Clinical assessment, radiographic imaging, and patient self-report for abdominal wall hernias.

BACKGROUND: Increasingly, abdominal wall hernias are being diagnosed incidentally through radiographic imaging. Such hernias are referred to as occult. However, the clinical significance of occult hernias is unknown. The objective of this study is to determine the prevalence of occult hernias and to assess the abdominal wall quality of life (AW-QOL) among patients with occult hernias. MATERIALS AND METHODS: A blinded, observational, cross-sectional study, October-December 2016, of patients presenting to single academic institution's general surgery clinics was performed. Inclusion criteria included all patients with a computed tomography scan of the abdomen or pelvis within the last year with no intervening abdominal or pelvic surgery. Patients were administered a validated AW-QOL survey and underwent a standardized clinical examination. Computed tomography scans were reviewed. Primary outcomes were prevalence and AW-QOL measured by the modified Activities Assessment Scale. AW-QOL of patients with no hernias was compared to that of those with occult hernias and clinically apparent hernias using Mann-Whitney U test. RESULTS: A total of 250 patients were enrolled of whom 97 (38.8%) had a hernia noted on clinical examination and 132 (52.8%) had a hernia noted on radiographic imaging. The prevalence of occult hernias was 38 (15.2%). Patients with no hernia had a median (interquartile range) AW-QOL of 82.5 (55.0-95.3), patients with clinically apparent hernias had AW-QOL of 47.7 (31.2-81.6; P < 0.001), and patients with occult hernias had AW-QOL of 72.4 (38.5-97.2; P = 0.36). CONCLUSIONS: Both clinically apparent and occult hernias are prevalent. However, only patients with clinically apparent hernias had differences in AW-QOL when compared to patients with no hernias. Prospective trials are needed to assess the outcomes of patients with occult hernias managed with and without surgical repair.

Component separation for complex congenital abdominal wall defects: not just for adults anymore.

PURPOSE: Operative repair of large abdominal wall defects in infants and children can be challenging. Component separation technique (CST) is utilized in adults to repair large abdominal wall defects but rarely used in children. The purpose of this report is to describe our experience with the CST in pediatric patients including the first description of CST use in newborns. METHODS: After IRB approval, we reviewed all patients who underwent CST between June 1, 2010 and December 31, 2012 at a large children's hospital. CST included dissection of abdominal wall subcutaneous tissue from the muscle and fascia and an incision of the external oblique aponeurosis one centimeter lateral to the rectus sheath. Biologic mesh onlay or underlay was used to reinforce this closure. Patients were followed for complications. RESULTS: Nine children, two patients with gastroschisis and seven with omphalocele, were repaired with CST at median (range) 1.1 years (5 days-10.1 years) of age. CST was the first surgical intervention for five children. There were minor wound complications and no recurrences after a median (range) follow up of 16 months (3-34 months). CONCLUSION: CST can be a very useful technique to repair large abdominal wall defects in children with a loss of abdominal domain.

Location of pulmonary metastasis in pediatric osteosarcoma is predictive of outcome.

BACKGROUND: The 3-year survival after pulmonary metastasectomy for osteosarcoma (OS) is approximately 30%. Resection of metastatic disease can prolong life in pediatric patients with OS. Our objective is to assess the outcome of pediatric patients with pulmonary metastases located centrally as compared with peripheral lesions. METHODS: A retrospective review of patients 0 to 21 years old with a diagnosis of OS with pulmonary metastases on computed tomographic scan between 1985 and 2000 was completed. Demographics, metastasis location, survival, morbidity, and mortality were evaluated. RESULTS: Of 115 patients who had pulmonary metastasis secondary to OS, there were 96 wedge resections and 13 lobectomy/pneumonectomies in 84 patients. The morbidity of wedge resection was 9% and lobectomy/pneumonectomy was 8%. There were no deaths from surgery. The median survival for patients undergoing lobectomy compared with wedge resection was 0.61 and 1.14 years, respectively, but did not reach statistical significance. The median overall survival for the entire cohort was 0.75 years. The median overall survival after initial detection of metastatic disease was 1.06 years among the patients with peripheral disease, compared with 0.38 years in patients with central disease (P = .008). CONCLUSION: Patients with central pulmonary metastases in OS have a very poor prognosis, even after operative treatment, compared with those with peripheral disease. Patients with central lesions may benefit from other nonsurgical treatment options.

An evidence-based review of a Lentinula edodes mushroom extract as complementary therapy in the surgical oncology patient.

The purpose of this review is to present the currently published evidence regarding the use, efficacy, potential mechanisms of action, and results of published clinical trials regarding the use of a Lentinula edodes mushroom-derived extract (active hexose correlated compound) as complementary therapy in patients with cancer. The authors explore the current preclinical and clinical evidence as it relates to this topic and its potential use in the surgical oncology patient. There has been a growing interest in stimulation of the immune system in trauma, cancer, and surgical patients in general. Little, however, has been written about some-of the supplements in widely used in Japan and China, but relatively unheard of in the United States.

Results of the Intergroup Rhabdomyosarcoma Study Group D9602 protocol, using vincristine and dactinomycin with or without cyclophosphamide and radiation therapy, for newly diagnosed patients with low-risk embryonal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

PURPOSE: Patients with localized, grossly resected, or gross residual (orbital only) embryonal rhabdomyosarcoma (ERMS) had 5-year failure-free survival (FFS) rates of 83% and overall survival rates of 95% on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III/IV. IRSG D9602 protocol (1997 to 2004) objectives were to decrease toxicity in similar patients by reducing radiotherapy (RT) doses and eliminating cyclophosphamide for the lowest-risk patients. PATIENTS AND METHODS: Subgroup A patients (lowest risk, with ERMS, stage 1 group I/IIA, stage 1 group III orbit, stage 2 group I) received vincristine plus dactinomycin (VA). Subgroup B patients (ERMS, stage 1 group IIB/C, stage I group III nonorbit, stage 2 group II, stage 3 group I/II) received VA plus cyclophosphamide. Patients in group II/III received RT. Compared with IRS-IV, doses were reduced from 41.4 to 36 Gy for stage 1 group IIA patients and from 50 or 59 to 45 Gy for group III orbit patients. RESULTS: Estimated 5-year FFS rates were 89% (95% CI, 84% to 92%) for subgroup A patients (n = 264) and 85% (95% CI, 74%, 91%) for subgroup B patients (n = 78); median follow-up: 5.1 years. Estimated 5-year FFS rates were 81% (95% CI, 68% to 90%) for patients with stage 1 group IIA tumors (n = 62) and 86% (95% CI, 76% to 92%) for patients with group III orbit tumors (n = 77). CONCLUSION: Five-year FFS and OS rates were similar to those observed in comparable IRS-III patients, including patients receiving reduced RT doses, but were lower than in comparable IRS-IV patients receiving VA plus cyclophosphamide. Five-year FFS rates were similar among subgroups A and B patients.

Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991-1997: a report from the children's oncology group.

PURPOSES: The aims of the study were to compare results of clinical/radiographic studies before second-look procedures (SLP) with SLP specimens from patients with gross residual sarcoma at diagnosis and to relate tumor viability to outcome. PATIENTS: Seventy-three patients underwent SLP before completing chemotherapy, with (n = 59) or without (n = 14) radiotherapy. Tumor sites were bladder/prostate (n = 27), head/orbit/parameningeal (n = 22), extremity/trunk (n = 14), and retroperitoneum/pelvis (n = 10). RESULTS: Of 14 patients, 1 (7%) with clinical/radiographic complete response (CR) had viable tumor. Of 59 patients, 35 (59%) without CR had viable tumor. Five-year failure-free survival (FFS) rates were 81% in 37 patients without viable tumor and 53% in 36 patients with viable tumor (Cox proportional hazards adjusted P = .05). Five-year FFS rates were 67% in 15 patients with clear margins and 43% in 21 patients with tumor-involved margins (n = 18) or viable gross tumor (n = 3) (Cox proportional hazards adjusted P = .04). Five-year survival was 78% to 79% among 73 patients with and 333 patients without SLP during treatment. CONCLUSIONS: Second-look procedures can show whether viable tumor is present and may be beneficial in selected patients with rhabdomyosarcoma. Disappearance of tumor (CR) usually correlated with no viable tumor at SLP. However, 41% of patients without CR had no viable tumor. Those without viable tumor had increased FFS but not survival compared to those with viable tumor.

Age as a prognostic factor for patients with osteosarcoma: an analysis of 438 patients.

BACKGROUND: It is unclear whether age at diagnosis is an important prognostic factor in patients with osteosarcoma. Understanding this relationship could yield valuable insight into therapeutic rationale, focus patient selection for clinical trials, advance molecular concepts and theories, and expand current principles guiding prognosis. Our aim was to understand if age at diagnosis is a prognostic indicator for eventual outcome, as measured by disease-free survival and overall survival in patients with osteosarcoma. METHODS: Our cohort consisted of 438 patients of all ages who were diagnosed with osteosarcoma between 1 January 1980 and 31 December 2000 and who underwent the majority of their treatment at M.D. Anderson Cancer Center (MDACC). Patient and tumor specific variables were collected including patient demographics, patient history, primary tumor information (i.e., location, size, histology, extension, necrosis, etc.), treatment strategy (i.e., surgery, chemotherapy, and/or radiotherapy), metastatic disease information, long-term follow-up, and eventual outcome. Statistical analyses, including univariate and multivariate analyses were performed, with overall survival and disease-free survival as the primary outcome measures. RESULTS: The median age at diagnosis was 18.1 years (range 2 months to 78.8 years). Median follow-up was 4.2 years (range 5 days to 22.8 years) for all patients and 12.3 years (range 1 month to 22.8 years) for 209 surviving patients. Survival rates at 5, 10, and 15 years were 54.1, 47.2, and 45.2%, respectively. On univariate analyses, age >or= 40 was found to be a poor prognostic factor. Other prognostic factors included tumor size, metastasis at diagnosis, soft-tissue tumor extension, surgery type, chemotherapy group, and tumor necrosis. Age was not identified a statistically significant prognostic variable on multivariate analysis. CONCLUSIONS: Age at diagnosis does not appear to be a significant independent prognostic variable for overall survival or disease-free survival in patients with osteosarcoma. Although our data indicate that patients in the fifth decade and older fare worse than younger patients, other variables such as tumor necrosis, tumor extension, and tumor location are likely responsible for the observed decline in overall survival and disease-free survival.

Outcomes of truncal vascular injuries in children.

BACKGROUND: Pediatric truncal vascular injuries occur infrequently and have a reported mortality rate of 30% to 50%. This report examines the demographics, mechanisms of injury, associated trauma, and outcome of patients presenting for the past 10 years at a single institution with truncal vascular injuries. METHODS: A retrospective review (1997-2006) of a pediatric trauma registry at a single institution was undertaken. RESULTS: Seventy-five truncal vascular injuries occurred in 57 patients (age, 12 +/- 3 years); the injury mechanisms were penetrating in 37%. Concomitant injuries occurred with 76%, 62%, and 43% of abdominal, thoracic, and neck vascular injuries, respectively. Nonvascular complications occurred more frequently in patients with abdominal vascular injuries who were hemodynamically unstable on presentation. All patients with thoracic vascular injuries presenting with hemodynamic instability died. In patients with neck vascular injuries, 1 of 2 patients who were hemodynamically unstable died, compared to 1 of 12 patients who died in those who presented hemodynamically stable. Overall survival was 75%. CONCLUSIONS: Survival and complications of pediatric truncal vascular injury are related to hemodynamic status at the time of presentation. Associated injuries are higher with trauma involving the abdomen.

Rhabdomyosarcoma in children.

PURPOSE OF REVIEW: Rhabdomyosarcoma is a rare childhood cancer that affects only approximately 300 children per year in the United States. The purpose of this review is to provide the reader a greater understanding of the complex diagnosis, assessment and treatment of rhabdomyosarcoma in children. RECENT FINDINGS: This review focuses on the new risk classification that is the foundation of all present rhabdomyosarcoma protocols developed by the Children's Oncology Group of the United States and Canada. The new risk classification of low, intermediate and high encompasses the staging and grouping categories that were previously utilized. SUMMARY: This review also provides a complete list of diagnostic tests and imaging required to identify rhabdomyosarcoma in any body site. Rapid diagnosis and recognition of this rare disorder will facilitate long-term survival. Rhabdomyosarcoma today has an overall survival of 70%, depending on the site, and in orbital and other sites survival is as high as 90%. The treatment approaches that have led to this doubling in survival over the last 25 years are reviewed. For a practitioner, this review can be used as a reference when a child with a suspicious mass is encountered.

Acute hemodynamic decompensation following patent ductus arteriosus ligation in premature infants.

INTRODUCTION: Patent ductus arteriosus (PDA) ligation can lead to postoperative hemodynamic instability requiring inotropic support, termed hemodynamic decompensation. The purpose of this study was to prospectively determine the incidence, predictors, and clinical impact of hemodynamic decompensation after PDA ligation in preterm infants. METHODS: All infants undergoing PDA ligation were eligible for this prospective cohort study. After undergoing ligation, patients were followed until 30 days after successful extubation, discharge from the NICU, or death. Data collection included perinatal and preoperative clinical information, operative details, postoperative course, and outcome. RESULTS: Ninety-six preterm infants were enrolled and underwent PDA ligation. Hemodynamic decompensation occurred in 27 patients (28%). Overall in-hospital mortality rate was 18%. Mortality was significantly higher among infants that developed hemodynamic decompensation (33% vs 11%, p = .012). Hemodynamic decompensation was associated with an adjusted odds ratio (OR) for death of 3.1 (95% confidence interval: 1.0-9.5, p = .05). Lower estimated gestational age, lower corrected age, and higher rate of preoperative mechanical ventilation were significant predictors of hemodynamic decompensation. CONCLUSION: Hemodynamic decompensation occurred in 28% of patients after PDA ligation, resulting in a significantly higher mortality. Younger patients requiring higher ventilator support are most likely to develop hemodynamic decompensation.

Melanoma growth and tumorigenicity in models of microgravity.

INTRODUCTION: Spaceflight involves numerous biological stressors that could affect long-term cancer incidence and tumor behavior. Ground-based models of microgravity can be used to investigate in vitro and in vivo tumor growth as a preparation for later work in space. The incidence of tumor growth and carcinogenesis in microgravity is as yet unknown. Hence, we investigated the effects of modeled microgravity on tumor growth and tumorigenicity using ground-based in vitro and in vivo models. METHODS: Murine B16-F10 melanoma cells were cultured in a tissue culture flask (FL) and in a rotating-wall vessel bioreactor (BIO) designed by NASA to simulate some aspects of microgravity. We then measured cell growth, melanin production, and apoptosis. After 48 h of cultures in FL and BIO, cells were inoculated subcutaneously in C57BL/6 mice, syngeneic hosts for B16-F10 tumor cells. Tumor sizes were then measured every other day. RESULTS: BIO cultures had 50% decreases in growth when compared with FL cultures while demonstrating an inversely proportional increase in doubling time. Melanin production (a marker of differentiation) increased at 24 and 48 h in BIO. Flow cytometry analysis demonstrated that there was an increase in the percentage of apoptotic cells in the BIO when compared with that in the FL. When BIO-cultured melanoma cells were inoculated subcutaneously in mice, there was a significant increase in tumorigenicity as compared with FL-cultured cells. CONCLUSION: Our results indicate that simulated microgravity may have altered the tumor cell characteristics and enhanced the invasive property. It is possible that the microgravity analogue culture environment may have selected highly tumorigenic cells for survival despite the decreased overall growth in the microgravity analogue.

Outcome after surgical resection of recurrent rhabdomyosarcoma.

BACKGROUND/PURPOSE: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Patients with localized disease have a cure rate of 50% to 90%; however, there has been little evidence that aggressive surgical resection for recurrent disease is of benefit. We reviewed our experience with aggressive surgical resection for recurrent RMS. METHODS: A retrospective review of the records for patients with RMS was performed. Data extracted included tumor site, histology, initial therapy, time to recurrence, treatment, and outcomes. RESULTS: From 1991 to 2002, 122 patients with RMS (3 months-18 years) were treated at the MD Anderson Cancer Center. Of 32 patients with recurrent RMS, 19 had surgical resection and 13 had biopsy only or no resection. The common primary sites included extremity (12), genitourinary nonbladder/prostate (7), and retroperitoneal/trunk (7). In the resection group, 33 operations were performed with 5 (15%) major complications and no deaths. Seventeen (52%) of these procedures (7 pelvic, 5 thoracic, 3 amputations, and 2 cranial) were classified as aggressive. After a mean follow-up period of 4.9 years, 7 patients (37%) had no evidence of disease, 8 (42%) died, and 4 were lost to follow-up. There was no correlation between survival and the type of resection. In the no-resection group only, 1 (8%) of 13 patients survived. CONCLUSIONS: Despite morbidity, aggressive surgical resection is warranted to improve survival in patients with recurrent RMS.

Long-term survival after aggressive resection of pulmonary metastases among children and adolescents with osteosarcoma.

PURPOSE: Although survival without resection of pulmonary metastases from osteosarcoma is unlikely, not all surgeons agree on an aggressive surgical approach. We have taken an approach to attempt surgical resection if at all feasible regardless of number of metastases and disease-free interval (DFI). This study presents information on long-term follow-up after this aggressive approach to resection. METHODS: A single-institution retrospective cohort study of osteosarcoma patients younger than 21 years with pulmonary metastases, limited to the contemporary chemotherapeutic period (1980-2000), was conducted. RESULTS: In 137 patients, synchronous (23.4%) or metachronous (76.6%) pulmonary nodules were identified. The median follow-up was 2.0 years (5 days to 20.1 years) for all patients. Overall survival among patients who had pulmonary nodules was 40.2% and 22.6% at 3 and 5 years, respectively. Ninety-nine patients underwent attempted pulmonary metastasectomy (mean survival, 33.6 months; 95% confidence interval, 25.1-42.1) and 38 patients did not (mean survival, 10.1 months; 95% confidence interval, 6.5-13.6; P < .001, t test). Characteristics that were associated with an increased likelihood of 5-year overall survival after pulmonary resection were primary tumor necrosis greater than 98% after neoadjuvant chemotherapy (P < .05) and DFI before developing lung metastases more than 1 year (P < .001). No statistically significant difference in overall survival or disease-free survival was found based on the number of pulmonary metastases resected. Characteristics including primary tumor size, site, or extension; chemotherapy; early vs late metastases; unilateral vs bilateral metastases; and resection margins did not significantly affect survival. CONCLUSIONS: Most patient and tumor characteristics commonly used by surgeons to determine utility of resection of pulmonary metastases among patients with osteosarcoma are not associated with outcome. Biology of the particular tumor (response to preoperative chemotherapy, measured by tumor necrosis percentage, and DFI), as opposed to tumor burden, appears to influence survival more significantly. We would advocate considering repeat pulmonary resection for patients with recurrent metastases from osteosarcoma.

Flow cytometry and fluorescence in situ hybridization to detect residual neuroblastoma cells in bone marrow.

BACKGROUND: In patients with neuroblastoma morphological assessment of BM for residual NB cells is not precise, particularly when the number of tumor cells is small. PROCEDURE: To develop a sensitive and rapid method of detecting NB cells in BM, we assessed the efficiency of flow cytometry (FCM) using markers CD9, CD56, and CD45. The percent of CD9+/CD56+/CD45- (NB phenotype) cells was determined by FCM in 41 samples (16 patients) at various time points. For confirmation fluorescence in situ hybridization (FISH) for 17q gain was performed. RESULTS: Nineteen of the 22 (86%) samples that were negative by morphology were positive by FCM (>0.006% CD9+/CD56+/CD45- cells). The longest time to complete the FCM study was 3 hr. In six FISH experiments the sorted CD9+/CD56+/CD45- population had a higher percentage of cells with 17q gain (11.5-95%) compared to a CD56-/CD45+ internal control population (2-8%). CONCLUSIONS: Our preliminary results suggest that FCM determination of the percent of CD9+/CD56+/CD45- cells is an effective method of rapidly detecting NB cells in BM.

Characteristics and outcomes of rhabdomyosarcoma patients with isolated lung metastases from IRS-IV.

PURPOSE: To better understand outcomes in children with rhabdomyosarcoma (RMS) and lung-only metastatic disease, the authors reviewed the experience from Intergroup Rhabdomyosarcoma Studies IV Pilot and IV. METHODS: Patients with lung-only (n = 46) vs other sites of metastatic disease (n = 234) were reviewed using patient charts and the database of Children's Oncology Group (COG). RESULTS: Sixteen percent of patients with RMS and metastatic disease had isolated lung metastases. Thirty-one (67%) had more than 5 metastatic lung lesions. These were bilateral in 34 (74%). Only 6 patients were biopsied at diagnosis. Sixteen children (35%) did not receive any lung radiotherapy. Patients that received lung radiotherapy had fewer lung recurrences ( P = .04), although this has no significant impact on overall survival (OAS, 47% radiotherapy vs 31% no radiotherapy). Compared with patients with other sites of metastatic disease, patients with lung-only metastases have a greater proportion of favorable histology (67% vs 39%, P = .0017), negative nodal involvement (67% vs 32%, P = .0013), and parameningeal primaries (39% vs 12%) and a smaller proportion of extremity primaries (20% vs 33%, P = .0005 for site of primary tumor). Overall survival at 4 years for lung-only metastases was not significantly different from other single-site metastasis (42% vs 34%). Survival was not improved for unilateral disease or fewer than 5 metastatic lesions. Factors associated with diminished OAS include unfavorable histology (P = .0001) and age >10 years (P = .015). CONCLUSIONS: Children with RMS and lung-only metastases usually present with extensive bilateral disease that is frequently not biopsied nor given protocol-recommended radiotherapy (XRT). However, outcome is comparable, although slightly better, than patients with other single-site metastasis.

Treatment issues in pediatric gastric adenocarcinoma.

Gastric adenocarcinoma is a rare diagnosis in children. Modern treatment includes chemotherapy, radiotherapy, and surgery and has evolved over the past decade. The authors report a case of an 8-year-old girl with gastric adenocarcinoma. The case will be followed by a brief discussion of the presentation, current management guidelines, and prognosis of this rare pediatric entity.