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Pseudomyxoma peritonei is a clinicopathologic entity characterized by the production of mucinous ascites and mostly originates from epithelial neoplasms of the appendix. As the tumor grows, the narrow lumen of the appendix becomes obstructed and subsequently leads to appendiceal perforation. The neoplastic cells progressively colonize the peritoneal cavity and copious mucin production builds up in the peritoneal cavity. Appendix tumors causing pseudomyxoma peritonei range from a benign pathologic appearance (disseminated peritoneal adenomucinosis), to malignant pathologic findings (peritoneal mucinous carcinomatosis), with some intermediate pathologic grades. Clinically, this syndrome ranges from early pseudomyxoma peritonei fortuitously discovered on imaging or during a laparotomy performed for another reason, to advanced cases with a distended abdomen, bowel obstruction, and starvation. The conventional treatment of pseudomyxoma peritonei is surgical debulking repeated as necessary to alleviate pressure effects. However, repeated debulking surgeries become ever more difficult due to progressively thickened intra-abdominal adhesions, and this treatment is palliative, leaving visible or occult disease in the peritoneal cavity.

Peritoneal dissemination develops in approximately 10–15% of patients with colon cancer, and despite the use of increasingly effective regimens of chemotherapy and biologic agents in the treatment of advanced disease, peritoneal metastases are associated with a median survival of 6 to 7 months.

Mesothelioma

Malignant mesothelioma is a relatively uncommon malignancy that may arise from the mesothelial cells lining the pleura, peritoneum, pericardium, and tunica vaginalis testis. In the U.S., 200-400 new cases of diffuse malignant peritoneal mesothelioma (DMPM) are registered every year, accounting for 10-30% of all-type mesothelioma. DMPM has traditionally been considered as a rapidly lethal malignancy with limited and ineffective therapeutic options. The disease is usually diagnosed at an advanced stage and is characterized by multiple variably sized nodules throughout the abdominal cavity. As the disease progresses, the nodules become confluent to form plaques, masses, or uniformly cover peritoneal surfaces. In most patients, death eventually occurs as a result of locoregional progression within the abdominal cavity. In historical case series, treatment by palliative surgery, systemic/intraperitoneal chemotherapy, and abdominal irradiation results in a median survival of approximately 12 months.

Surgical cytoreduction in conjunction with hyperthermic intraperitoneal chemotherapy has been proposed for these patients. The cytoreduction is designed to remove visible tumor deposits, and the intraperitoneal chemotherapy is designed to address remaining microscopic disease. By delivering chemotherapy intraperitoneally, drug exposure to the peritoneal surface is increased some 20-fold compared to systemic exposure. In addition, prior animal and in vitro studies have suggested that the cytotoxicity of mitomycin C is enhanced at temperatures greater than 39 degrees Celsius.

Cytoreductive surgery (CRS) consists of peritonectomy procedures and multivisceral resections, depending on the extent of intra-abdominal tumor dissemination. The surgical procedure may be followed intraoperatively by the infusion of hyperthermic chemotherapy, most commonly mitomycin C. Inflow and outflow catheters are placed in the abdominal cavity, along with temperature probes to monitor the temperature. The skin is then temporarily closed during the chemotherapy perfusion, which typically runs for 1 to 2 hours. This procedure is referred to as hyperthermic intraperitoneal chemotherapy (HIPEC). Other methods of intraperitoneal chemotherapy include early postoperative intraperitoneal chemotherapy (EPIC).

Hyperthermia Therapy is addressed in a separate policy.

Cytoreductive surgery and perioperative intraperitoneal chemotherapy for the treatment of pseudomyxoma peritonei may be considered medically necessary.

Cytoreduction surgery and perioperative intraperitoneal chemotherapy is considered investigational for peritoneal carcinomatosis from colorectal cancer.

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

7/6/2009: Policy reviewed, no changes

12/30/2010:  Added  “Pseudomyxoma Peritonei” to the policy title. Policy description updated regarding disease prevalence and treatment approaches. Policy statement added to indicate that cytoreduction and hyperthermic intraperitoneal chemotherapy for the treatment of pseudomyxoma peritonei may be considered medically necessary; investigational policy statement clarified to specify that the indication considered is peritoneal carcinomatosis from colorectal cancer. CPT code 77605 moved from non-covered to covered.  Added CPT code 96445 and ICD-9 code 197.6 as covered codes.

01/17/2012:  Policy title changed from "Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for the Treatment of Pseudomyxoma Peritonei and Peritoneal Carcinomatosis of Gastrointestinal Origin" to "Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy for the Treatment of Pseudomyxoma Peritonei, Peritoneal Carcinomatosis of Gastrointestinal Origin, and Peritoneal Mesothelioma."  Policy description updated. Policy statement added that cytoreductive surgery and perioperative intraperitoneal chemotherapy for the treatment of peritoneal mesothelioma may be considered medically necessary. Use of the term “hyperthermic” changed to “perioperative” in the title and policy statements to include early postoperative intraperitoneal chemotherapy. Use of the term “cytoreduction” changed to “cytoreductive surgery” to be more specific.

12/13/2012: Policy reviewed; no changes.

The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document. 

Covered Codes