Recent progress in pediatric soft tissue sarcoma therapy.

Soft tissue sarcomas of childhood are a heterogenous group of tumors with a wide spectrum of presentations and outcomes. Most patients require multimodal therapy with chemotherapy, surgery and/or radiation. Improved outcomes in recent decades have been achieved through improvements in the comprehensive care of these children through large cooperative group studies, even as little progress has been made in the standard chemotherapy backbone. A thorough understanding of the nuances of surgical therapy for these children is required to minimize both the risk of local failure and the possibility of loss of vital form or function.

An analysis of the survival impact of dexmedetomidine in children undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.

OBJECTIVE: Recent evidence suggests the α2-adrenoreceptor agonist dexmedetomidine may promote metastasis of cancer cells. In this study we sought to evaluate the impact of dexmedetomidine administration on the survival of children and adolescents with cancer. DESIGN: Retrospective chart review. SETTING: Comprehensive cancer center. PATIENTS: Children and adolescents who had undergone cytoreductive surgery with hyperthermic intraperitoneal chemotherapy for peritoneal carcinomatosis. INTERVENTION: Intraoperative and/or early postoperative (within 24 hours of surgery) administration of dexmedetomidine. MEASUREMENTS: Multivariable cox proportional hazard models were used to assess the association between dexmedetomidine administration and progression free survival (PFS) or overall survival (OS). MAIN RESULTS: Ninety-three patients were identified. The median age was 12 years, 42% were female, and 35% received dexmedetomidine. There were no significant differences between the baseline and perioperative characteristics of patients who received dexmedetomidine and those who did not. In the multivariable analysis, the administration of dexmedetomidine was not associated with PFS (HR = 1.20, 95% CI [0.60-2.41], p = .606) or OS (HR = 0.81, 95% CI [0.35-1.85], p = .611). CONCLUSION: In this retrospective study of children and adolescents who had undergone a major oncologic surgery, the intraoperative and/or early postoperative administration of dexmedetomidine was not associated with survival.

Perioperative factors associated with persistent opioid use after extensive abdominal surgery in children and adolescents: A retrospective cohort study.

BACKGROUND: In adults, preoperative opioid use and higher perioperative opioid consumption have been associated with higher odds of persistent opioid use after surgery. There are limited data on the prevalence and factors associated with persistent opioid use after major oncologic surgery in children. AIMS: In this study, we sought to determine the prevalence and factors associated with the development of persistent opioid use in a group of children and adolescents who had undergone cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. METHODS: A retrospective study of patients ≤19 years of age was performed. Univariable logistic regression was used to assess factors associated with a postdischarge persistent opioid use of up to 6 months. RESULTS: Eighty-six children were identified. The median age was 12 years, and 43% were female. The proportion of patients with persistent opioid use over the immediate 3, 6, 12 and 24 postdischarge months was 54/77 (70%), 18/51 (35%), 13/45 (29%), and 3/24 (13%), respectively. The daily average in-patient pain scores were higher in the group of children who subsequently developed persistent opioid use of up to 6 months (estimated difference 0.5, 95% confidence interval [CI]: 0.3, 0.8, P < .01). Furthermore, higher postoperative opioid consumption was associated with greater odds of a subsequent persistent opioid use of up to 6 months (odds ratio 1.03, 95% CI: 1.00, 1.07, P = .05). CONCLUSION: In this retrospective study of children and adolescents who had undergone a major oncologic surgery, higher in-patient pain scores and higher postoperative opioid consumption were associated with a persistent opioid use of up to 6 months.

Histiocytic sarcoma in a child-successful management and long-term survival with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Histiocytic sarcoma is an extremely rare tumor in children. It may occur sporadically or in association with other hematological malignancies. It arises most commonly in the lymph nodes but may occur anywhere in the body and clinical presentation is usually with advanced disease. Following tissue diagnosis and staging, management is with chemotherapy though there are no standard regimes. Surgery has been used successfully for local control. This is the first description of the use of peritonectomy and hyperthermic intraperitoneal chemotherapy to treat histiocytic sarcoma. The 4-year-old patient has been disease free for 6 years.

Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma that primarily affects adolescents and young adults. Patients can present with many peritoneal implants. We conducted a phase 2 clinical trial utilizing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) with cisplatin for DSRCT and pediatric-type abdominal sarcomas. PATIENTS AND METHODS: A prospective cohort study was performed on 20 patients, who underwent CRS-HIPEC procedures, with cisplatin from 2012 to 2013. All patients were enrolled in the phase 2 clinical trial. Patients with extraabdominal disease and in whom complete cytoreduction (CCR0-1) could not be achieved were excluded. All outcomes were recorded. RESULTS: Fourteen patients had DSRCT, while five patients had other sarcomas. One patient had repeat HIPEC. Patients with DSRCT had significantly longer median overall survival after surgery than patients with other tumors (44.3 vs. 12.5 months, p = 0.0013). The 3-year overall survival from time of diagnosis for DSRCT patients was 79 %. Estimated median recurrence-free survival (RFS) was 14.0 months. However, RFS for patients with DSRCT was significantly longer than for non-DSRCT patients (14.9 vs. 4.5 months, p = 0.0012). Among DSRCT patients, those without hepatic or portal metastases had longer median RFS than those with tumors at these sites (37.9 vs. 14.3 months, p = 0.02). In 100 % of patients without hepatic or portal metastasis, there was no peritoneal disease recurrence after CRS-HIPEC. CONCLUSIONS: Complete CRS-HIPEC with cisplatin is effective in select DSRCT patients. DSRCT patients with hepatic or portal metastasis have poorer outcomes.

Assessing the survival impact of perioperative opioid consumption in children and adolescents undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.

BACKGROUND: Several studies in adult patients have suggested an unfavorable association between opioid consumption and cancer progression. AIMS: This study investigated the impact of opioid consumption on the survival of children and adolescents undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. METHODS: A retrospective study of patients <19 years who had undergone cytoreductive surgery with hyperthermic intraperitoneal chemotherapy was performed. Univariate and multivariate Cox proportional hazard analyses were used to identify factors associated with recurrence-free survival and overall survival. RESULTS: Seventy-five patients were identified. Median age was 11.6 years (range, 1.8-18.9), and 43% was female. Median perioperative opioid consumption was 18.9 morphine dose equivalents per kilogram (range, 0.6-339.6). There was no statistically significant association between opioid consumption and recurrence-free survival [hazard ratio, 1.00; 95% confidence interval, (0.99-1.02), P = 0.55] or overall survival [hazard ratio 1.01; 95% confidence interval (0.99-1.03), P = 0.22]. Independent prognostic factors associated with poor survival included incomplete cytoreduction and extra-abdominal disease. CONCLUSION: In this retrospective study of children and adolescents who had undergone cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, there was no statistically significant association between opioid consumption and recurrence-free survival or overall survival.

Treatment outcomes in pediatric melanoma-Are there benefits to specialized care?

PURPOSE: The purpose of this study was to evaluate the impact of hospital specialization on survival in pediatric melanoma. METHODS: We reviewed all patients under 18years old with cutaneous melanoma evaluated at MD Anderson Cancer Center, a National Cancer Institute (NCI)-designated center, from 2000 to 2014. We compared overall survival (OS) and disease-free survival (DFS) between patients who underwent all treatments at MDACC (Group A, n=146) and those who underwent initial surgical treatment at a non-NCI center (Group B, n=58). Kaplan-Meier survival curves were compared using the log-rank test. RESULTS: Group A patients had significantly better OS and DFS (both p<0.001). Five-year OS was 97% (95% CI 92%-99%) in Group A versus 88% (95% CI 74%-94%) in Group B. Group survival differences were most notable in Stage 3 and 4 patients. Group A patients presenting with stage III or IV disease had a 5-year OS rate of 91.2% (95% CI 75.1%-97.1%) compared to 80.8% (95% CI 59.8%-91.5%) in Group B. The DFS rate was 94.4% (95% CI 88.5%-97.3%) in Group A versus 77.2% (95% CI 62.5%-86.7%) in Group B. CONCLUSION: Surgical treatment at a comprehensive cancer center may improve outcomes for pediatric melanoma especially for patients presenting with later stage disease. LEVEL OF EVIDENCE: Case-control study: Level III.

Evaluating the Impact of Total Intravenous Anesthesia on the Clinical Outcomes and Perioperative NLR and PLR Profiles of Patients Undergoing Cytoreductive Surgery with Hyperthermic Intraperitoneal Chemotherapy.

PURPOSE: The aim of this study was to assess the impact of total intravenous anesthesia (TIVA) on the perioperative inflammatory profile and clinical outcomes of patients undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC). METHODS: A retrospective review of patients undergoing CRS-HIPEC was performed. Patients receiving a combination of preoperative tramadol extended release (ER), celecoxib, and pregabalin, along with combined intraoperative infusions of propofol, dexmedetomidine, lidocaine, and ketamine were classified as receiving a TIVA regimen (TIVA group). The second group consisted of patients receiving volatile-opioid-based anesthesia (VO group). The neutrophil:leukocyte (NLR) and platelet: leukocyte (PLR) ratios were calculated to evaluate the perioperative inflammatory status of both groups. Length of stay (LOS) and complications of both groups were also evaluated. RESULTS: A total of 213 patients were included in the study-139 in the VO group and 74 in the TIVA group. No statistically significant differences were observed between the groups with regard to their postoperative inflammatory profiles, LOS, or complications by organ system; however, the incidence of renal complications was higher in the TIVA group (8.1 vs. 2.2 %) and approached statistical significance (p = 0.068). CONCLUSIONS: In this retrospective study of patients undergoing CRS-HIPEC, the combined use of preoperative celecoxib, tramadol ER and pregabalin followed by intraoperative TIVA with infusions of propofol, dexmedetomidine, ketamine, and lidocaine was not associated with a reduction in LOS or complications by organ system. Postoperative NLR and PLR profiles were also not significantly impacted.

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric ovarian tumors: a novel treatment approach.

PURPOSE: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been used in adults with ovarian carcinoma proving overall survival benefit in randomized trials, but measured in months. Diffuse peritoneal disease from pediatric type ovarian tumors is rare. We applied CRS and HIPEC to pediatric girls with diffuse peritoneal disease as part of a clinical trial. METHODS: In all patients complete cytoreduction was followed by HIPEC using 100 mg/m2 of cisplatin for 90 min in a closed technique. All received neoadjuvant chemotherapy. Patients with disease outside of the abdominal cavity were excluded. RESULTS: Of 101 pediatric CRS and HIPEC operations, 8 had ovarian primary tumors and multifocal peritoneal disease. There were three yolk sac tumors (germ cell, mixed teratoma), one Sertoli­Leydig, one PNET of the ovary, one choriocarcinoma, one juvenile granulosa cell tumor and one adenocarcinoma. Age ranged 4­18 years. Three of the 8 (37 %) recurred and died. The remaining 63 % are disease free 2­6 years post HIPEC. Overall survival and relapse-free survival in this cohort was 64 and 62 %, respectively [CI 0.64 (0.34, 1); 0.62 (0.37, 1)]. CONCLUSIONS: This is the first report of CRS and HIPEC in pediatric ovarian tumors. HIPEC may be effective in pediatric-type ovarian tumors. More study is needed in a larger cohort.

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.

Cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) for children, adolescents, and young adults: the first 50 cases.

BACKGROUND: Extensive peritoneal metastatic disease is rare in children. Although usually manifested as carcinomatosis in adults, sarcomatosis is more common in children. The authors began a pediatric hyperthermic intraperitoneal chemotherapy (HIPEC) program, and this report describes their initial results from the first 50 pediatric, adolescent, and young adult patients. METHODS: A single-institution, retrospective study investigated the first 50 cytoreductive surgeries and HIPEC by one surgeon for patients 3-21 years of age. The HIPEC was added to chemotherapy and radiotherapy treatment. Demographics, outcome, and complications were recorded. RESULTS: The median follow-up period for the surviving patients was 21.9 months. The most common diagnoses were desmoplastic small round cell tumor (n = 21), rhabdomyosarcoma (n = 7), mesothelioma (n = 4), and other carcinoma (n = 17). Multivariate analysis showed that patients treated with HIPEC and an incomplete cytoreduction had a greater risk for recurrence than those who had a complete cytoreduction (p = 0.0002). The patients with a higher peritoneal cancer index (PCI) (i.e., a large tumor burden) had a median overall survival (OS) time of 19.9 months relative to the patients with a lower PCI score, who had a median OS of 34 months (p = 0.049). The patients without complete cytoreduction had a median OS of 7.1 months compared with 31.4 months for the patients with complete cytoreduction (p = 0.012). No perioperative mortalities occurred. The incidence of major complications was 28 %. CONCLUSION: Cytoreductive surgery and HIPEC with a programmatic approach for patients 3-21 years of age is unique. The best outcome was experienced by patients with desmoplastic small round cell tumor and those with complete cytoreduction. Complete cytoreduction for patients without disease outside the abdominal cavity at the time of surgery affords the best outcome.

Evaluation of ototoxicity in patients treated with hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin and sodium thiosulfate.

OBJECTIVES: Ototoxic hearing loss associated with intravenous or intra-arterial administration of cisplatin is well documented. However, there is limited data regarding the ototoxic effect of cisplatin when perfused into the abdominal cavity using hyperthermic intraperitoneal chemotherapy (HIPEC). The purpose of this study is to assess and describe ototoxicity in patients treated with HIPEC with cisplatin and sodium thiosulfate for peritoneal surface malignancies. DESIGN: We performed a retrospective chart review (2007-2012) of patients treated for advanced peritoneal malignancies at a tertiary care center using HIPEC with cisplatin and sodium thiosulfate infusion. Thirteen patients (12 males, 1 female) met study criteria. Audiometric thresholds were compared before and after treatment. A 20 dB loss at any single frequency, 10 dB decrease at any two adjacent frequencies, or loss of response at three consecutive test frequencies defined a significant ototoxic change (). RESULTS: Despite minimal hearing change in six patients, none of the 13 patients in our study exhibited a significant ototoxic change in hearing sensitivity post HIPEC with cisplatin at any test interval in any test frequency. CONCLUSIONS: Our findings represent the first objective assessment of ototoxic effect after HIPEC with cisplatin and sodium thiosulfate infusion. Our results suggest that peritoneal perfusion of cisplatin with intravenous perfusion of sodium thiosulfate is not associated with ototoxic changes in hearing sensitivity. Further investigation of the administration and systemic mechanism of absorption of sodium thiosulfate as a potential protection against cisplatin ototoxicity is needed to confirm these findings.

Safety of epidural analgesia in the perioperative care of patients undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.

BACKGROUND: The perioperative coagulopathy, hemodynamic instability, and infectious complications that may occur during cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has raised concerns about the safety of epidural analgesia in patients undergoing such procedures. METHODS: We conducted a retrospective review of the perioperative anesthetic management of 215 adult patients who had undergone CRS with HIPEC with epidural analgesia. We reviewed epidural-related complications and analyzed the effect of early initiation of continuous epidural analgesia on estimated blood loss, intraoperative fluid administration, blood transfusion and vasopressor requirements, time to extubation, and length of stay. RESULTS: No epidural hematomas or abscesses were reported. Two patients (0.9 %) had delays in epidural removal because of thrombocytopenia, and two had epidural-site erythema (0.9 %). The majority of postoperative epidural-related hypotensive episodes were successfully treated with fluid boluses. Early initiation of epidural analgesic infusions (before HIPEC) was associated with significantly less surgical blood loss and fluid requirements (P = 0.005 and 0.02, respectively). Pre-HIPEC initiation of epidural infusions was not associated with a statistically significant difference in the following: volume of blood transfused, intraoperative vasopressors use, time to extubation, and length of hospital stay. CONCLUSIONS: With close hematologic monitoring and particular attention to sterility, epidural analgesia can be safely provided to patients undergoing CRS with HIPEC. Early initiation of continuous epidural infusions during surgery could lead to decreased blood loss and less intraoperative fluid administration. Prospective randomized studies are required to further investigate these potential benefits.

Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. METHODS: A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). RESULTS: Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). CONCLUSIONS: HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

Peritoneal sarcomatosis in pediatric malignancies.

Peritoneal sarcomatosis (PSC) is defined as peritoneal involvement of multiple sarcomatous tumors. Desmoplastic small round cell tumors (DSRCT) and rhabdomyosarcomas are the most common pediatric PSC cases. PSC has been treated with chemotherapy and mainly palliative surgery, but long-term outcome has been poor. New imaging technologies have improved the evaluation of disease extent and patterns of peritoneal dissemination, and cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is being evaluated as a treatment option to prolong remission in pediatric patients. We will review the clinical characteristics, potential biologic mechanisms, radiographic characteristics, and potential therapies for pediatric PSC patients.

Unusual cases: melanomatosis and nephroblastomatosis treated with hyperthermic intraperitoneal chemotherapy.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a well-established therapy for carcinomas, mesotheliomas, and some sarcomas. However, HIPEC has not been reported in melanomatosis or nephroblastomatosis. Here we present 2 unique cases of cytoreductive surgery and HIPEC. Cytoreductive surgery followed by HIPEC was performed in a 4-year old with melanomatosis and a 12-year old with nephroblastomatosis. A 4-year-old girl presented with leptomeningeal melanoma that metastasized from a congenital nevus. She had a ventricular peritoneal shunt placed as an infant. Melanomatosis involving the meninges and peritoneal surfaces was identified by positron emission tomographic scan imaging. Extensive plaques of melanoma were removed at the time of surgery, followed by HIPEC. She had no significant postoperative complications. Her abdomen remained free of disease 7 months after treatment. A 13-year-old girl presented with a Wilms' tumor at age 10 years. She then presented to us at the second local recurrence with multiple intraabdominal tumor implants (ie, nephroblastomatosis). We performed cytoreductive surgery followed by HIPEC. She had no postoperative complications and, at 1 year post-HIPEC, had no abdominal recurrence. We conclude that HIPEC can be performed safely for rare diseases such as melanomatosis and nephroblastomatosis. Although abdominal disease control can be realized, further systemic treatment options are necessary. A review of indications for HIPEC is included.

Anesthetic management and renal function in pediatric patients undergoing cytoreductive surgery with continuous hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin.

BACKGROUND: Extensive tumor implants secondary to sarcoma, sarcomatosis, or mesothelioma in children is rare. We conducted the first phase 1 trial of escalating doses of cisplatin during hyperthermic intraperitoneal chemotherapy (HIPEC) in children with sarcomatosis. The most devastating complication of cisplatin therapy is nephrotoxicity. Here we present the anesthetic management and analysis of the impact of intraoperative fluid management on the incidence of renal failure. METHODS: Of the 10 patients under 18 years of age who underwent HIPEC in the context of our phase 1 trial, six patients were under the age of 10 years. We reviewed the anesthetic management, intraoperative fluid and blood administration, and postoperative renal function in these patients. RESULTS: The average age and weight were 6 years and 20.9 kg, respectively. To avoid renal toxicity, urine output was maintained at an average of 3 ml/kg/h. Crystalloid and colloid were transfused at an average rate of 9 ml/kg/h. Percentage increase in creatinine postoperatively varied from 33 to 500 %. Volume of fluid administered did not correlate with percentage increase in creatinine. All patients had a temporary increase in their serum creatinine, but none required dialysis. CONCLUSIONS: Fluid administration at an average rate of 9 ml/kg/h was required to maintain satisfactory urine output. This rate of intraoperative fluid administration is similar to what is provided to adult HIPEC patients. There was no significant correlation in the volume or type of fluid delivered and the increase in serum creatinine. More studies are needed to determine optimal fluid management in children undergoing HIPEC with cisplatin.

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.

The diagnosis and management of desmoplastic small round cell tumor: a review.

PURPOSE OF REVIEW: Desmoplastic small round cell tumor (DSRCT) is a rare disease of children, adolescents and young adults, which begins in the abdominal cavity. Less than 200 cases are reported in the world literature. Because of the rarity of this disease, little is known about optimal treatment. Patients may present with dozens to hundreds of tumors studding the peritoneal cavity. Despite this presentation, it is not primarily considered metastatic but multifocal. It can metastasize to the liver or lung. Chemotherapy, radiotherapy, and surgical approaches have not been standardized. Neoadjuvant chemotherapy often yields a partial response; however, tumors may remain surgically un-resectable. An aggressive approach to treatment is required to maximize long-term remission. This review is designed to outline the evidence-based multidisciplinary approach to DSRCT. RECENT FINDINGS: Complete surgical resection, including 1-2 mm implants, is necessary to achieve long-term disease control. Hyperthermic intraperitoneal chemotherapy using cisplatin has recently been found to be a low morbidity treatment option for DSRCT patients. Yttrium microspheres have been used successfully to treat liver metastasis from DSRCT. SUMMARY: DSRCT is a rare tumor that requires a multidisciplinary approach which includes aggressive surgical extirpation to provide long-term disease control.

Childbearing after hyperthermic intraperitoneal chemotherapy: results from an international survey.

BACKGROUND: Cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) can improve survival in selected patients with primary or secondary peritoneal malignancies. With the opportunity for long-term survival, questions about the impact of those procedures in fertility in women of childbearing age can be raised. MATERIALS AND METHODS: An international survey was performed among all teams participating in the International Peritoneal Surface Malignancy Group in order to collect data about pregnancies and their outcome in women having undergone previous CRS with adjuvant HIPEC. RESULTS: There were 7 pregnancies reported after CRS and HIPEC in women treated for peritoneal malignancies. All these women conceived spontaneously, most of them within 2 years after the procedure. They delivered most often by vaginal way after an uneventful pregnancy. Their newborns were healthy, except 1 case of congenital diaphragmatic hernia requiring emergent surgery. There were 2 additional uneventful pregnancies reported after the diagnosis of pseudomyxoma peritonei and before CRS and HIPEC, with the support of the medical team. Another woman having undergone oocytes retrieval and embryo cryopreservation prior to the surgery was mother of twins after the procedure via a surrogate mother. CONCLUSION: Childbearing after cytoreductive surgery and heated intraperitoneal chemotherapy is possible in women conserving their genital organs after the procedure. The question of fertility should be considered and discussed in women in reproductive age prior to cytoreductive surgery and heated intraperitoneal chemotherapy. Different options could be offered in this setting. Multidisciplinary decision making involving surgical oncologists and fertility specialists is important.