Synchronous Bilateral Wilms Tumor: Five-Year Single-Center Experience with Assessment of Quality of Life.

CONTEXT: Synchronous Bilateral Wilms tumor (sBWT). AIMS: This study aimed to assess the outcome of patients with sBWT treated on SIOP protocol. SETTINGS AND DESIGN: Retrospective and prospective randomized study. SUBJECTS AND METHODS: SIOP 93-01 protocol was used to study nine patients of sBWT in a single center and followed up over a period from 2 to 5 years. STATISTICAL ANALYSIS USED: Unpaired t-test and Mann-Whitney U-test were used for analysis. RESULTS: Of nine patients, six were included in the study as three patients lost to follow-up. Among the six patients, there were four girls and two boys with a median age of 2 years. Mean regression in the size of tumor was 87% in four out of six patients. Tumor with unfavorable histology showed 32% response (ratio of favorable: unfavorable histology 2:1). Event-free survival rate was 81.3% and overall survival was 90% over 2-5 years. Recurrence was seen in two patients of whom one had Denys-Drash syndrome. Mean DTPA glomerular filtration rate was 91.4/ml/min/1.73 m2 preoperatively and that of 3 months after completion of treatment was 84/ml/min/1.73 m2. Health-related quality of life (HRQOL) using Pediatric Quality of Life Inventory and Lansky Play Performance Scale revealed significant improvement results of all functioning domains such as physical, social, emotional, and school subscales with P < 0.05 and performance scale (P < 0.04). CONCLUSIONS: We suggest SIOP protocol for sBWT and bilateral nephron-sparing surgery in two stages. However, long-term follow-up is required to assess the ultimate renal function outcome. HRQOL is an essential guide in improving the conditions of pediatric cancer survivors.

Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature.

Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.

Utility of PAS and ß-catenin staining in histological categorisation and prediction of prognosis of hepatoblastomas.

AIMS: The aim of this study was to assess the usefulness of PAS, ß-catenin and Ki-67 in categorising hepatoblastomas (HBs) and their significance in predicting prognosis. In addition, we have also documented the various chemotherapy induced histologic changes in HBs. METHOD: In this retrospective observational study of 29 cases of hepatoblastomas, 27 cases were considered for statistical analysis, excluding two cases of diagnostic core biopsies. All clinicopathological parameters and follow up data were collected. All HB cases including the mixed epithelial and mesenchymal HBs were classified into two groups: fetal predominant and embryonal predominant type according to the predominant epithelial component. PAS, ß-catenin and Ki-67 staining were done and their correlation with histologic subtypes was assessed. Kaplan-Meier survival analysis was performed in relation to histology, PAS, ß-catenin and Ki-67 staining characteristics. RESULT: Diffuse nuclear staining of ß-catenin was significantly more common in embryonal predominant type (p = 0.036), whereas strong PAS positivity was significantly associated with fetal predominant type (p = 0.002). But no significant correlation was seen between histologic type and Ki-67 staining (p = 0.42). Survival analysis showed cases with diffuse PAS positivity, focal nuclear ß-catenin staining and low Ki-67 LI had better survival. CONCLUSION: PAS is a simple stain and can be utilised in histological categorisation of HB and also predicting its outcome. Nuclear ß-catenin staining which is significantly common in embryonal elements in HB predicts shorter survival.

Usefulness of Wieneke criteria in assessing morphologic characteristics of adrenocortical tumors in children.

PURPOSE: Adrenocortical tumors (ACT) occur rarely in pediatric age group. Pediatric ACTs behave differently from their histologically similar adult counterparts and standard adult criteria often cannot accurately predict their clinical behavior. The aim of the present study was to document the clinicopathologic spectrum of pediatric ACTs and to assess the utility of Wieneke scoring system in predicting clinical behavior of these tumors. METHODS: This multi-institutional study comprised of 13 cases of pediatric ACTs from January 2005 to May 2014. Clinical features and gross pathologic characteristics were obtained from records. Comprehensive analyses of microscopic features were performed. Each tumor was assessed according to criteria proposed by Wieneke et al. and was assigned to benign, intermediate for malignancy or malignant group. The standard adult Weiss criteria were also applied for comparison. RESULTS: There were total 6 cases of adrenocortical adenomas and 7 cases of adrenocortical carcinomas. Most of the children (76.9%) presented with endocrine dysfunction. Lower age of presentation was significantly associated with better prognosis. Applying Wieneke criteria, there were 6 benign and 6 malignant cases and one case was assigned to intermediate for malignancy group. The clinical behavior of all the cases was consistent with Wieneke criteria categorization. Applying Weiss criteria, 3 cases with benign clinical behavior were assigned to malignant group. CONCLUSION: Our study validates the reliability of Wieneke scoring system in predicting malignancy in pediatric ACTs. It is simple and easy to use and therefore useful in day-to-day practice.

Focal nodular hyperplasia of the liver in children: A report of 2 cases.

Focal nodular hyperplasia (FNH) is a benign non-neoplastic lesion of the liver usually found in adults. It is uncommon in children, comprising 2-10% of all pediatric liver tumours. In children, it can occur at all ages, with increased frequency between 6-10 years. We present two cases of FNH in childhood- the first being that of a 5-month-old infant, and the second in a 6-year-old boy. The possibility of congenital FNH had been excluded in the first case. The second case posed diagnostic difficulty initially and was wrongly treated for hepatoblastoma by neoadjuvant chemotherapy, but later correctly diagnosed to be FNH. Both the children are doing well on follow-up. Paediatric FNH though rare, should be kept in mind while dealing with a hepatic mass. Radiological features can be variable and needle sampling may not be sufficient to reach to a diagnosis. Histological examination with glutamine synthetase immunostaining should be performed in doubtful cases to differentiate FNH from other paediatric liver masses, as management differs.

Diagnostic and prognostic utility of SF1, IGF2 and p57 immunoexpression in pediatric adrenal cortical tumors.

BACKGROUND: Adrenocortical tumors (ACT) are uncommon in the pediatric age group. Using the standard Weiss criteria in pediatric tumors leads to overdiagnosis. This has led to the development of newer systems such as Weineke criteria. Ki67 labeling index aids in differentiating adenomas from carcinomas. We aim to evaluate the diagnostic and prognostic role of Ki67 labeling index, along with immunoexpression of steroidogenic factor-1, insulin like growth factor 2 and p57, in pediatric ACTs diagnosed using Weineke criteria. METHODS: We have studied 25 cases of pediatric ACTs. Immunohistochemical staining for Ki67, SF-1, IGF2 and p57 was done in all cases and the result was correlated with the morphological diagnosis using the Weineke criteria. RESULTS: Ki67 labeling index showed complete concordance with the morphological diagnosis. SF-1 and IGF2 showed similar correlation with the diagnosis, with IGF-2 proving to be a more specific marker. Increased Ki67, SF-1 and IGF2 immunostaining also correlated with worse survival. p57 was more specific in determining benign status of a tumor. CONCLUSION: SF-1 and IGF2 are highly sensitive markers of malignancy in pediatric ACTs and can be used in combination with Ki67 expression for optimal diagnostic and prognostic assessment of pediatric ACTs. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

FNAC Aided Diagnosis and Categorization of Hepatoblastoma:: A Report of Three Cases.

Hepatoblastoma is the most common primary malignant hepatic tumour of infancy and early childhood. Histologically hepatoblastomas are categorized into pure epithelial and mixed epithelial-mesenchymal types and epithelial type is further subcategorized into pure fetal type, fetal and embryonal type, pure embryonal, and small cell types. This categorization has been shown to have prognostic and therapeutic implication. Fine needle aspiration cytology (FNAC) is useful in pre-operative diagnosis and categorization in most cases of hepatoblastomas. Periodic acid-Schiff (PAS) stain can be helpful to differentiate fetal subtype from embryonal subtype of hepatoblastoma. Here we describe three cases of hepatoblastomas diagnosed and categorized on cytology with subsequent confirmation on histological examination. Diagn. Cytopathol. 2017;45:77-82. © 2016 Wiley Periodicals, Inc.

Gastric teratoma: A series of 7 cases.

BACKGROUND: Gastric teratoma is a rare entity comprising less than 1% of germ cell tumors of childhood. We present a series of seven gastric teratomas with a review of literature. OBJECTIVE: To study the demographic profile, clinicopathological features and follow-up data of gastric teratomas. METHODS: We did a retrospective analysis of 7 cases of gastric teratomas more than 15years and studied their demographic profiles, clinicopathological features and follow-up data. RESULTS: We came across 7 cases of gastric teratomas out of which 5 were mature and 2 were immature. One case of immature teratoma came back with recurrence and another one had an unusual finding of presence of renal and pulmonary tissues, which has not been reported earlier. CONCLUSION: Ours is the second largest case series of gastric teratomas in the pediatric age group and 2 out of 7 of our cases had immature elements. We also take this opportunity to report a case of gastric teratoma with the unusual histological finding of immature renal and pulmonary tissues, which has not been described previously.

Cytological diagnosis of mesoblastic nephroma: A report of three cases with summary of prior published cases.

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal neoplasm, occurring most commonly in the first few months of life, with a favourable clinical outcome. Accurate pre-operative cytological diagnosis of this entity is important as pre-operative chemotherapy is not recommended and surgery is the treatment of choice. Cytodiagnosis of this rare tumor is discussed in only a few case reports. Here two cases of CMN and one case of cellular congenital mesoblastic nephroma (CCMN) diagnosed on FNAC along with their morphological differential diagnoses has been reported. They also take this opportunity to compare the cytological features of CMN with cellular CMN. Diagn. Cytopathol. 2016;44:823-827. © 2016 Wiley Periodicals, Inc.

Expression of COX-2 and p53 in juvenile polyposis coli and its correlation with adenomatous changes.

INTRODUCTION: Gastrointestinal polyps commonly affect the pediatric population. The commoner variety amongst these is the solitary rectal polyp. Juvenile polyposis coli (JPC) is rare, characterized by multiple polyps occurring throughout the gut. AIM: The role of cyclooxygenase-2 (COX-2) has been implicated in gastrointestinal tumorigenesis. We aimed to look at the clinicopathological spectrum of solitary vs juvenile polyposis and compare their differences in expression of COX-2 and p53. MATERIALS AND METHODS: We studied 38 polyps from eight cases of JPC, collected over the past 10 years along with 40 solitary rectal polyps (SRP). RESULTS: The size of polyps was significantly more in cases of JPC compared to SRP. Adenomatous change was observed significantly more often in JPC. COX-2 expression was also significantly higher in the JPC group compared to SRPs. All cases of JPC polyps with adenomatous change showed strong COX-2 expression. There was no significant difference in expression of p53 in the JPC and SRP groups. CONCLUSION: We observed significantly higher COX-2 expression in JPC. Establishment of the role of COX-2 in JPC will help us formulate chemopreventive therapies as an adjunct to its surgical management.

Weineke criteria, Ki-67 index and p53 status to study pediatric adrenocortical tumors: Is there a correlation?

PURPOSE: Adrenocortical tumors (ACTs) are rare in pediatric age group. Pediatric ACTs behave differently from their histologically similar adult counterparts and Weiss criteria often cannot accurately predict their clinical behavior. Wieneke et al. proposed a set of 9 macroscopic and microscopic criteria for diagnosis of malignancy in pediatric ACTs. The aim of the present study was to validate the Wieneke criteria in pediatric ACTs and to correlate Ki-67 labeling index and p53 expression with the Wieneke score. METHODS: Our study comprised 17 cases of pediatric ACTs more than 11years, from January 2005 to December 2015. Relevant clinical features were obtained from records. Comprehensive analysis of gross and microscopic features was performed, according to the criteria proposed by Wieneke et al. Each tumor was categorized as benign, intermediate for malignancy or malignant. Ki-67 and p53 immunostaining was done in all cases. The patients were followed-up over a period of 6months to 60months. RESULTS: Applying Wieneke criteria, there were 9 benign and 7 malignant cases, and 1 case was assigned as intermediate for malignancy. The most significant markers in favor of malignancy were capsular and venous invasion, followed by the presence of mitotic figures >15/20 HPF. p53 was over-expressed in 86% of the carcinomas. We found a significant correlation between Ki-67 index and Wieneke scoring system. All cases of adenoma achieved complete remission, while 3 patients with carcinoma died. CONCLUSION: Our study validates the utility of Wieneke criteria in differentiating adrenocortical carcinomas from adenomas in pediatric age group. Moreover, Ki-67 index and p53 status can be used as supplementary tools in distinguishing adrenocortical carcinomas from adenomas.

Lipoblastoma and lipoblastomatosis: A clinicopathological study of six cases.

Lipoblastoma and lipoblastomatosis are benign adipocytic tumors seen in children less than 3 years of age. Awareness amongst surgeons of this fast growing lipomatous tumor in young children is important to avoid mutilating surgery and chemotherapy. Histological diagnosis of lipoblastoma is sometimes difficult because of close resemblance with myxoid, round cell, and well-differentiated liposarcoma. We came across a series of six cases of lipoblastoma/lipoblastomatosis, over the last 10 years (2002-2012). Of these, we had three cases of lipoblastomatosis, one of which recurred after 2 months. All others were resected completely and successfully.

Blood glutathione peroxidase and selenium in abortion.

This study was carried out to estimate the levels of glutathione peroxidase and selenium in blood of abortion cases. Glutathione peroxidase and selenium were determined in 52 abortion cases (22 in 1(st) trimester, 30 in second trimester), 45 normal pregnant cases and 25 nonpregnant control cases. The selenium concentration in whole blood and plasma in abortion cases was almost the same as in normal pregnant women but significantly low when compared with the control non-pregnant group. The glutathione levels was higher in abortion cases when compared with normal pregnant and non-pregnant control groups. Red cell and plasma glutathione peroxidase activities of women who had abortion were significantly lower compared with both non-pregnant control group and normal pregnancies.

Metastatic ganglioneuroma: a misnomer.

Neuroblastoma, ganglioneuroblastoma and ganglioneuroma atumors arising from the neural crest cells. Ganglioneuroma is considered as the most mature amongst the three and usually has no metastatic potential. Spontaneous maturation of neuroblastoma into ganglioneuroma is, however, quite well-known. Here, we present a case of an 8-year-old girl child with evidence of metastasis of ganglioneuroma into a lymph node.

Isolated Cushing's syndrome in early infancy due to left adrenal adenoma: an unusual aetiology.

Bilateral macronodular adrenocortical disease as a part of McCune Albright Syndrome (MAS) is the most common cause of endogenous Cushing's syndrome (CS) in infancy. Adrenocortical tumors causing CS in infancy are extremely rare. We report the case of a girl with CS who presented at age 4 months with obesity and growth retardation. Her 8 am paired cortisol and adrenocorticotropic hormone levels were 49.3 µg/dL and <1 pg/mL, respectively with non-suppressed serum cortisol (41 µg/dL) on high-dose dexamethasone suppression test. Abdominal computed tomography scan demonstrated a 5.3x4.8x3.7 cm homogenous left adrenal mass with distinct borders. Laparotomy following pre-operative stabilization with ketoconazole 200 mg/day, revealed a 7.5x5x4 cm lobulated left adrenal mass with intact capsule and weighing 115 grams. Histopathology showed small round adrenal tumor cells with increased nucleo-cytoplasmic ratio and prominent nucleoli. The cells were separated by fibrous septae without any evidence of vascular or capsular invasion- findings consistent with adrenal adenoma. On the 8th post-operative day, after withholding hydrocortisone supplementation, the 8 am cortisol level was <1 µg/dL, suggestive of biochemical remission of CS. The patient improved clinically with a 7.5 kg weight loss over the next 3.5 months. This is perhaps the youngest ever reported infant with CS due to adrenal adenoma. Lack of clinical and biochemical evidence of hyperandrogenism as well as the benign histology in spite of the large tumor size (>7 cm diameter; 115 g) are some of the unique features of our patient.