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.

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.

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.

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.

Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997.

BACKGROUND: We reviewed 56 IRS-IV patients with localized rhabdomyosarcoma [RMS] of the retroperitoneum/pelvis to assess outcome and prognostic factors, including the value of initially excising >or=50% of the tumor (debulking) before chemotherapy. METHODS: Patients had embryonal RMS [N=38], alveolar RMS [N = 7], RMS not otherwise specified [NOS, N = 7], or undifferentiated sarcoma [N = 4]. Fifteen patients were debulked; 41 patients were biopsied. All received VAC; most received radiotherapy. RESULTS: Estimated 5-year failure-free survival [FFS] and overall survival rates were 70 and 75%, respectively. FFS rates were better for patients <10 years old and those with embryonal RMS compared to alveolar RMS/undifferentiated sarcoma. After adjusting for age and histological differences, FFS was better for patients whose tumor was debulked prior to beginning therapy [P = 0.02]. CONCLUSIONS: These results are superior to those of previous protocols for patients with RMS of the retroperitoneum/pelvis. Initial excision of >or=50% of the tumor may be associated with increased FFS.

Surgical management of gynecologic rhabdomyosarcoma.

The multidisciplinary treatment strategy for rhabdomyosarcoma (RMS) of the female gynecologic system is evidence of the progress made in the management of many childhood cancers. Previously, initial radical surgery (eg, complete vaginectomy, hysterectomy, and pelvic exenteration) and less effective single-agent chemotherapeutic agents were used with suboptimal results. Over the past 30 years, there has been an increase in overall survival with gynecologic RMS and a significant decrease in the hysterectomy and vaginectomy rates. The usual recommended treatment strategy for gynecologic RMS is initial biopsy to establish the diagnosis, followed by neoadjuvant chemotherapy. The specific chemotherapy regimen is selected based on the risk stratification of the particular patient, and this will often allow a more conservative surgical approach to be used, with the goal of complete tumor resection. Radiation therapy is considered before definitive surgical resection with minimal response to chemotherapy or after definitive surgical resection with positive margins. Recurrent or persistent RMS, including RMS of the female gynecologic system, represents a very aggressive disease with poor outcome. In these cases, aggressive management, including experimental chemotherapy agents and organ-sacrificing surgery, is often indicated to afford any chance of long-term survival. Continued enrollment of a high proportion of these patients into ongoing prospective clinical trials should allow the outcomes to continue to improve.

Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997.

BACKGROUND/PURPOSE: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines. METHODS: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement. RESULTS: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome. CONCLUSIONS: Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended.

Advances in the surgical management of sarcomas in children.

The management of children with soft tissue sarcomas has changed and survival has improved markedly during the past three decades. This is, in large part, due to the multi-modal approach to pediatric malignancies; decreasing the need for radical surgical procedures, while improving survival and decreasing morbidity. The management of patients with rhabdomyosarcomas (RMS) and nonrhabdomyosarcomas soft-tissue sarcomas (NRSTS) as well as neurofibromatosis and related tumors is best accomplished in an organized and systematic fashion with the surgeon involved early in the planning, diagnostic, and treatment phases. Cooperative trials for rhabdomyosarcoma, as part of the Intergroup Rhabdomyosarcoma Study, have led to improved survival with less radical surgery. Ongoing trials of the Soft Tissue Sarcoma Group of the Children's Oncology Group are under way. Since these tumors are rare, cooperative trials are essential to answering the remaining questions regarding extent of surgery and adjuvant therapy.