Cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy in the treatment of rare tumors with peritoneal metastases.

Purpose: Cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal intraoperative chemotherapy (HIPEC) is the standard treatment for tumors presented with peritoneal metastases (PM). Data in the literature about the treatment of rare tumors with PM are limited and of low-quality. The aim of the study was to assess the outcome and safety of CRS and HIPEC for these tumors.

Methods: Patients with rare tumors with PM that underwent CRS and HIPEC between 2005-2018, were retrospectively analyzed. Clinical and histopathological variables were correlated to survival.

Results: 43 patients, mean age 55.7 ± 12.9 years, underwent 48 cytoreductions. The most frequent histopathologic type was sarcomatosis (31.3%). The majority of the patients (70.8%) had limited extent of peritoneal disease. Complete or near-complete cytoreduction was achieved in 83.3% of the cases. Severe morbidity was recorded in 12.6%. The median disease-free survival and overall survival were 11 and 63 months, respectively. Although the completeness of cytoreduction was found to be significantly related to survival, the extent of peritoneal carcinomatosis was the single prognostic factor.

Conclusions: CRS followed by HIPEC is an effective and safe method in the treatment of rare tumors with PM. Further large, well-designed prospective studies are needed to validate these results.