Holly Green

Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion

BACKGROUND Less than 200 cases have been reported in the world literature since desmoplastic small round cell tumor (DSRCT) was first described in 1989. To date, chemotherapy, radiation therapy, and surgery have resulted in a poor survival of 30% to 55%. We used hyperthermic intraperitoneal chemotherapy (HIPEC) at the time of complete tumor resection as an adjunct to treatment of pediatric and adolescent patients with DSRCT. PURPOSE The aim of this study was to assess survival as a function of disease burden and response to HIPEC in patients with DSRCT. METHODS Twenty-four patients with DSRCT from 1995 to 2008 were evaluated. Eight patients undergoing cytoreductive surgery and HIPEC were compared with 16 historical controls that had chemotherapy +/- radiation therapy or surgery alone. RESULTS Median age was 12 years in 8 patients who underwent HIPEC. Significant morbidity after HIPEC included renal insufficiency and gastroparesis. There were no operative mortalities. The estimated median overall 3-year survival for patients not undergoing surgery or HIPEC was 26% compared with 71% in patients who underwent HIPEC. Extraabdominal metastasis correlated with poor survival (P = .021). CONCLUSION Hyperthermic intraperitoneal chemotherapy is safe in children with DSRCT. It may prolong disease-free survival in selected cases of DSRCT. It may have a limited role as an adjunct to local control in patients with DSRCT.

Outpatient and home chemotherapy with novel local control strategies in desmoplastic small round cell tumor

Desmoplastic Small Round Cell Tumor (DSRCT) has a very poor prognosis. This report illustrates novel chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive ascites and >1000 abdominal tumors. Neoadjuvant chemotherapy included vincristine (1.5 mg/m2), ifosfamide (3 g/m2/day × 3), dexrazoxane/doxorubicin (750/75 mg/m2), and etoposide (150 mg/m2). Continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin (100 mg/m2) was given after extensive cytoreductive surgery. This was followed by irinotecan (10 mg/m2/day × 5 × 2 weeks) + temozolomide monthly × 2, then abdominal radiation 30 Gy with simultaneous temozolomide (100 mg/m2/day × 5). A total of 12 cycles of irinotecan and temozolamide were given. Except for initial chemotherapy, subsequent courses were in the outpatient setting. Focal retroperitoneal relapse at 18 months was treated with IMRT with bevacizumab (5 mg/kg) and 2 perihepatic metastases with radio frequency ablation/cryoablation followed by chronic outpatient maintenance chemotherapy (valproic acid, cyclophosphamide, and rapamycin). Almost 2 years from diagnosis, the patient maintained an excellent quality of life. This is a novel approach to the treatment of children with massive abdomino-pelvic DSRCT.

Toxicity of hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric patients with sarcomatosis/carcinomatosis: Early experience and phase 1 results†

Intra‐abdominal metastasis is a rare form of tumor dissemination in children. Complete surgical resection is usually deemed impossible. Children are frequently offered palliative care only. We adopted an aggressive approach for these cases which includes removal of dozens to hundreds of tumor nodules followed by perfusion of the abdominal cavity with hyperthermic chemotherapy (HIPEC) with a curative intent.

Whole Abdominopelvic Intensity-Modulated Radiation Therapy for Desmoplastic Small Round Cell Tumor After Surgery

PURPOSE Desmoplastic small round cell tumor (DSCRT) is an uncommon pediatric tumor with a poor prognosis. Aggressive multimodality therapy is the current treatment approach; however. treatment toxicity is of concern. We report our results with whole abdominopelvic intensity-modulated radiation therapy (WAP-IMRT) as a component of multimodality therapy for DSCRT at a single institution. MATERIALS/METHODS Medical records of all patients with DSCRT who received WAP-IMRT as part of definitive treatment at MD Anderson (2006-2010) were identified and reviewed. RESULTS Eight patients with DSRCT received WAP-IMRT with a median follow-up of 15.2 months. All patients received multiple courses of chemotherapy followed by surgical debulking of intra-abdominal disease; seven also had intraoperative hyperthermic cisplatin. WAP-IMRT was delivered to a total dose of 30 Gy postoperatively; four patients received a simultaneous boost (6-10 Gy) to sites of gross residual disease. Seven patients received concurrent chemotherapy during WAP-IMRT. No Radiation Therapy Oncology Group Grade 4 nausea, vomiting, or diarrhea occurred during RT. Red-cell transfusions were given to two patients to maintain hemoglobin levels >10 g/dL. Grade 4 cytopenia requiring growth factor support occurred in only one patient; no other significant cytopenias were noted. WAP-IMRT resulted in 25% lower radiation doses to the lumbosacral vertebral bodies and pelvic bones than conventional RT plans. The median time to local or distant failure after WAP-IMRT was 8.73 months in seven patients. One patient who had completed RT 20 months before the last follow-up remains alive without evidence of disease. Five patients (63%) experienced treatment failure in the abdomen. Distant failure occurred in three patients (37.5%). CONCLUSIONS WAP-IMRT with concurrent radiosensitizing chemotherapy was well tolerated after aggressive surgery for DSCRT. Enhanced bone sparing with IMRT probably accounts for the low hematologic toxicity (vs. conventional WAP-RT). This modality should be considered as an additional local-regional control option for DSRCT.

Treatment of carcinomatosis using cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in adolescents and young adults

BACKGROUND Among colorectal carcinoma patients, approximately 150 patients/year are age 25 years old or younger according to Surveillance, Epidemiology, and End Results Program statistics. Because of lack of screening in their age group, they are at risk to have more advanced disease and have been largely unstudied. OBJECTIVE To determine outcome of colon cancer adolescent and young adult patients. METHODS Patients under the age of 26 were retrospectively reviewed from a single institution. RESULTS The 5-year overall survival rate from the time of the first surgery was .45 (95% confidence interval .17 to .70). The median overall survival for the cohort was 2.98 years. Patients aged 15 to 21 years had a poorer overall survival than patients aged 22 to 25 years (82% survival vs 100% at 2 years and zero vs 56% at 5 years). Five patients underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Three are alive at 82, 36, and 16 weeks after hyperthermic intraperitoneal chemotherapy. CONCLUSIONS Patients less than 21 years with nonsyndromic colorectal carcinoma may have a poorer outcome. Novel, more aggressive therapy may be necessary in these patients.

Care of pediatric oncology patients after continuous hyperthermic peritoneal perfusion

Continuous Hyperthermic Peritoneal Perfusion Cytoreductive surgery (removal of all visible disease to 1 cm or less of remaining tumor) followed by continuous hyperthermic peritoneal perfusion (CHPP) with chemotherapeutic agents was first developed in the 1980s for adults with peritoneal carcinomatosis. CHPP is a wellaccepted treatment in adults with refractory peritoneal carcinomatosis. In a randomized controlled trial in adults with peritoneal carcinomatosis associated with colorectal cancer, CHPP improved survival. Results of other clinical trials and retrospective data have indicated improved survival with CHPP in adults with appendiceal neoplasms, Riza V. Mauricio, RN, MSN, CCRN, CPNP-AC/PC Holly Green, BS, PA-C Andrea Hayes-Jordan, MD Care of Pediatric Oncology Patients After Continuous Hyperthermic Peritoneal Perfusion Pediatric Care