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Background Malignant salivary gland neoplasms are rare in children. The purpose of this study was to present our experience in the treatment of primary and recurrent salivary gland neoplasm and contribute to the fractional available data. Methods All patients

To determine the outcomes of resection of satellite lesions in PRE-treatment Tumor EXTension (PRETEXT) II and III multifocal hepatoblastoma irrespective of their disappearance after chemotherapy. To compare the overall outcomes of multifocal and unifocal hepatoblastoma. Fourteen patients with PRETEXT II (n = 7) and III (n = 7) multifocal hepatoblastoma treated between April 2006 and July 2014 were analyzed and their outcomes were compared with PRETEXT II and III unifocal hepatoblastoma treated in the similar period. Satellite lesion or the affected segments with disappeared satellite lesions were resected in 11 patients. Amongst them, all relapses were distant except one in the liver. In contrast, two of three patients developed liver relapse when the affected segments were not resected. None of the patients receiving intensive chemotherapy based on SIOPEL-3 guidelines developed a relapse. The 3-year event-free and overall survival were 38.6 and 42.9 % in multifocal hepatoblastoma and 86.4 and 92.4 % in unifocal hepatoblastoma (p = 0.001). Multifocality (p = 0.002) and AFP >10,000 after induction chemotherapy significantly affected event-free survival (p = 0.01). Multifocal hepatoblastoma is associated with poor outcomes as compared to unifocal hepatoblastoma. These preliminary observations of relapse and the role of chemotherapy intensification deserve further study in a multicenter controlled trial setting.

Neuroblastoma is the third common tumor in children. Imaging plays an important role in the diagnosis, staging, treatment planning, response evaluation and in follow-up of a case of Neuroblastoma. The International Neuroblastoma Risk Group task force has recently introduced an imaging-based staging system and laid down guidelines for uniform reporting of imaging studies. This review is an update on imaging in neuroblastoma, with emphasis on these guidelines.

Extramedullary hematopoiesis is production of blood cells outside bone marrow which occurs in a variety of disorders including myelofibrosis. We present a 15 month old child with stage 4 neuroblastoma with secondary myelofibrosis which resulted in intracranial extramedullary hematopoiesis, posing a diagnostic and therapeutic dilemma. This was resolved with a sulfur colloid isotope scan which confirmed extramedullary hematopoiesis and not a progressing metastasis. This helped us continue therapy with curative intent. To the best of our knowledge we did not come across any report of neuroblastoma with myelofibrosis and intracranial extramedullary hematopoiesis mimicking progression of metastasis.

Background A large majority of neuroblastoma continue to present with advanced disease with widespread dissemination. However, metastasis to the ovary or testis is infrequent with sparse literature even from the major neuroblastoma study groups. The aim of this study was to determine the group of patients in whom such a rare entity has occurred and evaluate any common factor and clinical implication. Materials and methods We retrieved records of all patients with abdominal neuroblastoma operated from January 2008 to August 2013. We selected the cases with overt gonadal (ovarian or testicular) metastasis at presentation or relapse and noted the details. Results Of the 186 cases of neuroblastoma four patients were having metastasis to the gonads (testis = 2, ovary = 2). All the four cases had extensive abdominal disease and received prolonged chemotherapy before the institution of local surgical treatment. Conclusion Protracted chemotherapy without timely and adequate local treatment may contribute to the metastases seen in unusual sites such as the gonads. However, the therapeutic implication of identifying gonadal metastases is uncertain in patients already having disseminated disease.

Neuroblastoma is infrequently associated with paraneoplastic syndromes. Amongst the few, opsomyoclonus (Kinsbourne syndrome) is the most common neurological paraneoplastic syndrome and diarrhea secondary to increased secretion of vasoactive intestinal peptide (Kerner-Morrison syndrome), hormonal paraneoplastic syndrome. Hypothalamic dysfunction (HD) is a rare disorder and its manifestation as a paraneoplastic syndrome of neuroblastoma is uncommonly reported. We present an interesting case of an unrelenting cervical neuroblastoma associated with HD, which posed a therapeutic challenge.

Rhabdomyosarcoma (RMS) is a common soft-tissue neoplasm in the pediatric age group. Common locations are head and neck, genitourinary areas, trunk, and extremities. Two pathologic variants of this malignancy are embryonal and alveolar. The involvement of breast is rare. Herein, we report two cases of alveolar RMS of the breast of which one is an isolated breast metastasis from an orbital primary, whereas the other is a primary RMS of the breast. Both the patients were treated with surgery followed by adjuvant chemotherapy and radiotherapy and are currently disease free at six and three year respectively, following completion of treatment.

Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.

Introduction/Purpose The outcomes of intra-arterial (IA) endovascular treatment in patients with large vessel occlusion (LVO) are time dependent. In order to identify emergency large vessel occlusion (ELVO) and notify the receiving hospital from the field, emergency medical services (EMS) in Ventura county of California use Ventura ELVO Scale (VES). Early notification of ELVO from the field can potentially shorten the door to needle and door to groin time by activation of Interventional Radiology (IR) team earlier and in future, it may help triage patients with stroke to comprehensive stroke center. Methods and Analysis Ventura ELVO Scale (VES) comprise of four components: 1) Eye Deviation 2) Aphasia 3) Neglect 4) Obtundation, each component scoring either 1 or 0. The maximum score is 4 and minimum score is 0. The score of 1 or greater will be considered as ELVO positive. VES was implemented by EMS to identify ELVO patients in the catchment area of Los Robles Hospital and Medical Center (LRHMC). A positive ELVO score along with positive Cincinnati scale prompts ELVO activation. EMS then calls the neuro-interventionalist who activates the neuro-intervention protocol at LRHMC. Retrospective de-identified data was collected from EMS run sheets, emergency department (ED) and IR records of LRHMC for cases in which the stroke code was activated by EMS during the period of Jan 2016 to Jan 2017. Result A total of 204 patients were activated as stroke code by Ventura county EMS, out of which 26 were activated as ELVO positive. The mean age of ELVO positive patients was 77 (SD ±13.01) with 11:15 male to female ratio. Large vessel occlusion (LVO) was confirmed in 18 out of 26 ELVO positive patients on subsequent angiogram. The mean door to IV t-pa time in ELVO positive patients with LVO was 23.20 mins (SD ±9.73) while the mean door to groin time was 73.20 mins (SD ±20.30). Mean door to reperfusion time was 135.20 mins (SD ±32.77). The sensitivity of VES was 94.74% while the specificity was 95.67%. The positive predictive value (PPV) was 69.23% while the negative predictive value (NPV) was 99.44%. Conclusion VES demonstrated reliable sensitivity, specificity, PPV and NPV and its implementation achieved target door to needle and door to groin time in patients with LVO. Disclosures M. Taqi: None. A. Sodhi: None. S. Suriya: None. S. Quadri: None. D. Shepherd: None. A. Salvucci: None. A. Stefansen: None.

Background Various techniques have been used for wrap-clipping a ruptured, fusiform intracranial aneurysm; however, there is no available literature on use of the falx cerebri for wrap-clipping. We present a review of the literature, with an illustrative case, of a ruptured fusiform pericallosal artery aneurysm firmly attached to the lower edge of the falx cerebri and not amenable to endovascular intervention. Methods Although the firm attachment between the inferior falx and the fusiform aneurysm was maintained, a section of the lower thinner part of the falx cerebri firmly attached to the aneurysm was dissected and wrapped around the fusiform aneurysm, and then stabilized with a fenestrated clip. We chose a segment slightly longer than the length of the fusiform aneurysm to avoid pre– and post–wrap-clipping stenosis. Results Postprocedure, except for a small area of numbness on the left distal anterolateral left leg, the patient was neurologically intact and remained neurologically intact at a 12-month follow-up. Conclusions An inferior thin segment of the falx cerebri can be used for wrap-clipping of ruptured fusiform anterior cerebral artery aneurysms. Furthermore, the inferior falx can be wrapped around the attached fusiform anterior cerebral artery aneurysm without compromising flow, offering a safe solution in these unusually complex cases.

Objectives Earthquakes are unpredictable and devastating natural disasters. They can cause massive destruction and loss of life and survivors may suffer psychological symptoms of severe intensity. Our goal in this article is to review studies published in the last 20 years to compile what is known about posttraumatic stress disorder (PTSD) occurring after earthquakes. The review also describes other psychiatric complications that can be associated with earthquakes, to provide readers with better overall understanding, and discusses several sociodemographic factors that can be associated with post-earthquake PTSD Method A search for literature was conducted on major databases such as MEDLINE, PubMed, EMBASE, and PsycINFO and in neurology and psychiatry journals, and many other medical journals. Terms used for electronic searches included, but were not limited to, posttraumatic stress disorder (PTSD), posttraumatic symptoms, anxiety, depression, major depressive disorder, earthquake, and natural disaster. The relevant information was then utilized to determine the relationships between earthquakes and posttraumatic stress symptoms. Results It was found that PTSD is the most commonly occurring mental health condition among earthquake survivors. Major depressive disorder, generalized anxiety disorder, obsessive compulsive disorder, social phobia, and specific phobias were also listed. Conclusion The PTSD prevalence rate varied widely. It was dependent on multiple risk factors in target populations and also on the interval of time that had elapsed between the exposure to the deadly incident and measurement. Females seemed to be the most widely-affected group, while elderly people and young children exhibit considerable psychosocial impact. Resumo Objetivos Terremotos são desastres naturais imprevisíveis e devastadores. Eles podem causar destruição em massa e morte, e os sobreviventes podem apresentar sintomas psicológicos severos. O objetivo deste estudo foi revisar estudos publicados nos últimos 20 anos pra compilar o conhecimento disponível acerca da ocorrência de transtorno do estresse pós-traumático (TEPT) após terremotos. A revisão também descreve outras complicações psiquiátricas que podem estar associadas a terremotos, oferecendo aos leitores um melhor entendimento geral sobre o assunto, e discute vários fatores sociodemográficos que podem estar associados com TEPT pós-terremoto. Métodos Foi realizada uma busca de literatura nas principais bases de dados, como MEDLINE, PubMed, EMBASE e PsycINFO, e também em revistas de neurologia e psiquiatria, e vários outros periódicos médicos. Os termos usados nas buscas eletrônicas incluíram, mas não se limitaram a, posttraumatic stress disorder (PTSD), posttraumatic symptoms, anxiety, depression, major depressive disorder, earthquake e natural disaster. As informações relevantes foram então utilizadas para determinar as relações entre terremotos e sintomas de estresse pós-traumático. Resultados A revisão revelou que o TEPT é a condição de saúde mental mais comum em sobreviventes de terremoto. Depressão maior, transtorno de ansiedade generalizada, transtorno obsessivo-compulsivo, fobia social e fobias específicas foram outras condições encontrados. Conclusão A prevalência de TEPT variou bastante. O transtorno foi dependente de múltiplos fatores de risco em populações-alvo e também do intervalo de tempo decorrido entre a exposição ao incidente fatal e a avaliação. As mulheres pareceram ser o grupo mais amplamente afetado, ao passo que idosos e crianças demonstração considerável impacto psicossocial.

Background and Objective The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non-metastatic ES of the mandible and maxilla in children. Methods Consecutive patients (mandible = 6, maxilla = 5) treated with surgery from August 2005 to January 2013 were selected. All patients received induction chemotherapy and were selected for surgical resection based on the presence of specific criteria for operability. Results The median age was 11.5 years (range 5–16 years). Free fibular osteocutaneous flap was commonly used for reconstruction. There were no complications related to microvascular anastomosis or flap loss. Five patients had 100% tumor necrosis and did not receive radiotherapy. Teeth alignment, chewing, swallowing, and speech were normal in all and donor site morbidity occurred in one. The 5-year overall, event-free survival, and local control are 87.5%, 72.9%, and 90%, respectively. Conclusion In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcome. Surgery also has the added advantage of identifying patients who may not need radiotherapy. J. Surg. Oncol. 2014 110:689–695. © 2014 Wiley Periodicals, Inc.

Congenital rhabdomyosarcomas (RMSs) are rare tumors with variable clinical presentations. A 2 month-old, term male neonate (37 weeks, 4 days), weighing 3.2kg, born to a 24 year-old primigravida, by simple vaginal delivery presented with multiple erythematous papulonodular lesions over his trunk that progressed to his whole body, on the first day of delivery. Prior to conception, his mother was treated for polycystic ovarian disease. On the tenth day, his chest computed tomogram scans revealed multiple, heterogeneously enhancing, bilateral pleural-based soft tissue density nodular lesions, along with multiple soft tissue density lesions, involving skeletal muscles of all his body parts. Microsections from two biopsies (on 10th day and after 2 months) revealed a malignant round cell tumor with cells arranged in a diffuse, solid pattern, comprising embryonal and solid alveolar components. Immunohistochemically, the tumor cells were diffusely positive for desmin, myoD1 and myogenin. Diagnosis of embryonal and alveolar (mixed type) RMS was offered. Further molecular cytogenetic analysis was negative for PAX3-FKHR and PAX7-FKHR. The patient was induced on chemotherapy as per intergroup rhabdomyosarcoma study IV protocol. There was treatment response with near total remission after 8 weeks of treatment. Thereafter, new lesions started appearing that also disappeared after modification of the chemotherapy drugs. However, after 16 months, the baby died of brain metastasis. The present case forms the fourth case report of an aggressive form of a congenital RMS with extensive cutaneous involvement and brain metastasis. A review of previously diagnosed cases of congenital RMSs is discussed herewith.

Regional techniques provides excellent post operative pain relief in pediatric patients. Transversus abdominis plane (TAP) block is a newer regional technique available. Though there is emerging evidence proving the efficacy of TAP blocks, there is limited literature on use of TAP catheters in pediatric patients. TAP catheters were placed in two children following laparotomy with transverse incisions and in both epidural was avoided, with good post operative pain relief. Ultrasound guidance was used in one child, while in the other the catheter was placed under direct vision after dissection of the plane between transversus abdominis and internal oblique. Intermittent boluses of high volumes of local anesthetic (0.6-0.7 ml/kg) were used through the TAP catheter, ensuring that the maximum permissible level of bupivacaine was not exceeded. In adults, continuous abdominal catheters have found a place for post-operative pain management, when epidural analgesia is contraindicated. At present, the use of TAP catheters by pediatric anesthesiologists is limited, though there exists diverse clinical scenarios when these catheters may be of benefit. Contraindication of neuraxial blockade and septic patients are the two scenarios we have reported. In conclusion, TAP catheters are effective analgesia technique for laparotomies with transverse incision in pediatric patients.

Retinoblastoma (RB) is a common intraocular tumor of child-hood arising in the retina and accounts for around 3% of thecancers occurring in children below 15 years [1]. The estimatedglobal annual incidence is approximately four per million childrenunder 15 years of age. RB usually presents before the age of2 years and 95% of cases are diagnosed before the age of 5 years.Approximately 40% of RB cases are due to germ line mutationswhile 60% are sporadic in nature. The genetic locus responsiblefor a predisposition to the development RB is located within theq14 band region of chromosome 13.Increased incidence of sporadic RB is seen in less affluentareas of the world such as Latin America, Africa, and Asia(including India) [2,3] suggesting that environmental factors as-sociated with low socioeconomic status may play a role in etio-pathogenesis of RB. The geographic variation observed in RBmay be due to maternal exposure to viral infections or otherenvironmental factors which could cause mutations in utero. Hu-man papillomavirus (HPV) infection is seen more frequently inpregnant than nonpregnant women [4]. An overlap is seen in theepidemiological risk factors for development of RB and HPVinfection. These include smoking, dietary supplements, poor gen-ital hygiene, multiple pregnancies, multiple sexual partners, andsexual intercourse at an early age [5]. This indicates that increasedincidence of RB has been observed in countries with a highincidence of cervical carcinoma [5].Despite the high incidence of RB in India, familial RB is rareand is noted in only 1.7% of cases and majority of patients havenonfamilial or sporadic RB [6].HPV induces posttranslational interactions of its oncoproteinsviz. E6 and E7 with cell cycle regulatory proteins. E6 binds to thetumor suppressor p53 and promotes its degradation via the pro-teosome /ubiquitin pathway. E7 binds to the tumor suppressor Rbproteins pRb, p107, p130, cyclin A, and cyclin E as well as cyclindependent kinases [7]. Binding of E7 to the pRb blocks its activityrendering the proteins inactive.These epidemiological and molecular studies suggest thatHPV may play a role in the development of sporadic RB. Severalstudies have shown a link between HPV infection and RB [5,8–13]. The aims of this study were to determine the presence ofHPV in sporadic RB and to elicit the effects of HPV infection onthe cell cycle regulatory pathway focusing on the pRb family ofproteins.

Broad ligament tumors are uncommon. Sarcomas rarely arise from the broad ligament, leiomyosarcomas being the most common. Rhabdomyosarcomas of the broad ligament are very rare. To the best of our knowledge, no case of an embryonal rhabdomyosarcoma of the broad ligament has been reported in literature.

Purpose Evaluate long-term clinical outcomes, adverse effects, and evolving practice of interstitial brachytherapy (BT) for pediatric soft tissue sarcomas (STS). Methods From September 1984 to December 2014, 105 children (median age 10 years) were included. There were 60 males and 45 females. The majority (74%) had primary lesions. Synovial sarcoma (22%) was the most frequent histology. Treatment included wide local excision and BT with or without external beam radiotherapy (EBRT). Eighty-five (81%) received BT alone. Results After a median follow-up of 65 months, local control (LC), disease-free survival (DFS), and overall survival (OS) at 10 years were 83, 66, and 73%, respectively. On univariate analysis, LC was superior with tumors 5 cm (93% vs. 75%, P = 0.10), Grade I/II versus Grade III tumors (97% vs. 73%, P = 0.01), nonround cell versus round cell histology (89% vs. 72%, P = 0.03), and trunk/extremity versus head and neck/genitourinary sites (87% vs. 57%, P = 0.0001). On multivariate analysis tumor size (P = 0.03) and location (P = 0.002) retained significance. Children receiving BT alone had comparable LC to those receiving BT and EBRT (84% vs. 80%, P = 0.43). There was no difference in LC between LDR versus HDR BT (86% vs. 83%, P = 0.30). Wound complications were seen in 6%. Subcutaneous fibrosis (25%), limb edema (6%), skeletal abnormalities (3%), and neuropathy (1%) were the late complications. One child (0.9%) developed a second malignancy after 7 years. Conclusion Interstitial BT with or without EBRT results in excellent outcomes. Radical BT alone, when used judiciously, results in excellent local control and function with minimal treatment-related morbidity.

Since Lynn and colleagues first described the use of focused ultrasound (FUS) waves for intracranial ablation in 1942, many strides have been made toward the treatment of several brain pathologies using this novel technology. In the modern era of minimal invasiveness, high-intensity focused ultrasound (HIFU) promises therapeutic utility for multiple neurosurgical applications, including treatment of tumors, stroke, epilepsy, and functional disorders. Although the use of HIFU as a potential therapeutic modality in the brain has been under study for several decades, relatively few neuroscientists, neurologists, or even neurosurgeons are familiar with it. In this extensive review, the authors intend to shed light on the current use of HIFU in different neurosurgical avenues and its mechanism of action, as well as provide an update on the outcome of various trials and advances expected from various preclinical studies in the near future. Although the initial technical challenges have been overcome and the technology has been improved, only very few clinical trials have thus far been carried out. The number of clinical trials related to neurological disorders is expected to increase in the coming years, as this novel therapeutic device appears to have a substantial expansive potential. There is great opportunity to expand the use of HIFU across various medical and surgical disciplines for the treatment of different pathologies. As this technology gains recognition, it will open the door for further research opportunities and innovation.

Context: Pediatric solid tumors include a heterogeneous group of tumors, and the burden of these tumors, especially from resource-challenged countries, is not well described. AIMS: The aim of this study was to describe the distribution of solid tumors in children and the treatment outcome of Wilms tumor and hepatoblastoma. Patients and Methods: All patients under 15 years of age with histologically confirmed tumors presenting at a tertiary cancer center from January 2012 to December 2016 were identified from the hospital database. Patients with lymphomas, bone, and central nervous tumors were excluded. The demographic profile including age, sex distribution, and the treatment received were recorded for all patients. Results: The mean age of the eligible 1944 patients was 5.7 years with majority (57.3%) in the 0–4 years age group. The male-to-female ratio was 1.4:1 with a male predominance in all tumors except germ cell tumors. Soft tissue tumors were the most common tumors followed by neuroblastoma and renal tumors, whereas liver tumors formed only 6.7% of all tumors. Seventy percent of the patients received treatment completely or partially at our institute, whereas 18.3% had no cancer-directed treatment. The 3-year overall survival of patients with Wilms tumor and hepatoblastoma was 85.4 and 78.5%, respectively. Conclusions: Extracranial and extraosseous pediatric solid tumors include a wide range of tumors with a predilection for male sex and children below 4 years of age. Soft tissue tumors, neuroblastoma, and renal tumors are the most common; the outcomes of Wilms tumor and hepatoblastoma are favorable.

We describe an unusual cause of an acquired cavo-varus foot deformity produced by progressive enlargement of an accessory calcaneal ossicle. A 13-year-old boy with constitutional plano-valgus flat feet noted a gradual change in foot shape associated with lateral ankle pain on ambulation following an inversion injury 2 years earlier. CT and MRI scans confirmed a large accessory calcaneal ossicle lying within the sinus tarsi, with associated marrow oedema. Following surgical excision of the ossicle, the foot returned to its original shape and the symptoms were alleviated. This is the fifth reported case of an accessory calcaneal ossicle, but the only case that has occurred in a flatfooted individual. We also present the first reported MRI images of the lesion confirming pathological marrow oedema as a response to mechanical stress.

Transient osteoporosis is a rare cause of pain in the foot. There is a broad differential diagnosis. A multidisciplinary assessment by both an orthopaedic surgeon and rheumatologist with review of the imaging by a radiologist is useful, as this condition is a diagnosis of exclusion.

A reputation function and associated reputation management system (RMS) seem to be at the heart of all online transactional activities that require trust. However, most of the first generation RMS used are yet very basic, ad hoc and often vulnerable to various attacks. In this research, we take a holistic approach to this interesting problem of RMS design. We propose a generalized set-theoretic phenotype reputation function where its specific components can be customized to meet the reputation requirements of wide variety of reputation assessment needs encountered in today's online activities. We show the construction of canonical classes of reputation functions built on this framework. We then analyze their attack tolerance against various socio-communal reputation attacks such as gang attacks, vendetta and Dr. Jekyll & Mr. Hyde.

Communities are the latest phenomena on the Internet. At the heart of each community lies a social network. In this paper, we show a generalized framework to understand and reason in social networks. Previously, researchers have attempted to use inference-specific type of relationships. We propose a framework to represent and reason with general case of social relationship network in a formal way. We call it relationship algebra. In the paper, we first present this algebra then show how this algebra can be used for various interesting computing on a social network weaved in the virtual communities. We show applications such as determining reviewers in a semi-professional network maintained by conference management systems, finding conflict of interest in a publication system, or to infer various trust relationships in a community of close associates, etc. We also show how future community networks can be used to determine who should be immunized in the case of a contagious disease outbreak and how these networks could be used in crime prevention, etc.

Hepatoblastomas are the most common liver tumours in children. However, they are rare as compared to other solid malignancies. Thus, there is a need to integrate data from surgical centers around the world to provide a clearer view on the outcomes of multidisciplinary management of these tumours. We set out to retrospectively review our experience of patients with surgically resected hepatoblastomas looking at primary and secondary outcomes. Children diagnosed with hepatoblastoma and managed surgically (along with chemotherapy) at a single institution between 1 January 2000 and 31 May 2007, were analyzed. Out of the 18 patients, there were 12 male and 6 female patients. The median age was 18 months (range 8-72). A palpable mass in abdomen was the presenting symptom in 88% patients. Sixteen patients (88.8%) underwent major liver resection. Sixteen patients (88.8%) received preoperative chemotherapy. Complete gross resection (stage I and II) was achieved in all 18 patients (100%). The mortality and morbidity rates were 0 and 11.2%, respectively. The 80-month disease-free survival was 67%. This series, the largest from India in terms of surgical resections for hepatoblastoma, reaffirms that major liver resection can be performed with minimal perioperative mortality and morbidity and that the use of chemotherapy has definitely helped in down staging tumours for liver resection.

Wilms' tumor (WT) is the commonest pediatric renal tumor, predominantly seen in children less than five years of age. The majority of patients present with an abdominal lump and CT scan is the usual imaging modality for determining the extent of disease. With multimodality management, the results of treatment of WT have improved dramatically over the last 50 years. The treatment protocols have been devised and modified repeatedly depending on evidence from randomized trials by several cooperative groups - mainly National Wilms' Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP). The NWTSG recommends primary surgery followed by chemotherapy while SIOP advocates four weeks of chemotherapy prior to surgery. The regimen, dose and duration of chemotherapy have been repeatedly modified to reduce toxicity while maintaining efficacy. The role of radiation therapy has also been customized. Most centers have reported excellent survival rates with the modern day treatment protocols, except in patients with an unfavorable histology. The results of treatment of relapsed WT have also improved with newer drugs and combinations being used for the same.

Benign liver tumors in children are rare and data regarding the surgical outcomes are deficient. We reviewed our experience in the management of these tumors particularly the extent, safety, and effectiveness of surgical resection. Between March 2005 and March 2014, 10/90 liver resections were performed for benign liver tumors. Three other patients received conservative treatment. Demographic characteristics, operative management, morbidity, and mortality were analyzed. The median age of the patients was 12 months. The distribution of pathology was infantile hepatic hemangioendothelioma/hemangioma (n = 7), mesenchymal hamartoma (n = 4), adenoma (n = 1) and focal nodular hyperplasia (n = 1). Median tumor size was 12.25 cm (range 3.5–21 cm) with a median tumor volume of 576.64 cm3 (range 13.9–1822.64 cm3). Non-anatomic resection was performed in 6/10 patients and the median blood loss was 100 ml (range 10–850 ml). Median length of hospital stay was 7 days (range 5–9 days). There were no mortality, none of the patients had a local recurrence, and all are alive at a median follow-up of 33 months. Two-third patients with benign liver tumors were managed with surgical excision, which comprised 11 % of our resectional practice. Non-anatomical resection whenever feasible can be performed safely and does not compromise the oncological outcomes.

Introduction Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions. Case description We report a case of a large, recurrent dermatofibrosarcoma protuberans in a child involving the anterior abdominal wall, which posed a challenge for reconstruction. Peritoneum sparing full thickness resection of the anterior abdominal wall, meshplasty and a free anterolateral thigh flap was performed for reconstruction of the defect. Discussion and evaluation Large composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction. Although these reconstructions are technically challenging in children, they are the only option available. Conclusion Complete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects. Free flaps remain the only option in this scenario and hence it is essential to have expertise for microvascular flap reconstruction.

Background: While mortality rates after radical gastrectomy have decreased, there is considerable morbidity after D2 lymphadenectomy. In this study, we assessed the perioperative results of D2 gastrectomy for gastric cancer. Materials: Data of 159 patients who underwent D2 gastrectomy for gastric adenocarcinoma at Tata Memorial Hospital was analyzed for interim analysis. The extent of resection, blood loss, transfusions, duration of hospitalization, number of lymph nodes dissected, complications, morbidity and mortality were analyzed. Results: 130 and 29 patients underwent distal and total gastrectomy, respectively (2002–2005) by single specialized surgical unit. Median age was 55 years (range 21–78) and blood loss was 450 ml (range 100–2,200 ml). The median duration of hospitalization was 13 days (range 7–52 days). The median number of dissected lymph nodes was 15 (range 2–46). Minor and major morbidity rate was 4.4 and 4.4% respectively. Mortality rate was 1.25%. Conclusion: Morbidity and mortality following D2 lymphadenectomy is low in this first prospective study from India. With Japan and Western countries having polarized views on D2 lymphadenectomy, future international multicenter trials could also incorporate data such as ours from areas of high incidence of gastric cancer since perioperative outcomes would no longer cloud their results and might provide a better global perspective on D2 lymphadenectomy.

Background and Objectives A significant paucity of data exists on non-squamous cancers of the maxillary sinus. The purpose of this study was to assess the clinical characteristics and outcomes of these tumors. Methods Retrospective review of 42 patients with complete available data and a diagnosis of non-squamous cancer of the maxillary sinus treated with curative intent between 1994 and 1999 were performed. Information recorded included demography, clinical characteristics, histopathological findings, treatment methods, and outcome. Survival was also compared with that of squamous cancers of the maxillary sinus. Results The majority of patients presented with locally advanced disease (83%). Malignancies were 14 sarcomas, 8 adenoid cystic carcinoma, 8 mucoepidermoid carcinoma, 2 adenocarcinoma, and 10 miscellaneous tumors. Fifteen (35.7%) patients developed recurrent disease and six developed distant metastases. The most common site of recurrence was local (32.5%). The overall mean survival was 71.7 months and 5-year overall and disease-free survival was 51% and 48%, respectively. In contrast, the mean survival in the selected 62 patients with squamous cancers was 40 months and 5-year survival was 29% (P = 0.01). Tumor stage, histological, and treatment type was not associated with significant survival advantage. Conclusions Patients with non-squamous cancers of the maxillary sinus present with locally advanced disease. Local tumor progression remains a significant pattern of failure; however, unlike squamous cancers they have a better prognosis. J. Surg. Oncol. 2006;93:362–367. © 2006 Wiley-Liss, Inc.

Giant cell tumors of bone are known for their unpredictable behavior characterized occasionally even by metastases. Most metastases lodge in the lungs but other rare sites are regional lymph nodes, mediastinum, skin, scalp and the pelvis. In this case report we document a case of giant cell tumor of the patella in which, associated with local recurrence, there were simultaneous metastases to lymph nodes and lungs.

A case of unicystic ameloblastoma of the mandible in a 10-year-old child is described. In addition to the mandibular swelling the patient also had a proliferative lesion over the alveolus that masqueraded as a neoplastic lesion.

The contemporaneous occurrence of adrenal ganglioneuroma and left-sided inferior vena cava is uncommon and is previously never reported. We present a case wherein the two entities were fortuitously associated on preoperative imaging. This information facilitated successful surgical resection and avoided catastrophic vascular injury. A review of literature of the common venous anomaly affecting therapeutic decision-making is also presented.

The Lauge-Hansen classification of ankle fractures is a historical landmark in fracture research. Despite this there are, however, some shortcomings in methodology and several papers have demonstrated poor reproducibility. This paper reviews Lauge-Hansen's work and the literature evaluating his classification.

Background: The importance of tumor free margins in outcome of cancer surgery is well known. Often the pathological margins are reported to be significantly smaller than the in situ margins. This discrepancy is due to margin shrinkage the quantum of which has not been studied in patients with oral cancers. Objectives: To quantify the shrinkage of mucosal margin following excision for carcinoma of the oral tongue and buccal mucosa. Methods: Mucosal margins were measured prior to resection and half an hour after excision in 27 patients with carcinoma of the tongue and buccal mucosa. The mean margin shrinkage was assessed and the variables affecting the quantum of shrinkage analyzed. Results: The mean shrinkage from the in situ to the post resection margin status was 22.7% (P < 0.0001). The mean shrinkage of the tongue margins was 23.5%, compared to 21.2% for buccal mucosa margins. The mean shrinkage in T1/T2 tumors (25.6%) was significantly more than in T3/T4 (9.2%, P < 0.011). Conclusions: There is significant shrinkage of mucosal margins after surgery. Hence this should be considered and appropriate margins should be taken at initial resection to prevent the agony of post-operative positive surgical margins.

Primary cervical (CN) and cervicothoracic neuroblastoma (CTN) is generally associated with good outcome; however, surgical resection can be challenging and not without morbidity. The aim of this study is to assess the overall outcome and compare the clinico-radiological features, treatment, and complications of CN and CTN. Sixteen consecutive patients, (CN = 9, CTN = 7) treated between November 2006 and December 2012 were selected from the prospective database for this analysis. The 2-year overall and event-free survival of entire cohort is 100 and 72 %, respectively. Respiratory symptoms due to compression of airway and intraspinal extension were common in CTN. Gross total resection was feasible in all patients with CN; in contrast, incomplete excision along with significantly longer duration of surgery and more blood loss occurred in CTN. Postoperative morbidity was seen in three patients with CN and only one patient with CTN. The extent of surgery did not affect the overall and event-free survival of CTN (p = NS). CN and CTN have characteristic clinico-radiological presentation and surgical specification. However, both have a favorable outcome, even though with a distinct but acceptable morbidity. The favorable outcome in CTN is unrelated to the extent of surgical excision.

The aim of the study was to determine the impact of negative but close resection margins on local recurrence in children with extraskeletal Ewing sarcoma (EES).We reviewed records of 32 patients with EES treated between March 2005 and March 2013. All patients except one underwent surgical excision either upfront or after induction chemotherapy. Patients with viable tumor and negative surgical margins, which were categorized as less than or greater than 1 cm, were selected. Local control and survival analysis were performed for patients in both the groups.The 5-year event-free and overall survival rates of entire cohort is 68 and 77 %, respectively. Surgical margins were negative in 23/26 (90.3 %) patients. There were no local recurrences in any of the patients with margins of less than 1 cm. Only one patient with a margin greater than 1 cm had a local recurrence along with distant metastases. A tumor-free margin of more than 1 cm did not affect overall or event-free survival (p = NS).Optimal local control is feasible in children with EES regardless of the quantitative extent of negative margins. Achieving a three-dimensional tumor-free margin should be the goal of surgical resection.

Paediatric renal tumors are commonly encountered entities in clinical oncology, Wilms’ being the most common histology. Other histological types include Rhabdoid tumor, Clear Cell Sarcoma, PNET, etc. Though CT/MRI scan is perfomed for staging, it cannot differentiate between the histological types. However, there are a few imaging features which indicate certain pathologies. Rhabdoid tumors of kidney are associated with subcapsular haemorrhage which leads to ‘Subcapsular fluid sign’. We present our case to acquaint the radiologists with this sign and recommend exercising caution while interpreting it.

Objectives To evaluate the feasibility and reliability of a self-designed Ventura Emergency Large Vessel Occlusion (ELVO) Scale performed by Emergency Medical Services (EMS). To identify, if field identification of ELVO, reduces door to groin time. Background Endovascular treatment of stroke is now the standard of care for large vessel occlusion (LVO) stroke within 6 hours of onset. The outcomes of intra-arterial (IA) endovascular treatment are time dependent. The time from arrival to groin vary during the day time versus after hours or infield transfers compared to hospital to hospital transfers. Pre-identification of LVO from field can shorten the door to groin time by activation of Interventional Radiology (IR) team earlier. We designed a scale that can be used by EMS personnel in the field to identify ELVO or non-Emergency Large Vessel Occlusion. Methods and analysis Ventura ELVO Scale (VES) comprise of four components: 1) Eye Deviation 2) Aphasia 3) Neglect 4) Obtundation, each component scoring either 1 or 0. The maximum score is 4 and minimum score is 0. The score of 1 or greater will be considered as ELVO positive. VES will be implemented by EMS to identify ELVO patients in the catchment area of Los Robles Hospital and Medical Center. Retrospective data will be collected from EMS, Emergency Department (ED) and IR records of participating patients. Door to groin time for intra-arterial (IA) treatment before implementation of the Ventura ELVO protocol will be compared with door to groin time after implementation of Ventura ELVO protocol. In prospective patients, EMS will measure ELVO score in the field using the VES. A positive ELVO score along with positive Cincinnati scale will prompt ELVO activation. EMS will call the neuro-interventionist who will then activate the neuro-intervention protocol at the receiving Hospital. Implications Ventura ELVO scale can help to reduce door to groin puncture time, which can possibly improve the management and triage of stroke patients with ELVO. Disclosures M. Taqi: None. S. Quadri: None. S. Suriya: None.

Background The rarity of Ewing sarcoma of rib has resulted in paucity of data, particularly on the prognostic factors and pattern of relapses. We analyzed the recurrences in patients with primary nonmetastatic Ewing sarcoma of the rib and examined prognostic factors of poor outcome. Methods From January 2004 to January 2011, 37 patients were treated. After induction chemotherapy, complete (from costal cartilage to vertebra) or partial excision of involved rib with or without adjacent ribs was performed. Postoperative radiotherapy was administered for positive margins, poor response to chemotherapy, and large primary tumors with significant soft tissue component at presentation. Results Disease relapsed in 16 patients: at the local site (n = 5), both local and distant (n = 2), and distant site only (n = 9). The projected 5-year cause-specific, relapse-free survival and local control were 50%, 44%, and 72%. Poor response to chemotherapy (> 5% residual tumor) and resection of adjacent lung parenchyma (a surrogate for tumor extension) were adverse prognostic factors for relapse-free survival in multivariate analysis. Conclusion Relapses occurred more often at distant sites and had a poor outcome. In this study, poor histologic response to chemotherapy (P = .04) and the infiltration of adjacent lung parenchyma (P = .01) are adverse prognostic factors.

Background A thoracoabdominal incision provides excellent exposure in the upper abdominal region. We present our experience with the use of this approach in children for resection of upper abdominal neuroblastoma. Methods Fifty-one of the 106 consecutive patients with abdominal neuroblastoma who underwent tumor resection using the thoracoabdominal approach between June 2006 and May 2011 were selected from the prospective database for this interim analysis. Results The median age was 3.1 years (range, 10 months-14 years), with 8 younger than 18 months and 22 younger than 4 years. Gross total resection was achieved in 44 patients. There were no major vascular injuries or perioperative mortality. Forty-two patients could be extubated immediate postoperatively, whereas 9 were extubated within 24 hours. Good pain relief was achieved in all patients, and prolonged analgesia was not required in any patient. There were no pulmonary complications, and wound infection occurred in 2 patients. The 2-year local control was 92%. The 2-year survival for stages 1 and 2 is 100%, 71.6% for stage 3, and 40.4% for stage 4. Conclusion The thoracoabdominal incision for difficult upper abdominal neuroblastoma is tolerated well in pediatric patients with no added morbidity. The enhanced exposure facilitates resection and improves local control.