Retinoblastoma is the most common intraocular tumor in childhood. In majority of early stage retinoblastoma, the eyeballs as well as vision can be preserved with chemotherapy and local intraocular therapy with laser or photocoagulation. However, more than half the patients in India and other developing nations present in advanced stage of the disease. This article reviews the genetics, clinical approach, and treatment options for retinoblastoma focussing on advances in chemotherapy for intraocular retinoblastoma (chemoreduction), as well as improvement in survival in advanced retinoblastoma with surgery, chemotherapy, radiotherapy, and bone marrow transplantation.

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Wilms' tumors is a renal malignancy commonly occurring in childhood with classic histopathological features. While the majority of the tumors present as renal masses, atypical presentations like extrarenal masses have also been known. However, the exact natural course of the disease and its outcome is not known. Here, we present two cases of extrarenal Wilms' tumor, discuss the pathophysiology and review the literature for the prognostic factors and the treatment outcome.

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Fetus-in-fetu (FIF) is a rare congenital condition in which a vertebrate fetus is incorporated within its host, a newborn or an infant. We report an interesting case of FIF presenting as an abdominal mass with respiratory distress in a 4-month-old girl, and review the existing literature. An ultrasound abdomen showed a right sided cystic mass containing multiple calcifications. Laparotomy revealed a well-encapsulated right retroperitoneal mass above the right kidney with two rudimentary limbs attached to an amniotic bag by an umbilical cord. Excision of the capsule revealed a yellowish fluid and an incompletely developed fetus covered by vernix caseosa. Pathologic examination showed an irregular fetiforme mass, weighing 1200 gm, with two rudimentary limbs, encephalus, stomach, duodenum, bowel, bone, cartilage, bone marrow, upper and lower respiratory tissue, and spleen. Complete excision of the FIF was performed. The postoperative period was uneventful and the patient was discharged on the seventh postoperative day.

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Positron emission tomography (PET) is a functional diagnostic modality, which is routinely used in oncology for diagnosis, staging, monitoring the response to therapy, and restaging of various tumors. PET used in conjunction with computerized tomography (CT) (PET-CT) is more useful as it provides anatomical details and the abnormal uptake on PET can be localized with highest accuracy. Much work has been done regarding PET/PET-CT in the adults but there is paucity in the same in pediatric group. A child must receive special attention when embarking on what is uncertain and often a frightening procedure. While performing PET in pediatric patients, emphasis is given to patient consent, intravenous access, bladder catheterization, and sedation. Based on existing literature and our own experience, we understand that PET-CT can play a very important role in initial staging, restaging, and evaluation of treatment response in lymphoma, neuroendocrine tumors and bone, and soft tissues sarcoma.

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Fine needle aspiration cytology (FNAC) is a useful pre-operative procedure in pediatric renal tumors. It is a safe technique that does not upstage the tumor and permits positive diagnosis of Wilm's tumor in almost all aspirates with use of immunocytochemistry for cytokeratin and WT-1. Advanced information on diagnosis of Wilm's tumor, especially, the presence of stroma predominance or anaplastic change is a guide to selection and monitoring of chemotherapy. Early diagnosis of clear cell sarcoma of the kidney (CCSK) by FNAC helps in the evaluation of bone metastasis and selection of Doxorubicin-based chemotherapy protocols to which the tumor is responsive. Thus, the routine use of FNAC is helpful for further investigation and management of pediatric renal masses.

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Background : In this study, we show the advantages of partial splenectomy (PS) over total splenectomy (TS) regarding the chances of overwhelming postsplenectomy sepsis (OPSI). Materials and Methods: From February 1991 to December 1999, 143 cases of β-thalassemia underwent PS. 1/3, 1/4 of the splenic tissue was preserved. One hundred and ten cases were followed for an average of 5 years. None of the patients received vaccination or prophylactic antibiotics. Pre- and postoperative hematological profiles, IgM levels, recurrence of hypersplenism and septic episodes were compared among the data of 60 cases TS; all these cases have been operated in the same hospital. Results: Hematological profile significantly increased and transfusion requirement approximately reduced to three-fold. After 3 years, 22.7% in PS and 13.3% in TS groups required the same amount of preoperative transfusion. After 5 years, these percentages were 27.3 and 18.3%, respectively. Two patients in PS and six in TS group developed signs of sepsis. Conclusion : Vaccination or prophylactic antibiotics are not necessary after PS. The risk of sepsis in PS without antibiotics is less than that in TS with antibiotics, and resplenectomy after PS is not associated with serious complications.

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Solid pseudopapillary tumor of the pancreas (SPT) is an extremely rare pancreatic tumor, which has a low malignant potential and occurs mainly in young women. We report a case of SPT arising from the head of the pancreas in a 13 year-old girl who presented with pain in the epigastrium and was treated successfully by local excision of the tumor with preservation of duodenum. A short review of the literature is also included.

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Purpose: To determine the prevalence, outcome and prognostic factors in children with hemolytic uremic syndrome (HUS) who underwent laparotomy. Materials and Methods: The medical records of 104 patients with HUS who presented to our center between 1986 and 2003 were reviewed retrospectively. Data were analyzed using Student's t test for comparing means, Fisher's exact test for frequencies and Pearson's correlation for finding the correlations. Results: 78% of cases presented with vomiting and diarrhea. Seven out of 104 needed surgical exploration. The indications of surgery were acute abdomen, severe abdominal distention and the sign of peritonitis. The findings at laparotomy were intussusceptions, perforation (colon, ileum), gangrene of entire colon, rectosigmoidal tearing, duodenal obstruction and toxic megacolon. Pathological findings were transmural infarction in two cases in which staged surgical management was performed (cecostomy, resection, later anastomosis). Four out of seven patients died because of pulmonary failure, coma and multiple organ failure ( P<0.05) compared to those who did not need laparotomy. The patients requiring surgery were young (<3 years), had high leukocyte count (>20000 mm ³ ) and low albumin level (<3g/dl) ( P<0.05). Conclusion: Surgical complications of HUS are rare but are assorted with high mortality due to respiratory failure and multiple organ failure. Early decision of laparotomy associated with intensive care, including mechanical ventilation, adequate dialysis and ultrafiltration, are recommended.

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Cystic hygroma is a disfiguring benign lesion commonly observed in the neck and face regions of children. The common complications of these malformations are respiratory obstruction, dysphagia, infection, and hemorrhage. Here, we present an uncommon complication of spontaneous rupture of a cystic hygroma. A two-day-old male baby presented with a swelling that was cystic, non-compressible, non-pulsatile and the transillumination was negative. With a clinical diagnosis of a massive cystic hygroma of the neck, CT scan was performed that demonstrated a large multiloculated cystic lesion. On the second day after admission, the swelling burst spontaneously with drainage of about 1000 ml of hemorrhagic fluid. The swelling shrunk considerably after that. The patient underwent surgical excision of the lesion and had an uneventful postoperative recovery. The histopathological examination confirmed the diagnosis of cystic hygroma.

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Objective: To report our experiences with surgical diagnostic procedures in patients with mediastinal pathologies. Materials and Methods: From 1995 to 2004, seven children with mediastinal pathology had seven surgical procedures for the purpose of histological tissue diagnosis. Of these seven patients, five were male and the average age was 11 years. Anterior mediastinotomy was performed in all patients. Results: Tissue diagnosis was achieved in all cases, enabling a specific diagnosis. The diagnoses were lymphoblastic lymphoma ( n = 1), Hodgkin disease ( n = 1) and thymus hyperplasia ( n = 5). There was no operation-related mortality although one patient required a postoperative blood transfusion. Conclusion: Anterior mediastinotomy is a safe surgical procedure, which can be performed quickly in children with a high diagnostic yield.

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Melanocytic neuroectodermal tumor of infancy is a relatively rare pigmented neoplasm that primarily affects the maxilla of infants. Pigmentation depends upon the amount of melanocytic element present. We report a case of melanocytic neuroectodermal tumor in a three month-old child with a greater extent of neuroblastic differentiation.

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Aldosterone-secreting adrenocortical adenoma is an unusual cause of secondary hypertension in childhood. We describe an eight year-old female child who presented clinically with weakness of lower limbs, headache and hypertension. Thorough clinical, laboratory and radiological evaluations confirmed the diagnosis of hyperaldosteronism due to a left adrenocortical adenoma. Left adrenalectomy after preoperative stabilization resulted in satisfactory recovery. Histopathological examination confirmed the diagnosis of an aldosteronoma. We review our experience with this rare entity and the pertinent literature.

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Benign mesenchymoma is a rare tumor composed of mature fat, thin small and medium-sized vessels and spindle cells of smooth muscle origin and has been found in variable locations. There have been only three reports of such cases in the retroperitoneal location. We report one such rare retroperitoneal tumor.

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The paper reports an infant presenting with leiomyosarcoma of the small intestine. The patient presented with intermittent abdominal pain. Examination revealed a hard and mobile intraperitoneal mass. The tumor arose from the mid-ileum with regional lymphadenopathy. Excision of the tumor along with the involved bowel was performed followed by three cycles of chemotherapy. Histological diagnosis was that of a low-grade malignant leiomyosarcoma of the small intestine. Surgical excision was followed by three cycles of chemotherapy. After surgery and three cycles of chemotherapy, the patient was followed up for four years with no evidence of recurrence or metastasis. Surgery followed by chemotherapy was curative for leiomyosarcoma in our patient. Intestinal leiomyosarcoma should be kept as a differential diagnosis for mobile solid intraabdominal tumors in childhood.

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Leiomyoma of the mesentery is an uncommon tumor among gastrointestinal stromal tumors. This has been documented in adults and children. However, there is only one case reported in an infant and we are presenting such a rare second case in a 6-month-old male infant. Exploration of the abdomen revealed a solid mass within the leaves of the mid-ileal mesentery that could be enucleated out entirely after careful dissection. Histopathology suggested it to be leiomyoma of mesentery.

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Massive left sided Wilm's tumours can be associated with significant distortion of the vascular anatomy. An anatomical explanation of mistaken ligation of the right renal artery will be provided in this article so that such a catastrophic complication can be avoided.

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