Peritonectomy and hyperthermic intraperitoneal chemotherapy as treatment for desmoplastic small round cell tumour

Abstract

IntroductionThe 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.Presentation of caseThis 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.DiscussionDSRCT 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.ConclusionIn 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.

Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery?

“…Forty-three patients with DSRCT who received intraperitoneal chemotherapy have been reported in the literature, including our 11 patients, and all after 2010 [ 15 , 20 – 24 ]. The only randomized controlled trial in patients with peritoneal metastases originating from sarcomas concluded that intraperitoneal chemotherapy (without hyperthermia) did not bring any survival benefit [ 25 ].…”

Intra-abdominal desmoplastic small round cell tumour in a 56-year-old female: Case report of a very rare presentation of an unusual tumour

“…The rarity of the pathology means there are currently no evidence-based therapeutic guidelines on post-resection management, although several small series have shown partial response to adjuvant chemotherapy (P6 Protocol) [ 4 , 5 , 8 ]. In select cases, cytoreductive surgery (CRS) and intraperitoneal hyperthermic chemoperfusion (HIPEC) has been provided; one case had recurrence at 8.85 months [ 11 ] while a small phase 2 trial showed overall survival of 44.3 months [ 10 ]. It should be noted the patients who underwent CRS + HIPEC were young, otherwise healthy males, while our patient is a late middle-age female and morbidly obese with numerous comorbidities.…”

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

“…In 2013, Hirano et al [8] also published data using isolated irinotecan as second-line treatment for patients with EFS and OS of 6 and 20 months, respectively. The following year, Fan et al [4]. again tested the combination of temozolomide with irinotecan as a second-line treatment for two patients, resulting in EFS of 6 and 15 months.…”

“…A desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive cancer that affects adolescents and young adults. The tumor usually begins and spreads over the surface of the peritoneum [4, 12, 15, 16].…”