Atypical presentations of hydatid cyst in children and their management.

Hydatid cyst (HC) is uncommon in children and usually involves a single organ, the lung being the most common site followed by the liver. A series of 18 children who presented with HC at different organs of the body managed at our institute over a period of 5 years is presented here. The clinico-radiological evaluation was done in all patients followed by pharmacotherapy/surgery and was followed up for 2 years. Demographic data, organs of involvement, clinical presentations and investigations, response to oral albendazole therapy, surgical procedures, operative findings, perioperative clinical courses, and surgical outcomes were recorded. The mean age of presentation was 7.7 years with a male:female ratio of 11:7. All patients were symptomatic at presentation and four (22.22%) had atypical symptoms (obstructive jaundice, bladder outlet obstruction, and acute abdominal pain). Liver HC was more common than lung HC. Three patients (16.6%) had synchronous involvement of the lung and liver/spleen. All the patients underwent surgical excision of the cyst as none of them responded to preoperative pharmacotherapy. Open surgery was done in 15 patients and laparoscopic excision was carried out in three (16.6%). Two patients had abnormal communications (cysto-biliary/cysto-bronchial), which were managed successfully. Neither any major perioperative morbidity nor mortality was nor any recurrence was seen in a 2-year clinical follow-up, no significant perioperative morbidity or mortality occurred, and no recurrence was noted. In conclusion, single organ HC is more common in children, with more prevalence of hepatic than pulmonary HC. Early surgical excision of the cyst should be considered (preferably laparoscopic whenever possible) instead of pharmacotherapy.

Spectrum of pediatric kidney tumours with special references to WT1 immunostain at a tertiary care hospital.

Context or Background: Pediatric renal tumours are the second most common solid tumours in children. The most common in this group is Wilms tumour with mesoblastic nephroma being the 2nd most common tumour in children, younger than 3 months. Aims and Objectives: The present study was conducted to study the epidemiological occurrence of pediatric renal tumours at a tertiary care hospital and to study the diagnostic efficacy of WT1 immunostaining in distinguishing Wilms tumour from other types of renal tumours. Materials and Methods: It was a single institution-based prospective and observational study conducted for 2 years (from October 2013 to September 2015) in the department of pathology in our hospital. A total of 50 cases were enrolled in this study all were below 15 years of age. Results: Nephroblastoma or Wilms tumour was found to be the most common type, occurring in 66% cases. Fourteen out of 33 cases of Wilms tumour showed triphasic components (blastemal, epithelial, and stromal) with Blastemal type Wilms being the second most common (11 cases). WT1 immunostaining was positive in 93.9% cases of nephroblastoma. The highest amount of nuclear positivity noted in blastemal cells followed by epithelial cells. Rhabdomyoblastic differentiation and regressive variant showed nonspecific cytoplasmic staining. Cystic partially differentiated nephroblastoma and diffuse anaplasia type Wilms tumour showed nuclear staining in blastemal cells. Conclusion: The expression of WT1 immunostain was found to be diagnostically significant in differentiating Wilms tumour from other renal tumours.

Role of EGFR and HER-2/NEU Expression in Gall Bladder Carcinoma (GBC).

Background Gall bladder carcinoma (GBC) is the most common malignancy of the biliary tract. Being known for its geographical and racial variations, and compared with the global statistics, its incidence is higher in the Indian subcontinent, mainly in the northern and eastern regions, accounting for 80 to 95% of cases. Aims and Objectives This study was conducted to evaluate the clinic-pathological spectrum and expression of EGFR and HER-2/NEU in GBCs and to understand their relation to prognosis, paving the way for targeted therapies for better treatment outcomes and patient survival. Materials and Methods This is a prospective study performed in a tertiary care hospital in 30 resected specimens of GBC cases recorded in our Department of Pathology from November 2017 to November 2019. Clinical history including the radiological reports and demographic parameters were included in the study pro forma. Immunohistochemical (IHC) staining for EGFR and HER-2/NEU was performed on all the selected cases. Clinicopathologic parameters like age, sex, histologic type, perineural, and lymphovascular invasion were compared and correlated with EGFR and HER-2/NEU status. Results Expression of EGFR was found in 93.33% of cases, which showed a highly significant correlation with histological tumor type ( p = 0.000). HER-2/NEU expression was found in 56.66% of cases, which also showed a significant correlation with histological tumour type ( p = 0.021). We found most of the cases with strong EGFR immunoreactivity (3+) were poorly differentiated tumors and most of the cases showing weak immunoreactivity for EGFR (1+) were well-differentiated. Conversely, in case of HER-2/NEU immunoreactivity, strong staining (3+) was seen in well-differentiated tumors and weak staining (1+) in poorly differentiated tumors. A significant correlation was also found between EGFR and HER-2/NEU expression ( p = 0.000) and between cholelithiasis and EGFR expression ( p = 0 .033). Conclusion EGFR is expressed in most cases of GBC. Its expression is more in poorly differentiated carcinomas as compared to the well-differentiated carcinomas, whereas HER-2/NEU expression is more in well-differentiated carcinomas. Therefore, they may serve as independent prognostic factors and also as targets for molecular therapy in GBCs.

A study on genetic polymorphism of RET proto-oncogene in Hirschsprung's disease in children.

BACKGROUND: Hirschsprung's disease (HD) is a genetic disorder with a complex pattern of inheritance. Some single-nucleotide polymorphisms (SNPs) are identified to be associated with the risk of Hirschsprung's Disease (HSCR). AIMS AND OBJECTIVES: The aim of this study is to know the association between the rearranged during transfection (RET) proto-oncogene polymorphism and HD and to characterize the SNPs of RET proto-oncogene affecting HD. MATERIALS AND METHODS: The study was conducted in the Department of Pathology in association with the Department of Pediatric Surgery. Blood samples were collected from the patients diagnosed with confirmed HD and from age- and sex-matched controls. This case-control study consisted of 53 HSCR cases and 50 controls. Genotypes of rs1800860 and rs1800861 were analysed in by polymerase chain reaction amplification and sanger sequencing. Associations with the risk of HSCR were estimated by odds ratio (OR) and their 95% confidence intervals (95% CI) using. RESULTS: We observed that in the case of rs1800860A > G genotype AG was not associated with the increasing risk of disease (OR with 95% CI = 0.568 [0.238-1.356]) while genotype GG was associated with increasing the risk of the disease (OR with 95% CI = 2.278 [0.967-5.366]). In the case of rs1800861G > T genotype GT was associated with lowering the risk of the disease (OR with 95% CI = 0.230 [0.0981-0.539]) while genotype TT was associated with increasing the risk of the disease (OR with 95% CI = 9.647 [3.830-24.302]). The difference in the genotypic distribution of GT and TT at rs1800861G > T between Short segment disease (SSD) cases and Long Segment Disease (LSD) and total colonic aganglionosis was made by Fisher's exact test and it was statistically significant (P = 0.0476 and 0.0054). CONCLUSION: The results of this study support the hypothesis that variations in RET pathway might play an important role in the development of HSCR.

Evaluation of diagnostic utility of imprint cytology in paediatric renal tumours with special references to Ki 67 proliferative marker.

BACKGROUND: Pediatric renal neoplasms comprise about 7%-8% of all neoplasms in children. Wilms tumour (WT) is the most common among pediatric renal tumours. AIMS AND OBJECTIVES: The study was undertaken to study the epidemiological occurrence of pediatric renal tumours in a tertiary care hospital and to ascertain the validity and reliability of touch smear imprint cytology in intraoperative diagnosis of renal tumours and correlate with subsequent histopathological diagnosis and to assess the expression of proliferation marker Ki-67 in different components and stages of WT. MATERIALS AND METHODS: It was a single-institution-based prospective and observational study, conducted for 2 years (from October 2013 to September 2015) in the department of pathology at our hospital. A total of fifty cases were enrolled in this study, all were below 15 years of age. RESULTS: Imprint cytology showed sensitivity, specificity, and diagnostic accuracy of 83%, 98%, and 95.74%, respectively, in diagnosing benign and malignant renal tumours. There was statistically significant correlation of imprint cytology with confirmatory histopathological examination of excision specimen (P < 0.001). Immunohistochemical analysis of Ki-67 was done in all WT cases. Epithelial component had higher proliferative index than blastemal component with P = 0.0082, which was highly statistically significant. CONCLUSION: Imprint cytology is found to be a less expensive, simple, and rapid method, which can be used as an adjunct to histopathology. Correlation between proliferation index as measured with Ki-67 antibody and tumour stage was found. Ki-67 is thus a relevant marker for assessing the proliferative activity.

Spectrum of meningioma with special reference to prognostic utility of ER,PR and Ki67 expression.

BACKGROUND: Meningiomas are the most common primary central nervous system neoplasms originating from the arachnoid cap cells and constitute between 13% and 26% of all intracranial tumors. AIMS AND OBJECTIVES: The aim of the study was to analyze the age-, sex-, and site-wise distribution of different histological patterns of meningiomas seen in our center and to assess the status of estrogen receptor (ER), progesterone receptor (PR), and proliferation marker Ki-67 in various grades of meningioma. MATERIALS AND METHODS: A prospective study was done in 90 cases. Patients presented with symptoms of headache and seizure and underwent subsequent excision surgery at Neurosurgery Department were taken. We have studied histological typing and grading of the tumors, and immunohistochemical staining was done for ER, PR, and Ki-67. STATISTICAL ANALYSIS: Two-group comparison was done using Mann-Whitney U-test and Fisher's exact test. Comparison of Ki-67 expression between Grade 1 and Grade 2 meningiomas was determined using Mann-Whitney U-test. Comparison of ER and PR status between different histological grades was done by Fisher's exact test. Two-tailed P < 0.001 was considered statistically significant. RESULTS: According to histological type, meningothelial meningioma is most common (38.8%) followed by transitional (22.2%). PR positivity is seen in 96.34% of Grade 1 tumors, and all Grade 2 tumors were PR negative (Fisher's exact test P < 0.001). About 3.66% of Grade 1 and all Grade 2 tumors were positive for ER (Fisher's exact test two-tailed P < 0.001). Mean Ki-67 labeling index (LI) was 2.57 ± 1.674 among Grade I tumors, 7.11 ± 1.084 in Grade II meningiomas. CONCLUSIONS: Most of Grade 1 meningiomas show PRs positivity and lack of ERs positivity. Meningiomas with higher proliferation index and negative PR are very likely to be Grade II or Grade III. Evaluation of ER, PR status, and Ki-67 labeling index (LI) with histological evaluation helps us in providing information about the biologic behavior of meningiomas.

Laparoscopy in the Evaluation of Impalpable Testes and Its Short-term Outcomes: A 7 Years' Experience.

AIMS: The aim of this study is to report and analyze results of laparoscopy in impalpable testes performed between 2009 and 2016 and its short-term outcomes. MATERIALS AND METHODS: Demographic data, laterality, laparoscopic findings, operative time, procedure, hospital stay, complications, and follow-up data of 76 patients with 79 impalpable testes from 2009 to 2016 were retrospectively collected and analyzed. Successful outcome was defined as maintenance of intrascrotal position with no atrophy at a follow-up of at least 6 months. RESULTS: Impalpable testes constituted 24% of undescended testes in our series. Mean age was 3.9 years. Forty-two patients had left-sided, 31 right-sided, and three bilateral impalpable testes. Of the 79 clinically impalpable testes, on laparoscopy, 3 were vanishing testes, 52 were intra-abdominal (6 high-lying and 46 low-lying), 18 canalicular and 6 nubbin testes. Ultimately, 52 underwent laparoscopic orchiopexy: 46 single-staged orchiopexy and 6 two-staged Fowler-Stephens procedure. Mean operating time was 77 min. Complications were few and mostly minor. Eleven patients were lost in follow-up. On a mean follow-up of 23 months, one testis that underwent single-staged laparoscopic orchiopexy atrophied whereas good size and intrascrotal position were maintained in the rest. CONCLUSIONS: Laparoscopy in impalpable testes was safe, feasible, and effective. Overall outcome was good which was obtained by minimal use of electrocautery, dissection with wide strip of peritoneum and extensive retroperitoneal dissection for mobilization. There is a need for wide reporting of cases from high-volume pediatric surgery centers in India.

Leydig Cell Tumor of Testis in a Child: An Uncommon Presentation.

Leydig cell tumors (LCTs) are rare testicular tumors. Incidence is 1%-3% of all testicular neoplasms, bilateral in 10%. They are frequently hormonally active, leading to feminizing or virilizing syndromes. LCTs can be either pure or mixed with germ cell tumors or other sex cord-stromal tumors. Here, we are reporting a benign pure LCT in a 6-year-old boy presented with pseudopuberty.

Immunohistochemistry-based comparative study in detection of Hirschsprung's disease in infants in a Tertiary Care Center.

BACKGROUND: Hirschsprung's disease (HD) is the major cause of pediatric intestinal obstruction with a complex pattern of inheritance. The absence of ganglion cells along with an analysis of hypertrophy and hyperplasia of nerves in the nerve plexus of submucosa and muscularis mucosae is regarded as a potential hallmark for its diagnosis. AIMS AND OBJECTIVES: This study was undertaken to ascertain the (1) clinical profile, (2) mode of presentation, and (3) to compare the role of calretinin immunostaining with acetylcholinesterase in the diagnosis of HD. MATERIALS AND METHODS: This prospective and observational study was conducted in the Department of Pathology, IPGME & R from June 2014 to May 2015. One hundred and four patients clinically and radiologically diagnosed with HD underwent surgery were included in the study. The data of every patient including age, sex, and presenting symptoms were recorded. Eventually, histopathological, calretinin, and acetylcholinesterase immunohistochemical examination were done. RESULTS: Total numbers of cases studied were 104, which aged between 0 days and 365 days. Male preponderance (76.92%) was noted. The overall sensitivity, specificity, positive, and negative predictive value of acetylcholinesterase were 100%, 86.44%, 84.91%, and 100%, respectively. The concordance of detection of ganglion cells and nerve fibers, and thereby diagnosis of Hirschsprung's and non-HD using calretinin and the gold standard was statistically in strong agreement (κ = 0.749, 95% confidence interval: 0.635-0.863). CONCLUSIONS: Calretinin stands out as the single and indispensable tool that differentiates HD from other mimickers.

Delayed primary end-to-end anastomosis for traumatic long segment urethral stricture and its short-term outcomes.

BACKGROUND:: The purpose of this study is to evaluate the aetiology of posterior urethral stricture in children and analysis of results after delayed primary repair with extensive distal urethral mobilisation. MATERIALS AND METHODS: This was a retrospective study carried out in a tertiary care centre from January 2009 to December 2013. RESULTS:: Eight children with median age 7.5 years (range 4-11 years), underwent delayed anastomotic urethroplasty: Six through perineal and two through combined perineal and transpubic approach. All the eight children had long-segment >2 cm stricture: Three posterior and five anterior urethral stricture. On a mean follow-up period of 33 months (range 24-48 m), all were passing urine with good flow and stream. CONCLUSION:: End-to-end anastomosis in post-traumatic long segment posterior urethral stricture between prostatic and penile urethra in children is possible by perineal or combined perineal and transpubic approach with good results without any urethral replacement.

Intragastric pressure: Useful indicator in the management of congenital diaphragmatic hernia.

AIMS: To assess the role of measuring of intragastric pressure (IGP) during closure of congenital diaphragmatic hernia. MATERIALS AND METHODS: Thirty-two cases were included in the study (August 2008 to February 2014), and IGP has been measured intraoperatively during closure. RESULT: Patients were categorized in group A (15 patients), group B (14 patients) and group C (3 patients). In group C, IGP was more than 30 cm of water pressure and all of tehm required creation of ventral hernia and ventilator support. CONCLUSION: Measurement of IGP as an objective, noninvasive and simple method to evaluate intra-abdominal pressure and prevention of abdominal compartment syndrome and intraoperative IGP value determines the types of abdominal closure.

Uncommon renal tumors in children: A single center experience.

AIMS: Scrutiny over the clinical behaviors, management, and the final outcome of some rare renal neoplasm in order to find out some hidden facts about these tumors which are playing an important role in the disease course and its management. MATERIALS AND METHODS: Retrospective evaluation of uncommon (non-Wilms') renal neoplasm in the pediatric population in a tertiary care center. Fifteen cases of uncommon renal tumors were treated in our institution over the last 5 years (January 2008 to December 2012). The cases were tabulated in the form of age, sex, mode of presentation, preoperative investigations, intraoperative grading, pathological type, postoperative management and the final outcome. The patients were followed up for 2 years (clinically every 3 months and ultrasonography abdomen in every 6 months for first 2 years) in order to see any evidence of recurrence and complications related to postoperative chemotherapy. RESULTS: Out of 15 cases, four cases were clear cell sarcoma (CCS) (26.6%), three cases were rhabdoid tumor (20%), three cases were congenital mesoblastic nephroma (20%), two cases were multilocular cystic nephroma (13.3%), two cases were renal teratoma (13.3%), and one case of teratoid Wilms' tumor (6.6%). There were two deaths (one CCS and one rhabdoid tumor) due to chemotherapy-related toxicity but no recurrence. Three patients were lost during postoperative follow-up; ten patients are doing well and getting a regular visit in the follow-up clinic. CONCLUSION: The clinical presentations of these uncommon renal tumors are similar to that of Wilms' tumor. Thus, preoperative diagnosis is difficult even with modern imaging techniques. Some of these tumors (CCS, rhabdoid tumor) are rapidly progressing and have a poor outcome. Hence, early intervention in the form of complete surgical resection of the tumor (whenever possible) and postoperative chemo/radiotherapy are imperative for fruitful outcome.

Hirschsprung's Disease in Neonates with Special Reference to Calretinin Immunohistochemistry.

BACKGROUND: Hirschsprung's disease is a classic example of a complex genetic disease, characterized by the lack of enteric ganglia in the submucosal and myenteric plexuses, along variable portions of the distal gut. The diagnosis of Hirschsprung's disease is based on a combination of clinical features, radiological appearance of the bowel and histological features in Haematoxylin & Eosin stained sections of intestinal biopsies. Calretinin Immunohistochemistry is emerging to be one of the newer methods. AIMS AND OBJECTIVES: This study was undertaken to ascertain 1) clinical profile; 2) mode of presentation; 3) to evaluate the role of Calretinin immunostain in the diagnosis of Hirschsprung's Disease. MATERIALS AND METHODS: This prospective and observational study was conducted in the Department of Pathology IPGME&R from July 2013 to September 2014. Eighty nine patients, clinically and radiologically diagnosed with Hirschsprung's disease underwent surgery and were included in the study. The data of every patient including age, sex and presenting symptoms were recorded. Eventually, histopathological examination & immunohistochemistry were done. RESULTS: Total number of cases studied was 89 which aged between 0 days to 28 days. Overall sensitivity in our study to diagnose presence or absence of ganglion cells by calretinin immunohistochemistry was 100% and the specificity is 97.44% with positive and negative predictive value of 84.62 % and 100 % respectively. CONCLUSION: Calretinin immunohistochemistry holds several advantages, and it's simple and not doubtful; and it is either positive or negative.

Colonic atresia associated with annular pancreas: An extremely rare and previously unreported association.

Colonic atresia is the rarest entity among the all intestinal atresias and may be associated with anterior abdominal wall defect, small intestinal atresia, Hirschsprung's disease, and other anomalies. Here, we are reporting a case of colonic atresia associated with annular pancreas, which has not been reported previously to the best of our knowledge.

Congenital cystic lesions of lung in the paediatric population: A 5-year single institutional study with review of literature.

BACKGROUND: The aim was to evaluate the clinical presentation, investigation modalities, operative management, pathology, outcome (morbidity and mortality) and short term follow-up of congenital cystic lesions of the lung. MATERIALS AND METHODS: This is a retrospective study. Study period was 5 years (December 2008-November 2013) in the Department of paediatric surgery, Nil Ratan Sircar Medical College. STUDY POPULATION: Total number = 10 patients. Age range: 2 days-7 years. (Neonate-4). Male and female ratio = 1:1. RESULT: Among 10 cases of lung cyst four having congenital lobar emphysema, four having congenital pulmonary airway malformation, one sequestration and one teratoma. All patients have undergone surgical excision in terms of lobectomy or excision of the lesion. Post-operative histopathology confirmed the diagnosis. Recovery was uneventful. CONCLUSION: Although our experience is limited, operative management of lung cysts seems to be safe with rewarding results. However we are yet to encounter many of the other varieties of the cysts found in the lung, which may be associated with other congenital anomalies and have an impact on prognosis.

Patent Urachus in a Neonate: a Rarity.

Here, we present a pictorial description of a rare case of patent urachus in a neonate and its management.

Esophageal atresia with tracheoesophageal fistula associated with situs inversus totalis.

Esophageal atresia with tracheoesophageal fistula with situs inversus totalis is an extremely rare association. We are presenting a case of a preterm neonate suffering from similar condition. Fistula ligation with primary repair was done via left thoracotomy to avoid technical difficulties. Preoperative echocardiography should be done to confirm dextrocardia and disposition of the aortic arch.