To assess the potential value of pre-operative CEA, CA 125 and CA 19-9 in identifying Pseudomyxoma peritonei (PMP) patients at risk of recurrence following complete cytoreductive surgery and intraperitoneal chemotherapy.

Between March 1996 and October 2001, 35 patients, with documented pre-operative tumour marker measurements, underwent complete cytoreduction. Outcome in the 32 survivors was recorded.

Median age was 53 years (range: 32-74). There were 17[53%] females. In 9/32[28%] all tumour markers were normal. The most common abnormality was elevated CEA level in 14/32[44%]. Median follow up was 23 months (range: 11-74). Recurrence was detected in 8/32[25%]. The 2-year recurrence free interval was 53% in those with elevated CEA compared with 94% with normal CEA. The 2-year disease free intervals were 100% in 9 patients with normal preop markers compared with 66% for the group with at least one abnormal marker.

PMP patients with elevated CEA, or more than one abnormal serum tumour maker, are at risk of developing recurrent disease.

To establish selection criteria for reoperation in patients with peritoneal dissemination from appendiceal malignancy.

The outcome of patients with mucinous appendiceal neoplasms with peritoneal surface dissemination has changed as a result of a better understanding of the clinical and pathologic features of this disease. This knowledge, combined with aggressive strategies for the use of perioperative intraperitoneal chemotherapy and peritonectomy procedures, has resulted in long-term disease-free survival in many of these patients. However, some of the patients develop progressive disease, and a question regarding additional surgery is appropriate. A critical analysis of the results of second-look surgery should assist in the management of patients with recurrent peritoneal surface dissemination of an appendiceal neoplasm.

Three hundred twenty-one patients with epithelial peritoneal surface malignancy of appendiceal origin underwent surgery during a 12-year period. Ninety-eight of these patients (30.5%) underwent a second-look procedure. A database of selected clinical features regarding these 98 patients was gathered from the clinical records. A critical statistical analysis of these clinical features and their prognostic impact was performed using survival as an endpoint. All patients were managed by a treatment regimen that used cytoreductive surgery and intraperitoneal chemotherapy.

The overall 5-year survival rate of these 98 patients was 73.6%. This compared favorably with a 68% survival rate of 223 patients who did not undergo reoperation. Survival based on the number of cytoreductive surgeries and the free interval between them showed no significant difference. Patients who had second-look surgery with bowel obstruction as a symptom and those in whom the amount of tumor was increased or minimally decreased at the first and second cytoreductions had a significantly inferior 5-year survival rate. A complete second cytoreduction was associated with an improved 5-year survival rate.

Follow-up of patients treated for peritoneal dissemination from neoplasms of appendiceal origin is indicated. Selected patients in whom recurrence develops are candidates for repeat cytoreductive surgery plus intraperitoneal chemotherapy with curative intent.

Peritoneal carcinomatosis, regardless of primary tumour type, has always been a lethal condition. Recently special treatments using cytoreductive surgery with peritonectomy procedures combined with peri-operative intraperitoneal chemotherapy have resulted in long-term survival. Pseudomyxoma peritonei may be especially appropriate for these aggressive local regional treatments. All patients treated prior to 1999 are presented; patients left with gross residual disease after surgery were not given intraperitoneal chemotherapy, but were later treated with intravenous chemotherapy after cytoreduction. The intraperitoneal chemotherapy was given in the peri-operative period, starting with mitomycin C. For patients whose pathology showed adenomucinosis, intraperitoneal chemotherapy was limited to treatment in the operating theatre with heated mitomycin C. Patients with mucinous adenocarcinoma or pseudomyxoma/adenocarcinoma hybrid had, in addition to mitomycin C, 5 consecutive days of intraperitoneal 5-fluorouracil. A complete cytoreduction was defined as tumour nodules <2.5 mm in diameter remaining after surgery. The histopathology categorized the patients as adenomucinosis, intermediate type, or mucinous carcinomatosis. A prior surgical score was used to estimate the extent of previous surgical procedures. The morbidity of treated patients was 27% and the mortality was 2.7%. In a multivariate analysis, prognostic factors for survival included the completeness of cytoreduction (P<0.0001), the histopathological character of the appendix malignancy (P<0.001) and the extent of previous surgical interventions (P=0.001). Patients with a complete cytoreduction and adenomucinosis by pathology had a 5-year survival of 86%; while hybrid pathology survival at 5 years was 50%. Incomplete cytoreduction had a 5-year survival of 20% and 0% at 10 years. Cytoreductive surgery and peri-operative intraperitoneal chemotherapy is the current standard treatment for selected patients with peritoneal surface spread of appendiceal primary tumours. Similar strategies for other patients with peritoneal surface malignancy such as peritoneal carcinomatosis from colon or gastric cancer, peritoneal sarcomatosis, or peritoneal mesothelioma should be pursued.

In the past, peritoneal carcinomatosis, regardless of primary tumor type, has always been a lethal condition. Recently, special treatments using cytoreductive surgery with peritonectomy procedures combined with perioperative intraperitoneal chemotherapy have resulted in long-term survival. Appendiceal malignancy with a low incidence of liver and lymph node metastases may be especially appropriate for these aggressive local regional treatments.

All patients treated with surgery before January 1999 are included. Patients left with gross residual disease after surgery were not given intraperitoneal chemotherapy, but were later treated with intravenous chemotherapy. The intraperitoneal chemotherapy was given in the perioperative period, starting with mitomycin C at 12.5 mg/m2 for males and 10 mg/m2 for females. For patients whose pathology showed adenomucinosis, intraperitoneal chemotherapy was limited to treatment in the operating theater with heated mitomycin C. Patients with mucinous adenocarcinoma or pseudomyxoma/adenocarcinoma hybrid had, in addition to mitomycin C, five consecutive days of intraperitoneal 5-fluorouracil at 650 mg/m2 instilled in 1-1.5 liters of 1.5% dextrose peritoneal dialysis solution. A complete cytoreduction was defined as tumor nodules <2.5 mm in diameter remaining after surgery. The histopathology categorized the patients as having adenomucinosis, adenomucinosis/carcinomatosis hybrid, or mucinous carcinomatosis. A previous surgical score was used to estimate the extent of previous surgical procedures.

The morbidity of treated patients was 27% and the mortality was 2.7%. In a multivariate analysis, prognostic factors for survival included the completeness of cytoreduction (P < .0001), the histopathological character of the appendix malignancy (P < .0001), and the extent of previous surgical interventions (P = .001). Patients with a complete cytoreduction and adenomucinosis by pathology had a 5-year survival of 86%; with hybrid pathology, survival at 5 years was 50%. Incomplete cytoreduction had a 5-year survival of 20% and 0% at 10 years.

Cytoreductive surgery and perioperative intraperitoneal chemotherapy can be used to salvage selected patients with peritoneal surface spread of appendiceal primary tumors. Similar strategies for other patients with peritoneal surface malignancy such as peritoneal carcinomatosis from colon or gastric cancer, peritoneal sarcomatosis, or peritoneal mesothelioma should be pursued.

Intraperitoneal chemotherapy and cytoreductive surgery have been combined to reach treatment objectives in patients with peritoneal carcinomatosis or sarcomatosis that neither modality by itself can achieve. These treatments must be combined with the proper selection of patients in order to observe "high value" results from therapy. There are three principles of management to support treatments for peritoneal carcinomatosis and sarcomatosis. First, the clinician must select patients who have isolated disease distributed on the surfaces of abdominal and pelvic structures. Patients treated for peritoneal implants who have persistent disease at other sites will profit little or not at all. Second, this disease must be reduced to its lowest possible mass by peritonectomy procedures and resections of viscera. Third, maximal intraperitoneal and systemic chemotherapy are utilized to eradicate the disease on peritoneal surfaces as well as control the primary or recurrent tumor. These principles of management and a presentation of the results achieved to date are reviewed.