayag-Beaujard AC, Isaac S, Gilly FN, et al. Peritoneal mesothelioma treated by cytoreductive surgery and intraperitoneal hyperthermic chemotherapy: results of a prospective study. Ann Surg Oncol. 2006 Mar. 13(3):405-12. Epub 2006 Jan 30. [View Abstract]
  30. Chouli M, Viala J, Dromain C, Fizazi K, Duvillard P, Vanel D. Intra-abdominal desmoplastic small round cell tumors: CT findings and clinicopathological correlations in 13 cases. Eur J Radiol. 2005 Jun. 54(3):438-42. [View Abstract]
  31. Cormio G, Di Vagno G, Di Gesu G, Mastroianni M, Melilli GA, Vimercati A, et al. Primary peritoneal carcinoma: a report of twelve cases and a review of the literature. Gynecol Obstet Invest. 2000. 50(3):203-6. [View Abstract]
  32. Devita VT, Hellman S, Rosenthal SA. Cancer. Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins (LWW); 2005.
  33. Gómez Portilla A, Cendoya I, Muriel J, Olabarria I, Guede N, Moraza N, et al. [Malignant peritoneal mesothelioma. Our experienced with triple combined therapy: cytoreduction, intraperitoneal perioperative chemotherapy and hyperthermia]. Cir Esp. 2007 Feb. 81(2):82-6. [View Abstract]
  34. Haslinger M, Francescutti V, Attwood K, et al. A contemporary analysis of morbidity and outcomes in cytoreduction/hyperthermic intraperitoneal chemoperfusion. Cancer Med. 2013 Jun;2(3):334-42.
  35. Jaaback KS, Ludeman L, Clayton NL, Hirschowitz L. Primary peritoneal carcinoma in a UK cancer center: comparison with advanced ovarian carcinoma over a 5-year period. Int J Gynecol Cancer. 2006 Jan-Feb. 16 Suppl 1:123-8. [View Abstract]
  36. Kim HC, Lee JM, Kim SH, Kim KW, Lee M, Kim YJ, et al. Primary gastrointestinal stromal tumors in the omentum and mesentery: CT findings and pathologic correlations. AJR Am J Roentgenol. 2004 Jun. 182(6):1463-7. [View Abstract]
  37. Ko SF, Chou FF, Huang CH, Ng SH, Wan YL, Lee TY, et al. Primary synovial sarcoma of the gastrocolic ligament. Br J Radiol. 1998 Apr. 71(844):438-40. [View Abstract]
  38. Mohamed F, Sugarbaker PH. Peritoneal mesothelioma. Curr Treat Options Oncol. 2002 Oct. 3(5):375-86. [View Abstract]
  39. Nam JH, Kim YM, Jung MH, Kim KR, Yoo HJ, Kim DY, et al. Primary peritoneal carcinoma: experience with cytoreductive surgery and combination chemotherapy. Int J Gynecol Cancer. 2006 Jan-Feb. 16(1):23-8. [View Abstract]
  40. Ordóñez NG. Pathologic characterization and differential diagnosis of malignant peritoneal mesothelioma. Recent Results Cancer Res. 2007. 169:123-36. [View Abstract]
  41. Papadatos D, Taourel P, Bret PM. CT of leiomyomatosis peritonealis disseminata mimicking peritoneal carcinomatosis. AJR Am J Roentgenol. 1996 Aug. 167(2):475-6. [View Abstract]
  42. Pavlidis N, Briasoulis E, Hainsworth J, Greco FA. Diagnostic and therapeutic management of cancer of an unknown primary. Eur J Cancer. 2003 Sep. 39(14):1990-2005. [View Abstract]
  43. Pickhardt PJ, Bhalla S. Primary neoplasms of peritoneal and sub-peritoneal origin: CT findings. Radiographics. 2005 Jul-Aug. 25(4):983-95. [View Abstract]
  44. Pickhardt PJ, Bhalla S. Unusual nonneoplastic peritoneal and subperitoneal conditions: CT findings. Radiographics. 2005 May-Jun. 25(3):719-30. [View Abstract]
  45. Popovska S, Veselinova T, Gorchev G, Tomov S, Elenchev L. [Primary peritoneal carcinoma: a report of two cases and a review of the literature]. Akush Ginekol (Sofiia). 2005. 44(1):8-10. [View Abstract]
  46. Shen P, Levine EA, Hall J, Case D, Russell G, Fleming R, et al. Factors predicting survival after intraperitoneal hyperthermic chemotherapy with mitomycin C after cytoreductive surgery for patients with peritoneal carcinomatosis. Arch Surg. 2003 Jan. 138(1):26-33. [View Abstract]
  47. Stafford-Johnson DB, Bree RL, Francis IR, Korobkin M. CT appearance of primary papillary serous carcinoma of the peritoneum. AJR Am J Roentgenol. 1998 Sep. 171(3):687-9. [View Abstract]
  48. Sugarbaker PH, Acherman YI, Gonzalez-Moreno S, Ortega-Perez G, Stuart OA, Marchettini P, et al. Diagnosis and treatment of peritoneal mesothelioma: The Washington Cancer Institute experience. Semin Oncol. 2002 Feb. 29(1):51-61. [View Abstract]
  49. Suh-Burgmann E, Powell CB. Cytoreductive surgery for gynecologic malignancies-new standards of care. Surg Oncol Clin N Am. 2007 Jul. 16(3):667-82. [View Abstract]

Dr. Oliver Zivanovic, MD, PhD, discusses the role of hyperthermic intraperitoneal chemotherapy in ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.

Dr. Oliver Zivanovic, MD, PhD, demonstrates hyperthermic intraperitoneal chemotherapy for ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.

Dr. Oliver Zivanovic, MD, PhD, discusses the role of hyperthermic intraperitoneal chemotherapy in ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.

Dr. Oliver Zivanovic, MD, PhD, demonstrates hyperthermic intraperitoneal chemotherapy for ovarian cancer. Courtesy of Memorial Sloan-Kettering Cancer Center.