Abstract
INTRODUCTION: In selected cases, cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is an established treatment for patients suffering from peritoneal metastases from colorectal, ovarian, gastric or appendiceal origin. The effectiveness of this extensive has not been elucidated within other rare diseases by now. METHODS: We conducted a retrospective analysis of patients treated with CRS for peritoneal carcinomatosis during the period between July 2010 and September 2015. Exclusion criteria were adenocarcinomas of the stomach, colon, neoplasms of the appendix, mesothelioma and ovarian cancers. Aim of this study was to examine the feasibility, complication rate and survival of patients with rare diseases. RESULTS: A total of 14 Patients were included: Four rare gynecological tumors, three adenocarcinomas of the small intestine, three retroperitoneal sarcomas, one cholangiocellular carcinoma, one neuroendocrine gastric tumor, one malignant peripheral nerve sheath tumor and one cancer of unknown primary syndrome. In 12 of 14 patients a macroscopically complete tumorresection could be achieved. No patient died during hospitalization. Seven of 14 patients experienced general complication of grade III according to NCI CTCAE V4.0, while two experienced complications of grade IV. Median follow-up and one year overall survival were 15.5 months and 46.8%, respectively. CONCLUSION: For patients with rare tumors, CRS and HIPEC is feasible with an acceptable perioperative morbidity and mortality. To improve knowledge in patient selection and outcome, rare tumors treated with CRS and HIPEC should be documented in central databases (as for example BIG RENAPE, Pierre-Benite, France).