Evaluation of CD133 and CD56/NCAM expression in Wilms tumor and their association with prognostic factors.

OBJECTIVES: To validate certain markers for cancer stem cell populations and their clinical importance in Wilms tumor (WT). MATERIALS AND METHODS: Immunohistochemical study for CD133 and CD56/NCAM was performed on forty-six cases of WT that were diagnosed between 1999 and 2015, and the association of these markers with survival and prognostic factors was analyzed. RESULTS: Thirty-four (73.9%) of WTs were positive for CD133 and thirty-nine (84.8%) were positive for CD56/NCAM. A significant positive correlation between CD133 and CD56/NCAM expression and the National Wilms Tumor Stage (NWTS) and death was found. Moreover, overall survival time was significantly correlated with CD133 and CD56/NCAM H-score, NWTS stage, and death. CONCLUSION: It seems that CD133 and CD56/NCAM expressions can be used as strong prognostic parameters for the survival of patients with WT and can be used to predict WT patients' stage. Moreover, their targeted therapies can abolish cancer stem cells in children with recurrent tumors.

Colonic basidiobolomycosis in a child: report of a culture-proven case.

Basidiobolomycosis is a rare fungal disease caused by Basidiobolus ranarum, which is endemic in tropical and subtropical regions of Africa, Asia, and Latin America. Gastrointestinal basidiobolomycosis poses diagnostic difficulties due to the non-specific clinical presentation and absence of predisposing factors. Eight pediatric cases of the disease have been reported recently from Iran, but none of these, or most of the other cases reported in the literature, has been proven by culture, which is the gold standard method for diagnosis. We present a case of culture-proven colonic basidiobolomycosis that occurred in a 3-year-old boy. The outcome was successful following surgical excision and antifungal therapy with posaconazole.

"Tie over ring" sutureless compression based gastrointestinal anastomotic method: experimental rat model.

INTRODUCTION: Giving the ever-rising trend of pediatric minimally invasive surgery besides early neonatal surgical interventions, intestinal anastomosis turns out to be a time consuming stage due to several anatomical as well as technical difficulties. A perfect bowel anastomosis method should be easy, rapid, safe and reliable in creation of bowel continuity with minimal tissue damage. In this light, sutureless anastomotic methods have been introduced, using compression based anastomosis with biofragmentable rings or powerful magnets. Accordingly, this experimental animal model study has evaluated the result of an easy, rapid intestinal sutureless anastomotic technique via simple tying over an intraluminal ring, in comparison with conventional handsewn bowel anastomosis. METHODOLOGY: Thirty Wistar-Albino male rats were enrolled and small bowel was transected via a midline laparotomy. A grooved plastic ring was inserted into the ileal lumen and both intestinal cutting ends were fixed over the ring with a simple tie in the first group. On the other hand, enteroenterostomy was performed by the conventional method of handsewn anastomosis in the second group. After 14 days, rats were sacrificed to evaluate for intraperitoneal adhesion and abscess formation in addition to other evidences of anastomotic leakage. Furthermore, the anastomotic site integrity, tensile strength and healing stage were assessed microscopically. RESULTS: The mean operative time and intraoperative bleeding in the tie over ring group were significantly less than those in the handsewn anastomosis group. Anastomotic stricture was more common in the conventional anastomosis group while the anastomotic tensile strength was significantly higher in the tie over ring group. Histopathological healing parameters and final healing score were almost similar in both groups but mean inflammatory cell infiltration in handsewn anastomosis was significantly higher. CONCLUSION: "Tie over ring" is a simple method of anastomosis that is feasible, fast, safe and functionally effective for bowel reconstruction in animal models that could be reconsidered in human bowel anastomosis.

Tailgut cyst in an infant with imperforate anus: a case report.

BACKGROUND: Tailgut cyst (TGC) is a rare congenital lesion that originates from remnants of the embryonic post-anal gut. It presents as a multilocular presacral mass mainly in young women. Microscopically, the cyst lining is composed of different types of epithelium such as stratified squamous, transitional, or glandular. CASE PRESENTATION: We present a term female newborn referred to our hospital for evaluation and management of imperforate anus. During dissection of the presacral space to release the rectum, a multicystic mass adherent to the distal part of rectum was detected and completely excised. Histopathology confirmed the TGC diagnosis. CONCLUSION: TGC is a very rare lesion, but it should be considered in differential diagnosis of any presacral mass, even in infancy. Complete excision is the preferred treatment and can be done more easily neonatally or in infancy.

Pediatric clear cell sarcoma of the kidney with atriocaval thrombus.

Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for almost 3 % of pediatric renal tumors. Cavoatrial tumor thrombosis is very rare and because of poor response to chemotherapy, invasive surgical interventions such as open heart surgery may be indicated. A 6-year-old girl with CCSK of right kidney was treated with neoadjuvant chemotherapy. According to poor chemosensitivity, surgical intervention was planned. Right atriotomy was done, but intra-atrial part of tumor was very firm and unsuctionable; so the procedure was completed by laparotomy and en bloc resection of tumoral kidney and its cavoatrial extension through a limited venotomy on inferior vena cava. Although radical resection of CCSK with intracaval involvement should be considered as a multidisciplinary approach and intensive care and supports should be provided, atriocaval tumor in growth in CCSK is firm, non friable and non-adherent, and tumor en bloc resection may be possible through a limited venotomy via primary abdominal approach.

Calretinin Immunohistochemistery: An Aid in the Diagnosis of Hirschsprung's Disease.

OBJECTIVES: Definite diagnosis of Hirschsprung's disease (HD) is based on histopathological study, but there are limitations associated with standard histology and histochemistry in this regard. The aim of this study was to investigate calretinin immunostaining patterns in both ganglionic and aganglionic HD intestinal specimens and to compare them with control specimens. MATERIALS AND METHODS: Specimens included 30 patients with histopathologic diagnosis of HD and 20 patients that underwent colectomy for other reasons (as control group). Eighty paraffin wax blocks of full thickness intestinal specimens (30 blocks of ganglionic segments, 30 blocks of aganglionic segments and 20 blocks of control group) were studied. Calretinin immunoreactivity and pattern of staining for ganglion cells (nuclear and cytoplasmic) and also nerve fibers in different layers of bowel were evaluated in IHC stained slides. RESULTS: There were positive immunostaining of nerve fibers in the lamina propria, submucosa and muscularis propria in control and patient group. There were also nuclear and cytoplasmic staining of ganglion cells in submucosa and muscularis propria in all specimens of both control group (100%) and ganglionic segments (100%). Calretinin immunoexpression of nerve fibers in muscularis propria of the aganglionic segments was negative in all but two cases (6.7%). This method had sensitivity of 93.3% and specificity of 100% for diagnosis of HD in full thickness specimens of intestinal wall. The positive predictive value was 100% and negative predictive value was 93.8%. CONCLUSION: Calretinin immunohistochemistry can be used on suction rectal biopsies as a reliable and adjunctive method to diagnose HD.

Multiple congenital epulis of the mandibular ridge: a case report.

Congenital epulis is a very rare benign soft-tissue tumor of uncertain histogenesis, which is also known as "gingival granular cell tumor of the newborn". It occurs almost exclusively as a single tumor along the alveolar ridge of the maxilla in newborn females. Although congenital epulis is strikingly similar to the more common adult granular cell tumor histologically, in contrast to the latter congenital epulis cells are negative for S-100 protein. This case report describes a 15-day-old female infant with multiple congenital epulis of the mandibular alveolar ridge.

Ameloblastic fibrosarcoma of the maxillary sinus in an infant: a case report with long-term follow-up.

Ameloblastic fibrosarcoma (AFS) or ameloblastic sarcoma is an extremely rare odontogenic neoplasm. The authors report AFS in the maxillary sinus of a 4-month-old boy. The tumor was composed of odontogenic epithelium, resembling that of ameloblastoma, and a mesenchymal part exhibiting features of fibrosarcoma. We also found some areas with deposition of dentinoid material closely adjacent to the ameloblastic epithelium. Although AFS has occurred in a wide age range, this is the first report of this tumor in infancy with long-term follow-up.

Tumor induced hypercalcemia in a patient with mediastinal synovial sarcoma.

Tumor-induced hypercalcemia is a frequent complication of advanced cancers, but it has been rarely reported in patients with sarcoma. We report a 16 year-old boy presenting with polyuria, polydipsia and severe dehydration. Laboratory examination revealed severe hypercalcemia (serum calcium 23 mg/dl) which caused emaciation and was accompanied by low serum phosphorus and suppressed parathyroid hormone. Diagnostic imaging revealed a huge anterior mediastinal mass. Hypercalcemia was successfully treated with pamidronate, a bisphosphonate, and the patient underwent surgical resection. Pathological and immunohistochemical analyses confirmed a diagnosis of biphasic synovial sarcoma. To our knowledge, this is the first case of mediastinal synovial sarcoma presenting with hypercalcemia.

Renal splenic heterotopia with extramedullary hematopoiesis in a thalassemic patient, simulating renal neoplasm: a case report.

Intrarenal splenic tissue (splenorenal heterotopia) is a rare condition. Simultaneous existence of splenorenal heterotopia and extramedullary hematopoiesis in a same patient can present as a mass of kidney. We present an interesting case of splenorenal heterotopia with extramedullary hematopoiesis in a thalassemic child simulating as a renal neoplasm. This is the first reported case in the right kidney.