Aim of the Review: To describe the significant improvement in the diagnosis, treatment and outcome of children diagnosed with hepatoblastoma (HB) that has occurred in the past four decades. Recent findings are mainly focused on lessons learned from the experiences of the Childhood Liver Tumors Strategy Group (SIOPEL). Important milestones were the risk stratification of HB that allowed to tailor down therapy for standard-risk HB and intensify treatment for high-risk HB. The multi-institutional international cooperative SIOPEL trials are reviewed and current treatment guidelines are given. Intensified cooperation between the SIOPEL and the Children's Oncology Group (COG) and the national study groups from Germany (GPOH) and Japan (JPLT) led to the acceptance and use of one staging system (PRETEXT) and the formation of a single robust database containing data of 1605 HB patients. This will allow analysis with enough statistical power of treatment directing factors that will form one of the bases of the next-generation clinical trial that is currently designed by all four collaborating study groups. Summary: Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.

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Pediatric germ cell tumors (GCT) are rare tumors: 80% are benign, 20% malignant (2-3% of all malignant pediatric tumors). The gonadal sites (ovary and testis) account for 40% of cases. Ovarian GCTs: Represent 30% of GCTs and 70% of neoplastic ovarian masses, being the most common ovarian neoplasms in children and teenagers. Benign and immature forms (teratomas) constitute about 80% of all ovarian GCTs, malignant forms represent 20% increasing during adolescence. The most common malignant entity in children is the yolk sac tumors (YST); dysgerminoma is frequent during adolescence and being bilateral in 10% of cases. Presentation is similar in malignant and benign lesions; abdominal pain (70-80%) and lower abdominal mass are common symptoms. Evaluation of alpha-fetoprotein (αFP) or beta subunit of human chorionic gonadotropin (βHCG) is essential to address the nature of the tumors: Their elevation means presence of malignancy. Surgery includes intraoperative staging procedures and requires ovariectomy or ovarosalpingectomy for malignant lesions, but may be conservative in selected benign tumors. Since malignant GCTs are very chemosensitive, primary chemotherapy is recommended in metastatic or locally advanced tumors. Testicular GCT: Represent 10% of pediatric GCT, and about 30% of malignant GCT with two age peaks: Children <3 years may experience mature teratoma and malignant GCTs, represented almost exclusively by YST, while adolescents may also show seminomas or other mixed tumors. The main clinical feature is a painless scrotal mass. Surgery represents the cornerstone of the management of testicular GCTs, with an inguinal approach and a primary high orchidectomy for malignant tumors, while a testis-sparing surgery can be considered for benign lesions. A retroperitoneal lymph node (LN) biopsy may be necessary to define the staging when the involvement of retroperitoneal LN is uncertain at imaging investigations. Conclusion: Patients with gonadal malignant GCTs fare better than those with extragonadal mediastinal germ cell tumors (MGCTs) and survival rate exceeds 90% in localized forms. Chemotherapy has significantly improved the outcome of malignant forms since the introduction of platinum based regimens. The surgical procedure has to be performed in agreement with the ongoing protocols.

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Intravascular extension of Wilms' tumor is a well-recognized phenomenon. Intravascular extension into the vena cava occurs in only 4-8% of patients with Wilms' tumors and intraatrial extension occurs in around 1-3% of patients. This review of the published literature in this cohort aims to summarize the findings of different case series to provide an optimum management plan. A literature search was performed and index papers were retrieved for review. The search included the following terms: Intracaval, intravascular, intraatrial and intracardiac extension of Wilms' tumor or nephroblastoma. The management of patients with intravascular tumor thrombus in Wilms' tumor is complex. A skilled multi-disciplinary team at a tertiary referral center with cardiothoracic surgery available should manage these patients. Multi-modal diagnostic and preoperative imaging are required to confirm and define the extent of the extension. Preoperative chemotherapy is advocated for all but exceptional circumstances and must be followed closely. Surgical resection should be planned according to the stage of intravascular extension with possible need for cardiopulmonary bypass and deep hypothermia with cardiac arrest if required. Surgical complications are more common in this group of patients, but outcome is comparable to those without intravascular extension, and is more closely correlated with the histological subtype then stage of intravascular extension. Operative imaging are required to confirm and define the extent of the extension.

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We report the identification of Kaposiform hemangioendothelioma of the cecum presenting as colocolic intussusception in a 4-month-old boy without Kasabach-Merrit syndrome. To our knowledge this is the first reported case in the literature of such a presentation.

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Inflammatory myofibroblastic tumor occurring at intra-abdominal sites in children can be confused with malignancy because of its large size and location. It is a tumor classified as 'intermediate' between benign and malignant, but usually benign, with a strong tendency for recurrence. Treatment is surgical excision. Here, we present a brief outline of three such cases presenting as abdominal mass in infants.

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Purpose: To evaluate the outcome of children with bilateral Wilms' tumor (BWT) treated on All India Institute of Medical Sciences-Wilms Tumor-99 (AIIMS-WT-99) protocol. Materials and Methods : All children with BWT, registered in our solid tumor clinic from August 1999 through December 2010 were included. Results: Of the 178 fresh cases of Wilms Tumor (WT) treated during this period, 11 (6.2%) had bilateral involvement. All patients except one (12 and 3 cm), had massive bilateral tumors of more than 10 cm on each side. There were eight boys and three girls in the age range 6-30 months. One patient had Denys-Drash syndrome. Twenty renal units were operated upon (12 tumorectomy, five partial nephrectomy, and three nephrectomies), while one patient with inferior vena cava (IVC) thrombus died of renal failure. Tumor spill occurred in three units, lymphnode was positive in two patients. Local recurrence occurred in four patients (six of 18 renal units (33%)-two bilateral and two unilateral). There was one recurrence in the liver that was treated with radio-frequency ablation. The 5-year overall survival (OS) was 90% (95% confidence interval (CI) = 50.8-98.6) and the relapse free survival (RFS) was 38% (95% CI = 6.1-71.6). Conclusion: Massive BWT respond poorly to preoperative chemotherapy, are often not amenable to partial nephrectomy/tumorectomy and have a higher local recurrence rate, giving a poor RFS.

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Aim: To analyze our experience with laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis for the lessons that we learnt and to study the effect of learning curve. Materials and Methods: This is a retrospective analysis of case records of 101 infants who underwent laparoscopic pyloromyotomy over 6 years. The demographic characteristics, conversion rate, operative time, complications, time to first feed and post-operative hospital stay were noted. The above parameters were compared between our early cases (2007-2009) (n = 43) and the later cases (2010-2013) (n = 58). Results: 89 male and 12 female babies ranging in age from 12 days to 4 months (mean: 43.4 days) were operated upon during this period. The babies ranged in weight from 1.8 to 4.7 kg (mean: 3.1 kg). Four cases were converted to open (3.9%): three due to mucosal perforations and one due to technical problem. The mean operative time was 45.7 minutes (49.7 minutes in the first 3 years and 43.0 minutes in the next 3 years). There were 10 complications-4 mucosal perforations, 5 inadequate pyloromyotomies and 1 omental prolapse through a port site. All the complications were effectively handled with minimum morbidity. In the first 3 years of our experience the conversion rate was 9.3%, mucosal perforations were 6.9% and re-do rate was 2.3% as compared to 0%, 1.7% and 6.9%, respectively, in the next 3 years. Mean time for starting feeds was 21.4 hours and mean post-operative hospital stay was 2.4 days. Conclusion: Laparoscopic pyloromyotomy is a safe procedure with minimal morbidity and reasonable operative times. Conversion rates and operative times decrease as experience increases. Our rate of inadequate pyloromyotomy was rather high which we hope to decrease with further experience.

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Although sliding indirect inguinal hernias containing the ipsilateral ovary and fallopian tube are not uncommon in infant girls, sliding hernias containing uterus with both ovaries and fallopian tubes are extremely rare. At surgery, a 5-month-old infant girl was found to have an indirect hernia in which the uterus and fallopian tubes were sliding components with a wide deep inguinal ring.

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Myelolipoma is a rare benign tumor of adrenal gland and rarer in children. Myelolipoma contains adipose tissue and myeloid precursor producing white blood cells (WBC), red blood cells (RBC) and megakaryocytes. Asymptomatic tumor does not require treatment whereas symptomatic tumor needs operation. We are reporting a rare adrenal myelolipoma in a child with review of literature.

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Malignant fibrous histiocytoma (MFH) a pleomorphic sarcoma of uncertain origin was first described by O'Brien and Stout in 1964. It is the most common primary soft tissue sarcoma of late adult life; its occurrence is rare in the pediatric population. MFHs are commonly known to arise in the extremities and the trunk although it can occur almost anywhere in the body. MFH of the scalp is extremely rare; moreover, there is paucity of literature with regards to prevalence of scalp and skull neoplasms. We present an unusual case of a primary MFH involving the scalp of a 5-year-old child and discuss its unusual clinical presentation, histology with immunohistochemistry correlation and its management. Reviewing the literature of primary MFH of the scalp, our patient to the best of our knowledge, is probably the youngest case reported so far.

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Extra-osseous Ewing's Sarcoma (EES) arising from the peripheral nerve is rarely reported in children. Here, we report an instance of EES arising from the left sciatic nerve mimicking an infected hemangioma. This case highlights the need for a high index of suspicion and early histological diagnosis to avoid diagnostic delay.

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Objective: The surgical approach to small bladder template in exstrophy bladder is difficult. Previously, many of these children underwent ureterosigmoidostomy and in recent times, the trend is to do a delayed primary closure. We have used ileal patch as a temporary cover for these small bladders with a view to encourage bladder growth and early results are encouraging. Materials and Methods: In five of the 45 children with bladder exstrophy managed by radical soft-tissue mobilization over 10 years, primary bladder closure was not possible due to repeated failed closures. A detubularized ileum was used to patch the bladder initially and after 4 months the patch was excised and bladder closure with sphincter repair was done in second stage. Results: In five children (three girls and two boys) the mean age at initial bladder closure was 14 months and mean age at ileal patch was 22 months. In four patients, the bladder grew facilitating closure and in one patient it failed. There were no complications with the use of gut in patch. Conclusion: A temporary ileal patch seems promising in managing failed bladder closure in exstrophy patients. Long-term studies are needed before such a technique can be used in all patients with failed primary bladder closures.

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Lateral clefts are rare in occurrence. The lateral cleft is cause by failure of fusion of the maxillary and mandibular dermatomes. It is also associated with preaurical tags. We present a case of a lateral cleft of the lip with multiple bilateral preauricular tags that was repaired using triangular flaps.

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Objective: The most common etiology for gallstones in children is hemolytic diseases; however, the prevalence of nonhemolytic gallstones, which are mostly idiopathic, is increasing. Several studies concerning the treatment of gallstones with respect to the influence of extracorporeal shock wave lithotripsy (ESWL) have been conducted in adults, but not to the same extent in children. Therefore, this study attempted to examine the effects of lithotripsy on idiopathic gallstones in children. Materials and Methods: In this study, 12 children, all of whom were under 12 years of age and diagnosed with idiopathic gallstones, were treated with ESWL. The average age of the children examined in this study was 6.5 years (range 3-11 years). Patients were treated with 2500-3000 shockwaves per session. The number of shockwaves was 90 shocks/min and the impulse intensity ranged from 10 to 12 kV. The final goal was the fragmentation of stones in pieces with less than 3 mm in dimension. Patients were followed up for 6-30 months. Results: A total of 12 patients were treated with ESWL for 14 rounds. In three patients, complete fragmentation occurred within the first trial and was cleared. The nine remaining patients underwent ESWL 10 times in which an acceptable change in the gallstone's condition was not observed. Five of the patients underwent surgery. The chemical composition of the gallstones showed that the dominant element in them was calcium. Conclusions: Our findings show that performing ESWL can be effective in some children. Further studies with larger population are recommended. Furthermore, it seems increasing the voltage intensity and frequency as conducted in adults accompanied with biliary acids prescription can be effective in children.

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