Hyperthermic Intraperitoneal Chemotherapy Following Cytoreductive Surgery Improves Outcome in Patients With Primary Appendiceal Mucinous Adenocarcinoma: A Pooled Analysis From Three Tertiary Care Centers

Abstract

Purpose. Appendiceal mucinous neoplasms (AMN) are a rare heterogeneous group of diseases. In the absence of randomized trials, AMN management is controversial. The goal of this study was to evaluate the impact of hyperthermic intraperitoneal chemotherapy (HIPEC) after cytoreductive surgery on survival in AMN patients. Patients and Methods. Patient data including demographics, pathology, type of therapy, and outcomes were collected from Emory University, the Ohio State University, and Wayne State University databases. One of the three centers did not use HIPEC. Statistical analysis evaluating overall survival (OS) of AMN patients was performed. Results. Between 1990 and 2010, 163 AMN patients were identified. Histology showed 60 patients had diffuse peritoneal adenomucinosis, 88 had peritoneal mucinous carcinomatosis (PMCA), and 15 had PMCA with indeterminate or discordant features. Complete surgical resection was achieved in 76 patients. HIPEC was used in 79 patients. The median OS was 77 months for patients who received HIPEC compared with 25 months for patients who did not (p , .001). In multivariable analysis, histopathologic subtype (p , .001), complete surgical resection (p , .001), and HIPEC (p , .001) were independent predictors for improved OS. A survival advantage for AMN patients treated at HIPEC-treating centers was observed (p 5 .0026). After adjusting for HIPEC therapy, no significant survival difference was observed between the non-HIPEC-treating center and the HIPEC-treating centers (p 5 .094). Conclusion. The addition of HIPEC to cytoreductive surgery likely provides a survival advantage and should be considered in the treatment strategy for AMN. The Oncologist 2015; 20:907-914Implications for Practice: The use of heated chemotherapy applied in the abdomen after removal of the appendiceal mucinous cancer by surgery lengthens survival of patients having this disease.

Appendiceal Mucinous Neoplasms: Diagnosis and Management

“…Use of systemic chemotherapy prior to CRS predicted worse OS and PFS [54]. Similarly, in the study by Shaib et al [60], systemic chemotherapy was associated with worse OS, with a median of 32 months compared with 82 months. In another retrospective study, Baratti et al [62] analyzed prognostic factors among 104 patients who had received CRS, 25% of whom had previously received systemic chemotherapy.…”

Incidence and Survival of Appendiceal Mucinous Neoplasms

“…We reported our institutional experience of advanced-stage AMN patients treated with HIPEC and surgery indicating survival advantage of this combined modality when compared with surgery alone. 21 This study has limitations related to the use of large national databases as previously described. 30 The most significant limitation is related to accuracy of the data especially to pathologic AMN grading where different pathologic classification systems were developed over the time period included in this analysis (1973 to 2011).…”

“…This has led to the adoption of this approach as the standard therapy for advanced AMN disease. [14][15][16][17] Although natural history and prognosis of AMN has been studied in single-institution studies, these studies were all rather small (N = 50 to 164) [18][19][20][21] and of limited generalizability except for 1 multicenter retrospective report (N = 2298). 15 This knowledge gap can be addressed through population-based studies that rely on national cancer surveillance data.…”

Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

“…23,26 Although hemicolectomy with adequate surgical margins is an established goal, 3,14 patients would benefit from peritoneal and omental exploration, perhaps even with prophylactic salpingo-oophorectomy and hysterectomy in post-menopausal women. 12,23,27 Additional hyperthermic intraperitoneal chemotherapy in a manner similar to appendiceal mucinous neoplasms 28,29 warrants further exploration as it has been linked to improved survival when compared with systemic chemotherapy. 26 The paucity of hematogenous metastasis (to organs such as liver) provides an additional argument for this approach.…”