title: Peritonectomy and hyperthermic intraperitoneal chemotherapy as treatment for desmoplastic small round cell tumour.
abstract: BACKGROUND
  The St George Hospital specialises in peritonectomy and hyperthermic intraperitoneal chemotherapy (HIPEC) for treatment of intra-abdominal malignancies. Despite performing around 800 peritonectomy and HIPEC procedures, we have rarely encountered desmoplastic small round cell tumours (DSRCT). We present our experiences with DSRCT, and propose peritonectomy and HIPEC as a treatment option for DSRCT.
  
  
  METHODS
  This is a case series of 3 cases. The first case was a 26-year-old male who presented with appendicitis which we diagnosed as DSRCT and treated with peritonectomy and HIPEC. The second case was a 14-year-old male referred to our centre for peritonectomy and HIPEC after initial presentation with a pelvic mass and treatment with chemotherapy. The third case was a 21-year-old male referred to our centre for peritonectomy and HIPEC for recurrent DSRCT after previously being treated with neoadjuvant chemotherapy and surgery without HIPEC.
  
  
  CONCLUSIONS
  DSRCT is a rare, almost exclusively intra-abdominal malignancy, which predominantly affects young males. Survival prognosis remains poor in DSRCT despite conventional treatment with surgery, chemotherapy and radiotherapy; however, HIPEC has offered promising survival results. Our recurrences with peritonectomy and HIPEC at 6 months and 15 months are comparable with the literature of 8.85 months.
  
  
  CONCLUSIONS
  In our experience, patients with DSRCT who present with nodal involvement or recurrent disease tend to recur early despite treatment with peritonectomy and HIPEC. Longer term follow up of our patients and future studies involving HIPEC in DSRCT would be useful in assessing long-term clinical outcomes and survival.
authors.affiliation: Department of Surgery, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia.
authors.forename: Howard S
authors.initials: HS
authors.lastname: Fan
authors.affiliation: Department of Anatomical Pathology, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia.
authors.forename: Bernie
authors.initials: B
authors.lastname: I'Ons
authors.affiliation: Department of Surgery, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia.
authors.forename: Ryan
authors.initials: R
authors.lastname: McConnell
authors.affiliation: Department of Surgery, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia.
authors.forename: Varahini
authors.initials: V
authors.lastname: Kumar
authors.affiliation: Department of Surgery, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia.
authors.forename: Saleh
authors.initials: S
authors.lastname: Alzahrani
authors.affiliation: Department of Surgery, University of New South Wales, St George Hospital, Kogarah, NSW 2217, Australia. Electronic address: david.morris@unsw.edu.au.
authors.forename: David L
authors.initials: DL
authors.lastname: Morris
keywords: Desmoplastic small round cell tumour (DSRCT); Hyperthermic intraperitoneal chemotherapy (HIPEC); Peritonectomy