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Survival in patients who have Ewing sarcoma is correlated with postchemotherapy response (tumor necrosis). This treatment response has been categorized as the response rate, similar to what has been used in osteosarcoma. There is controversy regarding whether this is appropriate or whether it should be a dichotomy of complete versus incomplete response, given how important a complete response is for in overall survival of patients with Ewing sarcoma. The purpose of this study was to evaluate the impact that the amount of chemotherapy-induced necrosis has on (1) overall survival, (2) local recurrence-free survival, (3) metastasis-free survival, and (4) event-free survival in patients with Ewing sarcoma.In total, 427 patients who had Ewing sarcoma or tumors in the Ewing sarcoma family and received treatment with preoperative chemotherapy and surgery at 10 international institutions were included. Multivariate Cox proportional-hazards analyses were used to assess the associations between tumor necrosis and all four outcomes while controlling for clinical factors identified in bivariate analysis, including age, tumor volume, location, surgical margins, metastatic disease at presentation, and preoperative radiotherapy.Patients who had a complete (100%) tumor response to chemotherapy had increased overall survival (hazard ratio [HR], 0.26; 95% CI, 0.14-0.48; p .01), recurrence-free survival (HR, 0.40; 95% CI, 0.20-0.82; p = .01), metastasis-free survival (HR, 0.27; 95% CI, 0.15-0.46; p ≤ .01), and event-free survival (HR, 0.26; 95% CI, 0.16-0.41; p ≤ .01) compared with patients who had a partial (0%-99%) response.Complete tumor necrosis should be the index parameter to grade response to treatment as satisfactory in patients with Ewing sarcoma. Any viable tumor in these patients after neoadjuvant treatment should be of oncologic concern. These findings can affect the design of new clinical trials and the risk-stratified application of conventional or novel treatments.

Respirable coal mine dust (RCMD) exposure is one of the utmost health hazards to the mining community causing various health issues, including coal worker pneumoconiosis (CWP). Considering multiple potential sources of RCMD having different physicochemical properties within the same mine suggests a wide range of health impacts that have not yet been studied extensively. In this work, we investigate the toxicity of lab-created RCMD based on different sources: coal seam, rock dust, host floor, and host roof collected from the same mine. Comparative samples obtained from several mines situated in various geographic locations were also assessed. This work quantifies metal leaching in simulated lung fluids and correlates dissolution with in vitro immune responses. Here, dissolution experiments were conducted using two simulated lung fluids; Gamble solution (GS) and artificial lysosomal fluid (ALF). In vitro studies were performed using a lung epithelial cell line (A549) to investigate their immune responses and cell viability. Si and Al are the most dissolved metals, among several other trace metals, such as Fe, Sr, Ba, Pb, etc. RCMD from the coal seam and the rock dust showed the least metal leaching, while the floor and roof samples dissolved the most. Results from in vitro studies showed a prominent effect on cell viability for floor and roof dust samples suggesting high toxicity.

Coal mine workers are continuously exposed to respirable coal mine dust (RCMD) in workplaces, causing severe lung diseases. RCMD characteristics and their relations with dust toxicity need further research to understand the adverse exposure effects to RCMD. The geographic clustering of coal workers’ pneumoconiosis (CWP) suggests that RCMD in the Appalachian region may exhibit more toxicity than other geographic regions such as the Rocky Mountains. This study investigates the RCMD characteristics and toxicity based on geographic location. Dissolution experiments in simulated lung fluids (SLFs) and in vitro responses were conducted to determine the toxicity level of samples collected from five mines in the Rocky Mountains and Appalachian regions. Dust characteristics were investigated using Fourier-transform infrared spectroscopy, scanning electron microscopy, the BET method, total microwave digestion, X-ray diffraction, and X-ray photoelectron spectroscopy. Inductively coupled plasma mass spectrometry was conducted to determine the concentration of metals dissolved in the SLFs. Finer particle sizes and higher mineral and elemental contents were found in samples from the Appalachian regions. Si, Al, Fe, Cu, Sr, and Pb were found in dissolution experiments, but no trends were found indicating higher dissolutions in the Appalachian region. In vitro studies indicated a proinflammatory response in epithelial and macrophage cells, suggesting their possible participation in pneumoconiosis and lung diseases development.

This is a case report that includes an analysis about extrapulmonary tuberculosis and his singularities, cause can be confused with cancer. Our patient an 83-year-old woman from Pasto-Colombia presents a mimicking metastasis with melanoma. This guides us to understand that extrapulmonary tuberculosis is a rare pathology, but it should be considered as a potential differential diagnosis of any osteolytic lesion. That is the reasons for to be one of the great imitators in medicine, we come up with are totally necessary in a differential diagnosis with malignancies, a high index of suspicion.

Background and objectives Mechanical augmentation with metal implants in the treatment of bone tumors limits magnetic resonance imaging and computerized tomography scan imaging due to the scattering effect. In addition, modulus of elasticity differences may cause chronic pain. We present a surgical technique in which cortical allografts are used as plates. This is a two-center experience focusing on functional and radiographic outcomes and complications. Methods Under IRB approval, we performed a retrospective patient review. Demographics, treatment indications, tumor characteristics, and complications were collected. Radiographic studies were reviewed to determine the time to osseous incorporation. The last follow-up functional evaluation included the Musculoskeletal Tumor Society (MSTS) score, the Karnofsky scale, and Eastern Cooperative Oncology Group (ECOG). Results Thirty patients, seventeen female and thirteen male, 16-year-old in average were included. The most common diagnoses were the humeral aneurysmal bone cyst and unicameral bone cyst. The Median follow-up was 6 years. Cortical allograft incorporation median time was 8.8 months whereas allograft void bone incorporation was 3 months. The average time to full weight-bearing without impact activity was 3.1 months. The most common complication was symptomatic hardware. Other complications included a superficial infection successfully treated with antibiotics, a temporary radial nerve neuropraxia, and an allograft fracture requiring revision surgery. Most patients had an MSTS score higher than 90 (excellent). Patients with malignant tumors had ECOG of 0 and a Karnofsky score of 100. Conclusion Biological osteosynthesis is a bone preserving and effective modality for mechanical augmentation with excellent functional results and low complication rates.

Acute compartment syndrome of the hand is a potentially devastating and infrequent condition observed after trauma, arterial injury, or prolonged compression of the upper limb. We present the case of a patient diagnosed with compartment syndrome of the hand after laparoscopic surgery for epithelial ovarian cancer. The patient is a 42-year-old woman with incidental finding of high-grade ovarian serous carcinoma after an emergency surgery. On imaging evaluation, the patient was found to have evidence of residual retroperitoneal adenopathy and was taken to the operating room for a staging procedure by laparoscopy. In the immediate postoperative period, she developed compartment syndrome of the right hand that required multiple fasciotomies and multidisciplinary management by plastic surgery, orthopedics, and rehabilitation medicine. The patient was discharged from the hospital 7 days after laparoscopic surgery to undergo rehabilitation. Three months after surgery, she is continuing to recover, with near complete recovery of hand function. The patient has completed a total of 3 cycles of chemotherapy with carboplatin/paclitaxel. Compartment syndrome of the hand is an uncommon event, but it can generate major functional deficits and even death if it is not diagnosed and treated in a timely manner. Strict criteria for patient positioning in laparoscopy surgery may avoid or reduce this complication. To date, this is the first case reporting such complications associated with laparoscopic gynecologic surgery.

PURPOSE: We retrospectively assessed the incidence of cataracts in patients with retinoblastoma (Rb) treated with either lens-sparing radiation therapy (LSRT) or whole-eye radiation therapy (WERT). A secondary aim of this study was to model the dose-response risk of cataract. METHODS AND MATERIALS: We reviewed 65 patients with Rb treated with radiation therapy (RT) at Children’s Hospital, Los Angeles from 1997 to 2015. Eyes that were enucleated before RT or lacked follow-up eye examinations were excluded. All patients underwent computed tomography simulation, and mean lens dose data were collected. Follow-up ophthalmologic examinations and intraocular lens implant history were reviewed for cataracts. The primary event-free survival (EFS) outcome was cataract development. Eyes without cataracts were censored on the last date of eye examination or post-RT enucleation, if applicable. Kaplan-Meier estimates were used to compare EFS outcomes, and dose response was projected with Cox regression and logistic regression models. RESULTS: Sixty-one patients (94 eyes) were analyzed with a median follow-up of 51.8 months. For eyes treated with WERT, cataracts developed in 71.7% versus 35.3% for LSRT. Median EFS for WERT and LSRT were 20.8 and 67.9 months, respectively. Compared with WERT, a significant EFS benefit was demonstrated for LSRT (P

An 18-year-old male patient with a high-grade osteosarcoma was initially treated with resection and reconstruction using an osteochondral allograft. The allograft collapsed after five years, and thus a revision with a constrained knee prosthesis was performed. After one year, the implant failed due to a fracture, requiring another revision with a new allo-prosthetic composite. The long-term results were satisfactory. Allo-prosthetic composites may offer good long-term results after sarcoma resection. The failure of a massive bone allograft does not preclude the use of another allograft to maintain the bone stock and preserve the function.

Background Multiple myeloma (MM) is an incurable and heterogeneous disease; often presents with skeletal-related events, including pathological fractures. Lytic bone disease is mediated mainly by dysregulation of interleukin 6 (IL-6), osteocalcin production, osteoblastic inhibition, osteoclast activation which prevents fracture healing. MM patients with fractures have a significantly higher risk of dying compared to those without fractures, highlighting the importance of preventing bone disease in MM. Usually, when it has manifested, it is necessary to provide adequate multidisciplinary treatment that could improve quality of life and survival. Methods Records of patients with MM and pathological fractures, requiring surgery were identified through National Cancer Institute of Colombia registry. A total of 799 MM records were reviewed; 10% of them required orthopedic surgery treatment. Thirty-six patients were excluded because loss of to follow up. A descriptive analysis of the clinical characteristics of the patients was carried out, as well as a bivariate analysis of the clinic and the outcomes, and finally, an analysis of overall survival of the entire sample using the Kaplan Meier method and with respect to the time of surgery before or after starting antimyeloma treatment. Results Forty-four patients with MM required surgery, since they had pathological fractures. Median age at diagnosis was 61 years (32-79), 26 were female and 18 were male patients. The most frequent MM subtype among these patients was the producer of immunoglobulin G (IgG) and the associated CRAB component, different from lithic lesions was anemia. A total of 75 fractures were confirmed in the 44 patients. The most frequent site of fractures was the femur, followed by spine and humerus. Twenty-five percent presented with two or more bone segments affected. Median number of days from fracture to surgery was 23 days (2-487). The orthopedic procedure that was mostly performed in the series was the placement of intramedullary nails. Postoperative complications included transfusions (47.72%), ICU requirement (9.09%), and electrolytic disbalances that required correction (9.09%). From the whole complete series, 63.3% of patients received chemotherapy prior to the surgical procedure with a median of 4 cycles. Twelve patients died corresponding to 27.9%, having found an early post-operative mortality in the first month of 4.54% and 12 months of 13.63%. There were no significant differences in survival with respect to the start of chemotherapy before or after surgery, but there was a trending for a better outcome in patients who did not receive preoperative chemotherapy. Conclusions Multiple myeloma remains as an incurable disease to date, frequently presenting skeletal-related events that affect quality of life and survival. There is a high risk of postoperative complications such as requiring intensive care, transfusions, and electrolyte disturbances in these patients. Despite not having found statistically significant differences regarding the start of chemotherapy; before or after the surgical procedure with respect to survival, improvement can be see it, when anti-myeloma therapy has not been started at the time of surgery. Prospective studies are required to elucidate whether there is benefit in delaying chemotherapy until after the patient is brought to fracture correction. Disclosures No relevant conflicts of interest to declare.

Background: Non-severe hemophilia A and B account for 50% of patients with hemophilia, in which factor level does not consistently correlate with bleeding phenotype. Clinical decision-making in regards to timely prophylaxis initiation and tailored surgical management could be informed by a biomarker that is more predictive of bleeding phenotype. We hypothesized that a global method to assess clotting potential, such as thrombin generation (TG), is more predictive of bleeding phenotype than factor level. Objectives: Determine the ability of TG, as compared to standard baseline factor activity, to differentiate bleeding phenotype severity in patients with non-severe hemophilia. Methods: Subjects were recruited from two hemophilia treatment centers (HTCs): Rady Children's Hospital San Diego (RCHSD) and Center for Inherited Blood Disorders (CIBD). Subjects were eligible for enrollment if they were at least one year of age and had a diagnosis of non-severe congenital hemophilia A or B, or were genetically confirmed or suspected female carriers. All enrolled patients or their parents completed the standardized, self-administered pediatric bleeding questionnaire (Self-PBQ) or bleeding assessment tool (Self-BAT). Clinical and laboratory information were extracted from the medical chart, including age at diagnosis, bleeding event history, past surgical history, treatment history and factor VIII or IX gene analysis. Validation of self-reported bleeding symptoms was performed using chart-derived data. Bleeding phenotype was assessed using standard calculation of the bleeding score, as defined by the respective validated self-reported tools. For the purpose of this analysis, we defined a high bleeding score as 13 or more, while a low bleeding score was defined as 12 or less. After a washout period of 5x the standard half-life of the administered factor product, blood samples were collected at time of enrollment. Platelet poor plasma was obtained according to a strict protocol to minimize pre-analytical variables. TG was measured by means of the calibrated automated thrombogram, with three different reagents: low (1 pM of tissue factor [TF]), regular (5 pM of TF) and High (20 pM of TF). The following TG parameters were evaluated: Peak TG (Peak), estimated thrombin potential (ETP) and velocity index (VI). Results: Eighty-one subjects were enrolled. The median age of our cohort was 15.6 years (IQR 21.2, range 4.9 - 59.8), with a slight female predominance (51%) due to inclusion of female carriers. The median follow-up period at the HTC was 5.3 years (IQR 7.5 years), with the majority having follow-up for at least three years. Enrolled patients had the following diagnoses: mild hemophilia A (70%), mild hemophilia B (3%), moderate hemophilia A (13%), moderate hemophilia B (3%), hemophilia A carriers with normal factor VIII levels (10%) and hemophilia B carriers with normal factor IX levels (1%). Median age at diagnosis was 8 years (IQR 26.3, range 0.1 - 56.1), which strongly correlated lower baseline factor activity (r=0.573, p-value < 0.0001). The median baseline factor activity was 21% (IQR 26, range 2 - 249). Factor exposure occurred in 46% of patients (n=37), of which 5 patients were on prophylaxis at time of enrollment. Chronic arthropathy was present in 2 patients (one with mild hemophilia A and one with moderate hemophilia A) and none of the patients had a history of an inhibitor. The majority of patients had a low bleeding score (74% vs. 26%). Baseline factor level and TG values obtained with regular reagent (5 pM of TF) showed significant correlation with bleeding score (r = -0.229 to -0.237, p-value < 0.05), while values obtained with other reagents did not show a significant correlation. Sensitivity/specificity analysis revealed the following optimal cutoff values for differentiating between bleeding severities, as obtained from TG with regular reagent: ETP ( Conclusion: Even though both TG and baseline factor level had comparable correlation with bleeding severity, all TG values with 5 pM TF showed a much higher sensitivity outcome and greater ability to differentiate between bleeding severities in this population. This approach shows potential for predicting bleeding severity in patients with non-severe hemophilia and should be validated in long-term prospective studies. Disclosures Nossair: Novo Nordisk: Other: Conference - Haemophilia Acadamy; Novo Nordisk: Research Funding. Hwang:BPI: Consultancy; Bayer: Consultancy; Hema Biologics: Consultancy; Shire: Consultancy; Bioverativ: Other: PI in clinical research study. Thornburg:ATHN: Research Funding; Bayer Pharmaceuticals: Research Funding; Biomarin: Consultancy; CSL Behring: Research Funding; Bioverativ: Consultancy; Genentech: Speakers Bureau; Octapharma: Research Funding; NovoNordisk: Research Funding; Shire: Research Funding; Johns Hopkins All Children's Hospital: Research Funding; Bluebird Bio: Consultancy.

SUMMARY. Laparoscopic Heller myotomy for achalasia has a 10–20% failure rate and may require re-operation to control persistent or recurrent symptoms. We report follow-up of 15 patients who underwent laparoscopic re-operation for failed Heller myotomy. Between 1993 and 2004, 15 patients underwent laparoscopic re-operation for failed Heller myotomy at our center. The mean duration between procedures was 23 months. Follow-up was completed at a mean duration of 30 months in 14 patients (93%) via a telephone questionnaire. Our overall failure rate for primary surgery (n = 106) was 5.6%. The mechanisms of failure were incomplete myotomy (33%), myotomy fibrosis (27%), fundoplication disruption (13%), too tight fundoplication (7%) and a combination of myotomy fibrosis and incomplete myotomy (20%). Significant symptom improvement was observed with postoperative symptom resolution seen in 71% of patients with dysphagia, 89% for regurgitation, 58% for heartburn and 40% for chest pain. Fifty percent reported excellent results and 79% would recommend the procedure to a friend. Subsequent dilations were performed in four patients (29%). Two patients required conversion to open surgery (13%). Three patients (20%) failed the re-operation and required further revisional surgery. Complications included intraoperative perforation in three (none of which resulted in postoperative morbidity) and a pneumothorax in one patient. Prior endoscopic therapies (pneumatic dilation or Botulinum toxin) were not associated with poor results. Laparoscopic re-operation for failed Heller myotomy is feasible and results are encouraging.

Objective: To assess whether reoperative surgery for failed Nissen fundoplication is beneficial and to classify all mechanisms of failure recognized. Summary Background Data: Antireflux surgery is often necessary, but a 10% failure rate is commonplace. We report results for patients undergoing reoperative surgery and present a nomenclature of mechanisms of failure. Methods: A total of 104 patients, who had a previous fundoplication for gastroesophageal reflux disease (GERD), underwent reoperative surgery. Manometry (n 86), endoscopy (n 101), pH monitoring (n 27), upright esophagram (n 90), gastric emptying (n 26), and symptom assessment (n 104) were performed prior to reoperative surgery. Patients were also assessed before and during reoperation for mechanism of failure using a newly proposed classification. The operative approach was laparoscopic in 58 patients, via open laparotomy in 12, and a thoracotomy in 34 patients. Follow-up was conducted by phone interview and was completed in 97 patients (97%; 3 were deceased) with a mean follow-up of 32 months (range, 1‐146 months). Results: The conversion rate to laparotomy for laparoscopic patients was 8%. The perioperative complication rate was 32%. One patient died of respiratory insufficiency after a laparotomy. Seven patients required additional surgery for correction of persistent or recurrent symptoms. The short and long-term complication rate was similar for the different operative approachs. Symptom resolution (rare or absent) occurred in 74% of patients with dysphagia, 75% with heartburn, 85% with regurgitation, and 94% with chest pain. The overall post-reoperative patient satisfaction was 7 on a scale of 1 to 10 and 3 on a scale of 1 to 4 when patients were asked to grade the operative result. There was no difference in the symptom resolution for patients operated upon by the laparoscopic approach as compared with laparotomy, but those patients undergoing a Collis gastroplasty had poorer results. The preoperative accuracy of assessment for mechanism of failure was 78%. A nomenclature of mechanisms of failure is included to aide reoperative assessment and new mechanisms of failure are described. Conclusion: Reoperative surgery results for GERD are satisfactory. A variety of operative approaches proved equally effective. Poorer results were observed in patients with more advanced disease.

The surgical management results of recurrent hiatal hernia repair are unknown in the laparoscopic era. The experience of the senior authors (CJF) and (SKM) is reported herein. From 1993 to 2004, 52 patients underwent re-operative hiatal hernia surgery at our center. Preoperative symptoms were heartburn, chest pain, dysphagia, regurgitation and pulmonary manifestations of gastroesophageal reflux disease. Patients had preoperative evaluation by upper endoscopy, pH-monitoring, esophagogram and manometry to assess the mechanism of failure. Pre- and postoperative symptoms were assessed utilizing a standardized questionnaire. Patients underwent laparoscopic repair (n=18), open laparotomy (n=6) and transthoracic surgery (n=28). Ninety-five percent follow-up was achieved with a mean follow-up of 34 months. Thirty-seven percent of patients encountered para-operative complications one of them died due to respiratory insufficiency. Five patients experienced a re-recurrent hernia. The symptom resolution was 65% for dysphagia, 68% for heartburn, 95% for chest pain and 79% for regurgitation. The overall patient satisfaction was 6.94 on a scale of 1-10. There was no significant difference in patient outcome when comparing the operative approaches or disease process. Surgical repair of recurrent hiatal hernias is safe and effective. Laparoscopic surgery is an appropriate alternative approach for recurrent hiatal hernia repair in selected patients.

The high prevalence of gastroesophageal reflux disease (GERD) in Western societies has accelerated the need for new modalities of treatment. Currently, medical and surgical therapies are widely accepted among patients and physicians. New potent antisecretory drugs and the development of minimally invasive surgery for the management of GERD are at present the pivotal and largely accepted approaches to treatment. The minimally invasive treatment revolution, however, has stimulated several new endoscopic techniques for GERD. Up to now, the data is limited and further studies are necessary to compare the advantages and disadvantages of the various endoscopic techniques to medical and laparoscopic management of GERD. New journal articles and abstracts are continuously being published. The Food and Drug Administration has approved 3 modalities, thus gastroenterologists and surgeons are beginning to apply these techniques. Further trials and device refinements will assist clinicians. This article will present an overview of the various techniques that are currently on study. This review will report the efficacy and durability of various endoscopic therapies for gastroesophageal reflux disease (GERD). The potential for widespread use of these techniques will also be discussed. Articles and abstracts published in English on this topic were retrieved from Pubmed. Due to limited number of studies and remarkable differences between various trials, strict criteria were not used for the pooled data presented, however, an effort was made to avoid bias by including only studies that used off-PPI scoring as baseline and intent to treat.