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Journal of Clinical Oncology, Vol 22, No 8 (April 15), 2004: pp. 1518-1520 © 2004 American Society of Clinical Oncology. DOI: 10.1200/JCO.2004.05.126
Unusual Abdominal TumorsCASE 1. Pseudomyxoma Peritonei: Response to CapecitabineDon Monti Division of Oncology, Department of Medicine, North Shore University Hospital-New York University School of Medicine, Manhasset, NY; and the Washington Cancer Institute, Washington, DC. A 54-year-old male without a significant past medical history presented with abdominal pain and ascites. A paracentesis demonstrated a thick gelatinous fluid with cytology positive for malignant mucinous epithelial cells. An exploratory laparotomy revealed diffuse gelatinous ascites with multiple tumor nodules, prompting a cytoreduction, including a subtotal colectomy. Pathology revealed mucinous tumor involving the appendix with dissemination around the abdomen and pelvis. He was discharged without further treatment. Fourteen months later, a follow-up computed tomography (CT) scan revealed a large tumor accumulation beneath the right and left hemidiaphragms, the stomach encircled by tumor, and the sigmoid colon replaced by tumor. A cytoreduction was performed, which included removal of the tumor from the abdominal wall, perineum, both hemidiaphragms, cholycystectomy, pelvic peritonectomy, total colectomy, lesser omentectomy, total gastrectomy, and installation of heated intraoperative intraperitoneal hyperthermic mitomycin-C (IPHC). Tumor markers before surgery showed a carcinoembryonic antigen (CEA) level of 22.4 ng/mL (nL = 3.6 ng/mL) and CA 19-9 of 1,414 U/mL (nL = 36/U/mL). Postoperatively, the CEA fell to 0.6 ng/mL and the CA 19-9 decreased to 51 U/mL. Pathology again confirmed focally proliferating mucinous epithelium consistent with disseminated peritoneal adenomucinosis. Figure 1 is a CT of the abdomen showing the 2- to 3-cm mass superior to the groove between the vena cava and aorta. Figure 2 is a hematoxylin and eosin stained section (10x objective) showing pools of mucin partially lined by simple focally-proliferating columnar epithelium within fibrous stroma. There is no significant atypia or signet ring cells seen. Arrow shows focal proliferation of simple columnar cells (magnified in Fig 3). This is diagnostic of pseudomyxoma peritonei (PMP). The patient did well postoperatively and presented 8 months later for closure of the jejunostomy as well as a "second look." CT scan before surgery revealed a new 2 cm nodule in the right pelvis. Intraoperatively, the patient required lysis of adhesions, as well as cytoreduction of approximately 250 new tumor nodules located mostly between the mesentery. Patient again received IPHC with mitomycin-C. CEA and CA 19-9 were 0.5 ng/mL and 11.3 U/mL postoperatively, respectively. His CT scans remained negative and tumor markers remained stable until May 2001, when a CT scan revealed new retrocrural and para-aortic adenopathy. The CA 19-9 rose to 315 U/mL and CEA rose to 8.7 ng/mL. A biopsy attempt was made via CT guidance, however the tissue showed only blood without malignant cells. The patient was placed on capecitabine, 1,000 mg twice daily. After several weeks of treatment, the CA 19-9 and CEA were lowered (Fig 4). The patient is tolerating capecitabine well, except for mild hand-foot syndrome.
This case highlights new advances in our understanding and treatment of PMP. First described in 1842, PMP is a rare, often fatal intra-abdominal disease characterized by gelatinous ascites and multifocal peritoneal epithelial implants. In 1901, there was the first association of gelatinous ascites with an appendiceal rupture. Since then, a heterogeneous group of mucinous tumors have been identified, but it was not until recently, when PMP was formally classified according to histology, was it better understood.1 A classification system was created based on the morphologic features of the epithelium identified in peritoneal implants and on the nature of the underlying lesion. Further studies have shown that each category defined has a different prognosis and optimal treatment modality.2 The first category, disseminated peritoneal adenomucinosis, describes lesions containing abundant extracellular mucin associated with simple- to focally-proliferative epithelium with minimal cytologic atypia and mitotic activity. Fifty-seven percent of these cases examined were secondary to ruptured appendiceal mucinous adenomas.3 These tumors are usually superficial and noninvasive with only abdominal peritoneal implants. These patients have a 68% 10-year survival with hyperthermic intraoperative intraperitoneal mitomycin-C for treatment.3 In contrast is peritoneal mucinous carcinomatosis, which also has peritoneal lesions with abundant extracellular mucin, but with more epithelial cells and signet ring cell morphology. These cases are diagnosed as carcinomas with adenomucinosis and mucinosis features, and generally arise from gastrointestinal neoplasms.1 They cause invasive peritoneal implants with parenchymal invasion, lymphadenopathy, and often metastasize beyond the abdominal cavity. The 10-year survival is just 3%, warranting more aggressive treatment with intra-operative and postoperative hyperthermic intraperitoneal mitomycin-C and fluorouracil.3 A third category, a hybrid of the first two, shows features of disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis in primary and peritoneal lesions. These neoplastic cells have both bland mucinous adenoma and well-differentiated mucinous carcinoma. The 10-year survival is 21%.1,3 It is clear from literature that the overall prognosis is based on the primary tumor type and histologic appearance. However, recent studies now show that improved treatment for these patients includes optimal cytoreduction followed by hyperthermic intraperitoneal mitomycin-C.4-7 In the literature, only a limited number of patients have received adjuvant systemic chemotherapy.3 There are no published reports of the effectiveness of capecitabine in these patients. Capecitabine is an orally administered precursor of 5'deoxy-5-fluorouridine. It is absorbed as the intact molecule through the intestinal mucosa and undergoes activation to fluorouracil in three metabolic steps.8 Our patient, with hybrid grade histology, has been optimally cytoreduced and received only IPHC. Further treatment was necessary based on rising tumor markers and new lesions on CT scan.9 As clearly shown in the graph above, capecitabine has had a significant effect in reducing the patient's tumor markers and controlling recurrent disease. Follow-up CT scans did not show evidence of disease progression. The minimal chronic toxicity of capecitabine makes it a suitable treatment for this disorder. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Ronnett BM, Zahn CM, Kurman RJ, et al: Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei". Am J Surg Pathol 19:1390-1408, 1995[Medline] 2. Jackson SL, Fleming RA, Loggie BW, et al: Gelatinous ascites: A cytohistologic study of pseudomyxoma peritonei in 67 patients. Mod Pathol 14:664-671, 2001[CrossRef][Medline] 3. Ronnett BM, Yan H, Kurman RJ, et al: Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer 92:85-91, 2001[CrossRef][Medline] 4. Witkamp AJ, de Bree E, Kaag MM, et al: Extensive surgical cytoreduction and intraoperative hyperthermic intraperitoneal chemotherapy in patients with pseudomyxoma peritonei. Br J Surg 88:458-463, 2001[CrossRef][Medline] 5. Yan H, Pestieau SR, Shmookler BM, et al: Histopathologic analysis in 46 patients with pseudomyxoma peritonei syndrome: Failure versus success with a second-look operation. Mod Pathol 14:164-171, 2001[CrossRef][Medline] 6. Sugarbaker PH, Chang D: Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. Ann Surg Oncol 6:727-731, 1999[CrossRef][Medline]
7. Shen P, Levine EA, Hall J, et al: Factors predicting survival after intraperitoneal hyperthermic chemotherapy with mitomycin c after cytoreductive surgery for patients with peritoneal carcinomatosis. Arch Surg 138:26-33, 2003 8. Van Cutsem E, Peeters M: Developments in fluoropyrimidine therapy for gastrointestinal cancer. Curr Opin Oncol 11:312-317, 1999[CrossRef][Medline] 9. van Ruth S, Hart AA, Bonfrer JM, et al: Prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 19.9 measurements in patients with pseudomyxoma peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol 9:961-967, 2002[CrossRef][Medline]
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Copyright © 2004 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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