A Massive Ewing Sarcoma of the Rib: A Case Report with Literature Review.

A 9-year-old boy with a 16 cm chest wall mass, presenting with progressive cough and exertional dyspnea, was finally diagnosed with Ewing sarcoma of the rib. Such massive tumors usually present with metastasis and carry a bad prognosis. Fortunately, we present here a successful treatment approach for Ewing sarcoma of the ribs, defeating the overwhelming obstacles commonly faced in chest wall tumors. Delays in diagnosis, misdiagnosis, difficulty with general anesthesia, opportunistic infections, disruptions in chemotherapy delivery, and debilitating chest wall deformities are all potential challenges that could complicate the course of treatment.

Antenatally diagnosed intraspinal-posterior mediastinal neurenteric cyst - what is the optimal management?

Neurenteric cyst is a rare congenital anomaly that belongs to the spinal dysraphism spectrum. It is classically a disease of late childhood, with some rare reports of intrauterine and adult presentation. The increase in incidental antenatal diagnosis raises new management questions. We present a case of an asymptomatic combined intraspinal-posterior mediastinal neurenteric cyst. The cyst was diagnosed on antenatal ultrasound and was initially treated with image-guided aspiration in early infancy. However, the cyst recurred and the patient developed recurrent episodes of bacterial meningitis. Two-stage surgical resection was performed, although the infection prohibited complete excision. We caution against image guided aspiration of neurenteric cysts, and suggest a management algorithm based on the available literature.

Port-a-Cath fracture and migration in paediatric cancer patients: incidence and management at a tertiary care centre - a 15-year experience.

INTRODUCTION: Port-a-Cath or chemoport provides prolonged central venous access for cancer patients requiring prolonged chemotherapy. Prolonged use of chemoport is associated with many complications. Dislodgement and migration of chemoport catheter is a rare and reportable complication with potentially serious consequences. METHODS: The medical charts of 1222 paediatric cancer patients admitted to the Children's Cancer Center in Lebanon who had chemoports inserted for long-term chemotherapy were retrospectively reviewed. Descriptive analysis of data was conducted. RESULTS: Chemoport fracture and migration were found in seven cases with an incidence of 0.57%. The duration of chemoport use before the event of dislodgement varied from 2 months to 102 months. Non-functioning chemoport was the most common presentation. Totally, six cases were managed successfully by loop snaring, three cases by paediatric cardiology team, and three cases by interventional radiology team. One case was managed surgically during chemoport removal. CONCLUSION: Fracture and migration of chemoport catheter is a rare complication of uncertain aetiology and with potentially serious consequences. Percutaneous retrieval, done by experienced cardiologist or interventional radiologist, is the first choice for management of this complication as it is considered as a safe and effective approach.

Clinical Prognostic Factors and Outcome in Pediatric Osteosarcoma: Effect of Delay in Local Control and Degree of Necrosis in a Multidisciplinary Setting in Lebanon.

PURPOSE: Outcomes in pediatric osteosarcoma have dramatically improved over the past few decades, with overall survival rates of 70% and 30% for patients with localized and metastatic disease, respectively. PATIENTS AND METHODS: We retrospectively reviewed clinical characteristics and outcomes of 38 patients treated between 2001 and 2012 at a single institution in Lebanon. All patients received a uniform three-drug chemotherapy regimen consisting of cisplatin, doxorubicin, and methotrexate. Ifosfamide and etoposide were added to the adjuvant treatment regimen in case of metastatic disease and/or poor degree of tumor necrosis (< 90%). RESULTS: After a median follow-up of 61 months (range, 8 to 142 months), patients with localized disease had 5-year overall and event-free survival rates of approximately 81% and 68%, respectively, whereas for metastatic disease, they were approximately 42%. The most common primary site was the long bones around the knee (n = 34; 89.5%). Six patients (15.8%) had metastatic disease to lungs, and three (7.9%) had synchronous multifocal bone disease with lung metastases. Adverse prognostic factors included nonlower extremity sites, metastasis, poor degree of necrosis, and delay of more than 4 weeks in local control. In bivariable analysis, only degree of necrosis was a prognostic predictor for survival and disease recurrence. CONCLUSION: Treatment of pediatric osteosarcoma in a multidisciplinary cancer center in Lebanon resulted in survival similar to that in developed countries. Delay in local control was associated with worse outcome. The only statistically significant inferior outcome predictor was poor degree of necrosis at the time of local control.

Collaborative Pediatric Bone Tumor Program to Improve Access to Specialized Care: An Initiative by the Lebanese Children's Oncology Group.

BACKGROUND: Children with malignant bone tumors have average 5-year survival rates of 60% to 70% with current multimodality therapy. Local control modalities aimed at preserving function greatly influence the quality of life of long-term survivors. In developing countries, the limited availability of multidisciplinary care and limited expertise in specialized surgery and pediatric radiation therapy, as well as financial cost, all form barriers to achieving optimal outcomes in this population. METHODS: We describe the establishment of a collaborative pediatric bone tumor program among a group of pediatric oncologists in Lebanon and Syria. This program provides access to specialized local control at a tertiary children's cancer center to pediatric patients with newly diagnosed bone tumors at participating sites. Central review of pathology, staging, and treatment planning is performed in a multidisciplinary tumor board setting. Patients receive chemotherapy at their respective centers on a unified treatment plan. Surgery and/or radiation therapy are performed centrally by specialized staff at the children's cancer center. Cost barriers were resolved through a program development initiative led by St Jude Children's Research Hospital. Once program feasibility was achieved, the Children's Cancer Center of Lebanon Foundation, via fundraising efforts, provided continuation of program-directed funding. RESULTS: Findings over a 3-year period showed the feasibility of this project, with timely local control and protocol adherence at eight collaborating centers. We report success in providing standard-of-care multidisciplinary therapy to this patient population with complex needs and financially challenging surgical procedures. CONCLUSION: This initiative can serve as a model, noting that facilitating access to specialized multidisciplinary care, resolution of financial barriers, and close administrative coordination all greatly contributed to the success of the program.

Wilms tumor: Successes and challenges in management outside of cooperative clinical trials.

OBJECTIVE/BACKGROUND: Management of Wilms tumor (WT) in children depends on a multidisciplinary approach to treatment, and outcomes have significantly improved as reported by cooperative group clinical trials. Here, we review the clinical outcomes of patients with WT and identify challenges and barriers encountered in multidisciplinary management outside of cooperative clinical trials. METHODS: We retrospectively reviewed the clinical records of 35 children with WT treated between April 2002 and June 2013 at the Children's Cancer Institute in Lebanon. RESULTS: Upfront resection was performed in 23 cases. Biopsies were performed for Stage V tumors (n=4), those with unresectable tumors or inferior vena caval thrombus (n=5), and patients who had partial surgery performed elsewhere prior to presentation (n=2). One patient died due to toxicity prior to surgery. The tumor was Stage I in eight patients, Stage II in five patients, Stages III and IV in nine patients each, and bilateral (Stage V) in four patients. Adherence to The National Wilms Tumor Study-5 recommendations was adequate. At the time of analysis, 30 patients were free of disease and four patients had relapse-all having metastatic disease initially. CONCLUSION: The National Wilms Tumor Study-5 therapy resulted in favorable outcomes in children with nonmetastatic Wilms tumor in the setting of a multidisciplinary approach to therapy and resolution of financial barriers to medical care. Upstaging due to prior intervention and lung radiation therapy to all those with computed tomography-detected lung nodules may both have resulted in overtreatment of a subset of patients. Finally, the relatively high incidence of bilateral tumors suggests the need for further genetic and molecular studies in this patient population.

A case of battery ingestion in a pediatric patient: what is its importance?

This is a case of a two-year-old boy who has been suffering from food regurgitation and frequent vomiting over the past seven months which were progressively worsening with time. He was initially diagnosed with gastroesophageal reflux disease and treated accordingly but responded only minimally. Investigations and interventional procedures including a chest X-ray showed a metallic round object in the upper esophagus consistent with a button battery which was removed via a thoracotomy after an esophagoscopy was not successful. This child would not have developed such serious complications and would not have required major surgery had the foreign body been identified and removed early on.

Outcome of Ewing sarcoma in a multidisciplinary setting in Lebanon.

Treatment of Ewing sarcoma (ES) necessitates coordinated multi-disciplinary care. We analyzed outcome for 39 patients treated at a single institution in Lebanon, a developing country with available multidisciplinary treatment modalities, where financial barriers to care are overcome by a fundraising system. Median follow-up was 58 months. Five-year overall and event-free survival were 76% and 58%, respectively, for localized disease, and 40% and 38%, respectively, for metastatic disease. We conclude that, in a country with emerging economy, by following international protocols and ensuring availability of needed resources, outcome of patients with ES is similar to that in developed countries.

Left-sided appendicitis in children with congenital gastrointestinal malrotation: a diagnostic pitfall in the emergency department.

Acute appendicitis is one of the most common conditions requiring emergency surgery. However, acute appendicitis presenting with left lower quadrant abdominal pain is extremely rare. Imaging, particularly CT , plays an important role in establishing an accurate and prompt diagnosis, as delay in diagnosis may occur due to lack of uniformity in the clinical signs and symptoms. We report a rare case of a 10-year-old boy who presented with persistent left lower quadrant pain of several days duration, in which the CT scan of the abdomen and pelvis was essential in establishing the correct diagnosis. The malpositioned inflamed appendix was clearly identified in the left side of the abdomen, with the characteristic CT findings of uncomplicated intestinal malrotation. Left-sided acute appendicitis should be considered in the differential diagnosis of young patients presenting with left lower quadrant pain, in order to avoid delay in diagnosis and guide the surgical intervention.

Intestinal polypoid arteriovenous malformation: unusual presentation in a child and review of the literature.

AIM: We report a jejuno-jejunal intussusception with a polypoid arteriovenous malformation as a lead point in a 12-year-old boy, presenting with lower abdominal pain and non-bloody non-projectile vomiting. METHODS: A computed tomography scan of the abdomen and pelvis showed proximal jejuno-jejunal intussusception in the right upper quadrant. Exploratory laparotomy revealed a 5.5 × 2.5 × 2 cm polypoid mass within the wall of the jejunum. Consequently, jejunal segment resection was performed with end to end jejunostomy. Our case is distinctive because it involves a rare vascular lesion at an atypical site, the jejunum, in a child with an unusual presentation of intussusception treated surgically. CONCLUSION: Many paediatric benign and surgical conditions present with similar clinical symptoms; the physician in the emergency department should try to narrow the differential diagnosis and recognize surgical emergencies to avoid any delay in intervention that could be life-threatening.

Limb salvage surgery for children and adolescents with malignant bone tumors in a developing country.

PURPOSE: To describe the 5-year experience of a multidisciplinary limb salvage program for children and adolescents with malignant bone tumors in Lebanon. PATIENTS AND METHODS: Between January 2002 to February 2007, 30 children and adolescents (2 with Ewing sarcoma and 28 with osteosarcoma) underwent Limb Salvage Surgeries (LSS) at the American University of Beirut Medical Center after partnering with the multidisciplinary team at St. Judes Children's Research Hospital (SJCRH). Procedures performed included 12 Repiphysis, noninvasively expandable, prostheses inserted in skeletally immature children, 15 modular prostheses, 2 allografts and 1 rotationplasty. All patients received pre- and postoperative chemotherapy. RESULTS: With a mean follow-up of 31 months, 20 patients are now off therapy, 4 died, and 6 are receiving chemotherapy (3 due to pulmonary recurrence). Complications of surgery included infections in three cases, failure of the expansion mechanism in two, femoral stem fracture in one, prostheses femoral stem loosening in one and wound dehiscence in one. Lengthening of Repiphysis prostheses was achieved by subjecting the limb to an electromagnetic field that would allow controlled release of the Repiphysis expansion mechanism. Ten patients underwent a total of 42 lengthening procedures with an average of 9 mm lengthened per procedure (range 2-15 mm). All patients have good function of the affected limb. CONCLUSION: Our LSS results are comparable to those reported in the literature. Collaboration with SJCRH and fund raising were critical to the program's success. This endeavor could serve as a model for establishing LSS programs in developing countries.

Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1.

Malignant peripheral nerve sheath tumors (MPNST) are uncommon in children and almost half of the cases occur in patients with neurofibromatosis 1 (NF1). We report a child with a primary MPNST of the lung without NF1. MPNST of the lung has similar clinical and radiologic characteristics as pleuropulmonary blastoma. We suggest to include MPNST of the lung in the differential diagnosis of intrapulmonary masses in children.

Gallstone pancreatitis in children: atypical presentation and review.

UNLABELLED: Pancreatitis in children is less common than in adults and is mostly related to trauma, infection and anatomical anomalies. Gallstone pancreatitis is an even rarer entity in infants and children. We present an unusual case of gallstone pancreatitis in a 17- month-old girl who presented with jaundice of 1 month duration. The laboratory studies showed conjugated hyperbilirubinaemia with associated acute pancreatitis and pseudo-hyponatraemia secondary to the markedly elevated lipid profile. Further evaluations revealed obstruction at the Ampulla of Vater with no evidence of a choledochal cyst and no mass lesion seen in the pancreas. The patient initially underwent urgent decompressive cholecystostomy with intraoperative cholecystography. When the biochemical parameters improved, the patient underwent formal cholecystectomy with common bile duct exploration, extraction of multiple impacted stones in the ampullary region and Fogarty balloon sphincterotomy. The post-operative course was uneventful. CONCLUSION: Acute gallstone pancreatitis in children may present as jaundice or abdominal pain. Recognition, early diagnosis and surgical intervention are the mainstay for a good outcome.

Acute respiratory distress associated with external jugular vein catheterization in the newborn.

We report on the acute onset of respiratory distress secondary to fluid accumulation in the chest within hours of placement of an external jugular venous line in a newborn. External jugular venous catheterization in the newborn is a procedure with potentially serious complications, and should be avoided unless the patient is monitored closely.

Urinary retention secondary to müllerian duct remnant in an infant.

To date only 2 cases of urinary retention secondary to a müllerian duct cyst have been reported in infancy. We report a third case of bladder outlet obstruction caused by massive dilatation of persistent müllerian remnant in a 2 month old boy, the youngest to date. Surgical intervention was done because patient continued to have retention following conservative management. The cyst was large, located in the midline, and was associated with an absent left kidney.