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.

Primary Renal Rhabdomyosarcoma: An Unusual Bone Metastasizing Tumor of Kidney.

Rhabdomyosarcoma (RMS) is one of the common malignant tumors in the pediatric age group. There is only a single case report of primary renal alveolar RMS. Fine needle aspiration (FNA) findings of primary renal RMS has not been reported so far. Hence we present an unusual case of primary alveolar RMS of the kidney. An 11 year old boy presented with an abdominal mass. On FNA a diagnosis of undifferentiated sarcoma and anaplastic Wilms tumor were considered. The tumor was resected and showed histopathological features of alveolar rhabdomyosarcoma. He developed multiple bony metastases and succumbed to the illness despite aggressive chemotherapy. RMS of the kidney should be considered in the differential diagnosis of children with a renal mass, and may have an aggressive clinical course with bone metastases.

The Bethesda system for reporting thyroid fine needle aspirates: A study of 1763 patients; with histopathological correlations, in eastern India.

BACKGROUND: Fine-needle aspiration cytology (FNAC) is an important test for triaging patients with thyroid nodules. The 2007 National Cancer Institute Thyroid Fine-Needle Aspiration State-of-the-Science Conference helped instigate the recent publication of The Bethesda System for Reporting Thyroid Cytopathology (TBS). AIMS AND OBJECTIVES: Terminology and therefore the probability of malignancy vary between pathologists and institutions. The purpose of this study was to evaluate a single institution's experience to determine if sub-classification (neoplasm versus lesion) aids in identifying patients at higher risk for malignancy. Also, an effort in regional and worldwide variation of significance of adoption of Bethesda classification has been evaluated. MATERIALS AND METHODS: From 2018 to 2021, all patients with thyroid lesions' were evaluated for FNAC correlation with the surgical diagnosis. During this period, 1763 thyroid FNACs were reported. Histopathological examination (HPE) outcome data was available in 444 (25.2%) cases. RESULTS: The cytologic-histologic diagnostic discrepancy rate was 11.5%. The sensitivity and specificity of thyroid FNA for the diagnosis of malignancy were 71.4% and 49.5%, respectively. In our study, the maximum cases were benign (84.5%). The malignancy risk for the different categories in our study, as seen by follow-up HPE, has corroborated well with the Bethesda System. CONCLUSION: The current results indicated that FNAC provides an accurate diagnosis of thyroid malignancy. The classification is directly related to the risk of malignancy in each category and this helps in accurate clinical management of that category.

Perinatal and Fetal Autopsies in Neuropathology: How I do it.

Fetal and perinatal autopsies are useful to identify the accurate cause of death and in the process recognize disorders which may require counselling for future pregnancies. Abnormalities of the CNS are an important cause of fetal loss and perinatal deaths. Most of these are structural abnormalities of the CNS, however a smaller portion show changes pertaining to prematurity, infections and even congenital tumors. In this review we evaluate CNS abnormalities of the fetus and the newborn as detected in autopsy series. We also describe our experience in a tertiary care hospital with a specialized neonatology unit over the last 8 years and discuss some of the newer methods like virtual autopsy.

Utility of cell block preparation in diagnosis of paediatric abdominal neoplasms.

BACKGROUND: Paediatric abdominal neoplasms are fairly common. Fine needle aspiration cytology (FNAC) is used for the initial evaluation of paediatric abdominal neoplasms. However, sometimes FNA interpretation can be difficult on limited material, owing to tumour heterogeneity and overlapping features. Therefore, we attempted to obtain additional information using cell block (CB) preparation from residual aspiration material along with immunohistochemistry (IHC) to enhance the diagnostic accuracy. AIM: To evaluate the correlation between CB preparation and FNAC in diagnosis of paediatric abdominal tumours with the help of an extended panel of IHC markers and to highlight some of the diagnostic difficulties. MATERIALS AND METHODS: A total of 113 cases of paediatric abdominal masses were studied. In addition to routine FNA smears, CBs were prepared from the residual material for IHC analysis as considered appropriate. RESULTS: This study included a total of 113 children with abdominal masses. Histopathology following surgical resection was available in 53 cases. Histology was taken as gold standard to measure the diagnostic accuracy with reference to sensitivity (Sn), specificity (Sp), positive predictive value and negative predictive value. The Sn of FNA alone was 87.5% and Sp was 97.78%. The Sn and Sp increased with use of CB alone and were 100% and 97.78% respectively. The highest Sn and Sp were observed when CB was combined with IHC where both the values were 100%. CONCLUSION: CB with IHC is a useful adjunct to the routine FNA smears that further contributes to enhance the accuracy of the cytopathological diagnosis and is useful for choosing pre-operative chemotherapeutic regimen.

Diagnostic utility of cytology smears and cell block in adrenal lesions.

BACKGROUND: Cytology of the adrenal gland is a less performed technique even in tertiary care centres; yet cytological evaluation is an important diagnostic tool for assessment of adrenal lesions. Our objective was to evaluate the diagnostic utility of FNAC smears and cellblock with immunohistochemistry (IHC) in lesions of the adrenal. MATERIAL AND METHODS: We had a total of 50 cases over a period of 2 years where both FNAC smears and cellblocks were taken. The smears and cellblocks were examined for adequacy. They were subsequently categorised into four groups: unsatisfactory, benign, suspicious of malignancy and malignant. The diagnostic accuracy of FNAC smears and cellblock with IHC were evaluated and compared, taking histopathology, wherever available, as the gold standard, RESULT: We had 50 cases with age ranging from 6 to 53 years with a median of 7.5 years. Of these, 54% were cytologically malignant and neuroblastoma was the commonest lesion. Histopathology was available in 23 cases only, where the diagnostic accuracy was evaluated. The sensitivity and specificity of FNAC smear was 100% and 85.71%, respectively whereas the sensitivity and specificity of cellblock with IHC was 100% and 92.86%, respectively. CONCLUSION: Cellblock together with IHC provides a higher degree of specificity, reduces the unsatisfactory rate and improves the diagnostic accuracy in lesions of the adrenal gland. Immunohistochemistry is an important adjunctive tool.

A study of spectrum of pulmonary pathology and expression of thyroid transcription factor-1 during neonatal period.

CONTEXT: Neonatal period is the single most hazardous period of life. The major causes of neonatal death are prematurity and respiratory distress syndrome. We report a series of neonatal autopsies in our Neonatal Intensive Care Unit with special emphasis on pulmonary pathology. The spectrum of pathological changes in the lungs and thyroid transcription factor-1 (TTF-1) expression was studied in detail with reference to its spatial distribution. AIMS: This study aims to analyze the causes of neonatal death with special attention to pulmonary pathology along with associated histopathological changes in lungs. We also evaluated the expression of TTF-1 at different levels of the airway. MATERIALS AND METHODS: After taking consent and anthropometric measurements, autopsy was performed. Weights of all organs were taken, and histological sections were examined under hematoxylin and eosin stain. TTF-1 immunostaining was done on lung sections. Localization of TTF-1 was evaluated at the intrapulmonary level of terminal bronchioles (TBs), distal bronchioles, and alveoli. RESULTS: We performed a series of 25 autopsies in neonates. In our series, most of the neonates were preterm (64%), had low birth weight (44%), and died within the first 7 days of life (80%). Majority (60%) of the neonates died due to pulmonary causes, followed by septicemia (24%), congenital anomalies (12%), and birth injury (4%). Among the respiratory causes, hyaline membrane disease (HMD) was diagnosed in maximum number of cases (32%), followed by pneumonia (12%) and pulmonary hemorrhage (12%). The TTF-1 expression in TBs, distal airways, and alveoli was significantly reduced or absent in cases of HMD compared to the control group. CONCLUSIONS: In this study, we observed that HMD is the most common cause of perinatal death among respiratory disorders, and in this disease, the expression of TTF-1 is significantly reduced in TBs, distal airways, and alveoli compared to the control group.

Cytological and histological correlation of granular cell tumor in a series of three cases.

Granular cell tumor (GCT) is an uncommon soft tissue tumor characterized by proliferation of cells with granular eosinophilic cytoplasm. We came across three such tumors, one in the tongue, one in the chest wall, and one in the right deltoid region, which were referred for fine-needle aspiration cytology. On cytological examination, the first two cases were diagnosed as GCT, and the mass in deltoid region was suggestive of proliferative myositis. The cytological details of these cases are discussed. The excision biopsies of the first two cases and Tru-cut biopsy of the deltoid mass confirmed the diagnosis of GCT.

Cytological diagnosis of adrenocortical carcinoma: A report of 2 cases in children.

Adrenocortical carcinoma (ACC) is a rare tumour, which sometimes affects pediatric age group. Fine needle aspiration cytology (FNAC) is a rarely performed technique in adrenal cortical tumours. There is sparse literature available describing the cytological findings of ACCs in children. Here we describe the cytological findings of 2 cases of ACCs in children. The first case describes the FNAC findings in a 4 year old girl who presented with a large abdominal mass. The second case narrates the intra-operative imprint cytology findings in a 2-year-old boy who came with precocious puberty. However, diagnosis of adrenocortical tumours based on cytology alone can be difficult and definitive diagnosis should be made after correlating cytological features with the clinical profile, radiology, histopathology, and immunohistochemistry.

Sirenomelia associated with discoid adrenal and lumbar meningocoele: An autopsy report.

Mermaid syndrome or Sirenomelia is a rare congenital deformity in which the legs are fused and bears resemblance to mermaid's tail. It carries a poor prognosis, due to associated urogenital and gastrointestinal abnormalities. An early antenatal diagnosis using Magnetic Resonance Imaging (MRI) can help in termination of pregnancy. Embryologically, it is considered as the extreme form of caudal regression syndrome due to the persistence of vitelline artery. Here, we report a case of Sirenomelia associated with bilateral renal agenesis along with the rare findings of discoid adrenal, lumbar meningocoele and abnormalities of the hand.

FNAC aided diagnosis of clear cell sarcoma of kidney: Report of two cases in infants.

Clear cell sarcoma of kidney (CCSK) is a rare aggressive malignant renal neoplasm with a high metastatic potential. Its outcome has however, improved with the advent of doxorubicin based neoadjuvant chemotherapy. Here, we present two cases of CCSK in infants diagnosed on cytology followed by nephrectomy. The first case presented in the neonatal period and had the unusual histological finding of islands of cartilage. The second case presented at the age of eight months. The possibility of CCSK should, therefore, be considered in the differential diagnoses of renal masses in infants as well as neonates. Diagn. Cytopathol. 2017;45:761-765. © 2017 Wiley Periodicals, Inc.

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.

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.

Sacrococcygeal teratoma with complete adrenal gland.

Sacrococcygeal teratoma is the most common congenital neoplasm in neonates. We came across a post term (42 weeks) newborn baby girl delivered by normal vaginal route. The baby presented with a large soft, cystic mass over the sacrococcygeal region. Radiological examination showed a soft tissue mass with variegated appearance. Complete excision of the mass was done. Histopathological examination revealed the mass to be a mature sacrococcygeal teratoma with the extremely uncommon finding of a complete adrenal gland within the teratoma sac. Sacrococcygeal teratoma is a component of a continuum with other tumors including fetiform teratoma, fetus in fetu, parasitic and conjoint twins.

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.

Central nervous system teratomas in infants: a report of two cases.

Intracranial teratomas are uncommon neoplasms with most of them being encountered in the pediatric age group. Teratomas are composed of derivatives of all the three germ cell layers and are classified into mature, immature and teratoma with malignant transformation. Two cases of intracranial teratomas in infants are presented here with the idea of highlighting this relatively uncommon condition in an uncommon age.

Expression of CDX-2 and Ki-67 in different grades of colorectal adenocarcinomas.

BACKGROUND: CDX2 is a caudal homeobox gene essential for intestinal differentiation and is specifically expressed in colorectal adenocarcinomas. Its role in colorectal carcinogenesis is not fully elucidated. AIMS AND OBJECTIVES: To study the expression pattern of CDX2 and Ki-67 in different grades of colorectal adenocarcinomas and to observe the relationship of their staining patterns in various tumor stages and to look for correlation if any, between Ki-67 labeling index (Ki-67 LI) and CDX2 expression. MATERIALS AND METHODS: A total of 74 cases were enrolled. Detailed clinical profile, peroperative findings, histological grading and staging were noted. Immunohistochemistry for CDX2 and Ki-67 was done, and Ki-67 LI was calculated. CDX2 staining was graded semi-quantitatively, and statistical analysis was done. RESULT: Age of presentation ranged from 20 to 75 years, and the male:female ratio was 1.83:1. There were 8, 47 and 13 cases of well, moderate and poorly differentiated adenocarcinomas, respectively. The mean Ki-67 LI of well, moderate and poorly differentiated adenocarcinomas were 14.25, 31.34 and 43.08 respectively, and their difference was statistically significant, correlation was also noted with stage. CDX2 expression appeared to be stronger in poorly differentiated cases, but there was no significant difference in its expression in the different grades and stages. There was no correlation between Ki-67 LI and CDX2 immunostaining pattern. The lymph node metastasis showed CDX2 positivity in all the cases. CONCLUSION: Expression of CDX2 does not significantly change with the grade of colorectal adenocarcinomas. However, it is an important diagnostic marker in metastatic colonic lesions. The Ki-67 LI, on the other hand, showed a strong correlation with histopathological grades.

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.