Clinicopathological, Immunological, and Laboratory Parameters of Childhood Lupus Nephritis: A Study from Northeast India.

Background Lupus nephrtis in children is associated with high morbidity and mortality. The incidence of childhood systemic lupus erythematosus (SLE) ranges from 3.3 to 8.8/100000 children with a higher Asian preponderance. The predominance of SLE in female pediatric patients increases gradually with age to the values observed in adults. Objectives To assess the clinical, immunological, and histopathological spectrum of childhood lupus nephritis in northeast India and explore the relationship between clinical, biochemical, serological, and histopathological findings. Materials and Methods A retrospective descriptive study was performed over 8 years. Histopathology slides were reviewed by two pathologists, whereas other details were collected from patients' records. Statistical Analysis Statistical analysis was based on the chi-square test and a p -value < 0.05 was considered statistically significant. Results Fifty-three cases of lupus nephritis were included in the study. The patients' age ranged from 5 to 18 years with a mean age of 14.5 years and a female: male ratio of 6.5:1. Edema and hypertension were the commonest clinical presentations, whereas proteinuria was the commonest presenting laboratory parameter. Amongst all the immunological markers, dsDNA was the commonest. Histopathologically, predominantly study population belonged to class IV lupus nephritis. The patients with class IV showed a statistically significant correlation with proteinuria and hematuria at the time of diagnosis. Immunological markers, namely, ANA and anti-ds-DNA positivity were significantly associated with advanced renal histopathology. Conclusion cSLE in northeast India presents mostly as Class IV LN presenting mostly with deranged laboratory parameters and preponderance of various immunological markers and clinical presentations.

Study of Programmed Death Ligand 1 and EGFR/HER2 Expression in Non-Small-Cell Lung Carcinoma With a Clinicopathological Spectrum.

Non-small-cell lung carcinoma (NSCLC) is a disease characterized by the upregulation of programmed death ligand 1 (PD-L1) along with alterations in epidermal growth factor receptor (EGFR) and HER2-neu (HER2) amplification in addition to EGFR mutation. In the present study, the expression of PD-L1 and EGFR and HER2-neu in NSCLC was studied and their expression in relation to various clinicopathological parameters was analysed. We studied 49 core biopsy specimens of NSCLC for PD-L1, EGFR and HER2-neu expressions using immunohistochemistry. Scoring was based on the intensity and percentage of tumour cells expressing the immunomarkers. PD-L1, EGFR and HER2-neu expression was seen in 20.4%, 32.7% and 14.2% of NSCLC, respectively. The analysis showed no significant difference in PD-L1 expression in relation to any clinicopathological parameters. Low or negative EGFR expression was significantly associated with positive lymph node status (P=0.04). HER2-neu expression showed a significant difference in relation to tumour histology (adenocarcinoma; P=0.01). Also, there was no difference noted with PD-L1 expression in relation to EGFR and HER2-neu expression. As our study has a small number of cases, the validation of the predictive and prognostic value of these markers in lung cancer patients requires further studies.

Tumour Behaviour of Low-Grade Papillary Urothelial Carcinoma: A Single-Centre Retrospective Study.

Background and objective Carcinoma of the urinary bladder is the most common urological cancer, and it accounts for 3.9% of all cancer cases in men. Patients with the subset of noninvasive low-grade papillary urothelial carcinoma (LG-UrCa) are at higher risk for tumour recurrence. In this study, we aimed to analyse the histopathological features of LG-UrCa and to correlate those with recurrence potential as well as disease stage and grade progression. Materials and methods We conducted a retrospective study from January 2016 to December 2018. All cases with presenting biopsy initially reported as LG-UrCa were included in the study. All cases with initial biopsy reported as high-grade papillary urothelial carcinoma (HG-UrCa) were excluded from the study. We used the 2016 World Health Organization/International Society of Urological Pathology (WHO/ISUP) guidelines for the classification of papillary urothelial neoplasm. Results A total of 48 initially diagnosed cases of LG-UrCa were identified. Two out of 48 cases were reclassified as high-grade urothelial carcinoma and were excluded from the study. The mean age of patients at presentation was 56.7 years. The mean duration of follow-up was 19.8 months. The mean size of initial tumours was 3.4 cm. Tumour recurrence was encountered in 14 (30.4%) of 46 patients. Out of the four patients who had high-grade progression (8.7%), two also developed TNM stage progression. These two patients eventually underwent radical cystectomy. Patients with larger initial tumour sizes were found to have an increased tumour recurrence rate (p=0.009). Patients with multiple lesions at initial diagnosis had a significantly higher tumour recurrence rate than those with a single tumour (p=0.02). There was no significant difference with regard to intravesical Bacillus Calmette-Guérin (BCG) and tumour recurrence (p=0.065). None of the clinicopathological parameters were significantly associated with the grade and/or stage progression. Conclusion Based on our findings, patients with larger initial tumour size and tumour multiplicity at presentation had an increased tumour recurrence rate.

IgA nephropathy superimposed on diabetic nephropathy: A case report with review of literature in eight Indian studies.

Diabetes mellitus is one of the foremost diseases in the modern era and diabetic nephropathy contributes to a major percentage of end-stage kidney disease. Although diabetic nephropathy is difficult to revert back, detection of nondiabetic renal disease (NDRD) is important to detect as most of them are treatable. We describe a case of a 62-year-old female with long-standing diabetes mellitus presenting with massive proteinuria and clinical features of nephrotic syndrome. The kidney biopsy showed class III diabetic nephropathy along with focal proliferative glomerulonephritis with crescent formations. The immunofluorescence study showed strong mesangial IgA deposition with the predominance of lambda stain. This confirmed the diagnosis of diabetic nephropathy with superimposed IgA nephropathy. We reviewed eight Indian studies herewith to demonstrate NDRD spectrum in the Indian diabetic scenario.

Mélange of Lymphoepithelial Lesions of Salivary Glands from a Tertiary Care Center of North East India: Diagnostic Conundrums.

Background Lymphocytic infiltrates of the major salivary glands are involved in a spectrum of diseases that range from reactive to benign and malignant neoplasms. Occasionally, these pathologic entities present difficulties in the clinical and pathological diagnosis. Aim and Objective The aim of this study was to highlight the importance of meticulous cytopathological and histopathological examination (HPE) in solving the diagnostic challenges encountered in the analysis of these salivary gland lesions. Materials and Methods A retrospective analysis of salivary gland lesions was undertaken over a period of 5 years from 2013 to 2018 in the Department of Pathology at our institute. Salivary gland pathologies diagnosed either as chronic sialadenitis or reactive/benign/malignant lymphoepithelial lesions on fine-needle aspiration cytology (FNAC) and as lymphoepithelial carcinoma (LEC) were included in this study. Results A total of 86 cases of salivary gland lesions diagnosed as mentioned above were found during this period. Out of the 86 cases, 16 were subjected to HPE. Biopsy was not warranted in most of the cases diagnosed as chronic sialadenitis. HPE was concordant with the FNAC diagnoses in 13 out of the 16 cases (81.3%), with a single case misinterpreted as LEC on FNAC. Conclusion Benign and malignant lymphoepithelial lesions of salivary glands may sometimes be difficult to differentiate not only from one another on FNAC but also from other malignant lesions. FNAC is an effective tool for the diagnosis of nonneoplastic lesions, but in cases of benign lymphoepithelial lesions in the absence of salivary acini, biopsy is advisable.

Metanephric Adenoma with cystic changes- An uncommon presentation of a rare tumor in a young adult.

Metanephric adenoma (MA) is a rare benign neoplasm of the kidney that is usually asymptomatic and incidentally diagnosed. MA usually present as a solid mass; however, a cystic presentation has been reported. The main differential diagnosis of MA is the epithelial predominant Wilms tumor (e-WT) and the solid variant of papillary renal cell carcinoma (pRCC). The presence of the BRAF gene mutation has recently been reported in 85% of MA, and less than 10% of cases of MA do not express this specific gene mutation. Herein we report a 22-year-old man who presented with back pain and abdominal discomfort with a renal mass on the computed tomographic scan. The diagnosis of metanephric adenoma was confirmed histopathologically. In our case, the tumor presented as a solid and cystic mass hence mimicking a papillary renal cell carcinoma. The VE1 protein, which correlates with BRAF gene mutation, did not show any significant expression. We want to highlight that MA can present as a cystic lesion that should be taken into account to avoid unnecessary radical nephrectomy. Also, we demonstrated that a subset of MA might not harbor the BRAF gene and, they are classified as the BRAF wild type MA.

Angiomyolipoma of the broad ligament.

Angiomyolipoma is a benign mesenchymal neoplasm of the renal parenchyma, accounting for 1% of all renal parenchymal tumors. However, this entity may rarely occur in extrarenal sites. Extrarenal angiomyolipoma has been documented in various sites of the body, but angiomyolipoma of the broad ligament was reported in only two cases. We report the reputed third case of angiomyolipoma of the broad ligament in a 33-year-old female, who presented clinically with abdominal distension. With a working diagnosis of low-grade neoplasm, an en-bloc excision of the left broad ligament mass was performed. Based on histopathology and immunohistochemistry, a diagnosis of the classical variant of angiomyolipoma of the left broad ligament was made. The post-operative period was uneventful with no recurrence after 6 months of follow-up.

A Retrospective Study of Placenta Accreta, Percreta, and Increta in Peripartum Hysterectomies in a Tertiary Care Institute in Northeast India.

BACKGROUND: Abnormal placentation such as placenta accreta, increta, and percreta are frequent causes of post-partum hemorrhage, which results in maternal morbidity and mortality. A previous history of cesarean section, placenta previa, and pre-eclampsia are the important risk factors for abnormal placentation. A reliable antenatal diagnosis and planned surgical approach can reduce the incidence of maternal morbidity and mortality from massive hemorrhage. AIM: To study the incidence of abnormal placentation and the association of various risk factors with abnormal placentation. MATERIAL AND METHODS: A retrospective study over a period of eight years in patients with peripartum hysterectomies due to abnormal placentation presenting with massive hemorrhage. RESULTS:  We received a total of 10 emergency hysterectomy specimens during an eight­year period. Of the cases, placenta accreta accounted for 40% (4/10), increta up to 40% (4/10), and percreta 20% (2/10). Analysis of these findings with parity showed 20% of the women were uniparous (2/10), and 80% were multiparous (8/10). Risk factor analysis showed previous cesarean sections in 40% (4/10), placenta previa in 50% (5/10), and pre­eclampsia in 10% (1/10). CONCLUSION: The present study highlights the incidence of abnormal placentation in a tertiary care institute in Northeast India. Placenta accreta and increta constituted the major forms of abnormal placentation. Multiparous women with placenta previa followed by previous lower segment cesarean section were more at risk of having abnormal placentation. These findings will guide in antenatal care by risk prioritization and management planning of these patients.

Fine-needle aspiration cytology of mediastinal masses: An institutional experience.

INTRODUCTION: Mediastinal masses are uncommon in clinical practice. Fine-needle aspiration cytology (FNAC) is an important and useful investigation and is considered next to imaging in the diagnosis of mediastinal lesions. AIM: To analyze the mediastinal masses diagnosed on FNAC. MATERIALS AND METHODS: We retrospectively retrieved twenty-five cases, which underwent FNAC under ultrasound or computed tomography (CT) guidance for mediastinal masses, over a period of 4 years. Histopathological correlations were done in cases wherever available. RESULTS: Among twenty-five cases, which were diagnosed through FNAC, eighteen were males and seven were females with age ranging from 6 to 85 years. Ultrasonography (USG)-guided FNAC was performed in three patients, while CT-guided FNAC was performed in twenty-two patients. Out of twenty-five cases, seventeen cases were malignant, six were benign, and two cases were inadequate. FNAC was useful in the diagnosis of 83.3% of cases. Biopsy and/or cell block correlations were available in fourteen cases. In the malignant category, mediastinal invasion by either squamous cell carcinoma or adenocarcinoma constituted the highest number with eight (47%) out of seventeen cases. Among the nonneoplastic conditions, nonspecific inflammation was the most common cause with two cases (8%) out of total cases followed by one case each of tuberculosis, schwannoma, thymoma, and cystic lesion. CONCLUSION: USG or CT-guided FNAC is a safe, minimally invasive, and cost-effective procedure, which can provide a precise diagnosis in the mediastinal masses, and may obviate the need for an invasive surgical approach.

Metanephric Adenoma with cystic changes- An uncommon presentation of a rare tumor in a young adult

Metanephric adenoma (MA) is a rare benign neoplasm of the kidney that is usually asymptomatic and incidentally diagnosed. MA usually present as a solid mass; however, a cystic presentation has been reported. The main differential diagnosis of MA is the epithelial predominant Wilms tumor (e-WT) and the solid variant of papillary renal cell carcinoma (pRCC). The presence of the BRAF gene mutation has recently been reported in 85% of MA, and less than 10% of cases of MA do not express this specific gene mutation. Herein we report a 22-year-old man who presented with back pain and abdominal discomfort with a renal mass on the computed tomographic scan. The diagnosis of metanephric adenoma was confirmed histopathologically. In our case, the tumor presented as a solid and cystic mass hence mimicking a papillary renal cell carcinoma. The VE1 protein, which correlates with BRAF gene mutation, did not show any significant expression. We want to highlight that MA can present as a cystic lesion that should be taken into account to avoid unnecessary radical nephrectomy. Also, we demonstrated that a subset of MA might not harbor the BRAF gene and, they are classified as the BRAF wild type MA.

Angiomyolipoma of the broad ligament

Angiomyolipoma is a benign mesenchymal neoplasm of the renal parenchyma, accounting for 1% of all renal parenchymal tumors. However, this entity may rarely occur in extrarenal sites. Extrarenal angiomyolipoma has been documented in various sites of the body, but angiomyolipoma of the broad ligament was reported in only two cases. We report the reputed third case of angiomyolipoma of the broad ligament in a 33-year-old female, who presented clinically with abdominal distension. With a working diagnosis of low-grade neoplasm, an en-bloc excision of the left broad ligament mass was performed. Based on histopathology and immunohistochemistry, a diagnosis of the classical variant of angiomyolipoma of the left broad ligament was made. The post-operative period was uneventful with no recurrence after 6 months of follow-up.

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group.

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Relooking the curriculum and assessment in undergraduate pathology.

INTRODUCTION: Pathology is basic science, and steps are being taken to integrate the clinical sciences in undergraduate pathology curriculum. The present study was undertaken with the aim to assess the need for revision of the undergraduate pathology curriculum with a focus on assessment methods. MATERIALS AND METHODS: This study included a questionnaire-based, anonymous, survey in a 5-point Likert-type scale for undergraduate students who have completed pathology subject including the interns and a qualitative approach through interviews and focus group discussions from the pathology senior residents and faculties using the essentialist thematic analysis. RESULTS: There were a total of 109 feedbacks from undergraduate students and also the interns. A total of 10 feedbacks from the senior residents and faculties were analyzed. About 70%-90% of the students were satisfied with the pathology curriculum and teaching and clinical integration in pathology. However, only 52.3% of students felt morphology being extremely important as compared to 80% of faculties, showing a discrepancy between the students' perception of the importance of morphology and that of the faculties. CONCLUSION: Although both students and faculties find the curriculum adequate, it is suggested that teaching could be made more clinical oriented. The assessment based on morphology should be given less emphasis during the assessment.

Castleman's disease associated with calcifying fibrous tumor: A rare association with review of literature.

Castleman's disease is a rare disease characterized by unicentric or multicentric enlargement of the lymph node, and it is mostly of the hyaline vascular type. Calcifying fibrous tumor is a neoplasm characterized by large areas of hyalinized collagen with paucicellular areas along with dystrophic calcification and scattered lymphoplasmacytic infiltrates. There are only five case reports to describe an association between these two entities. We report a case of a 38-year-old male with seropositivity for hepatitis B virus presenting with a right-sided cervical swelling. Fine-needle aspiration cytology (FNAC) was done, and a diagnosis of follicular hyperplasia was offered. Biopsy confirmed the diagnosis of Castleman's disease. However, there was associated calcifying fibrous tumor in the lymph node induced by trauma due to FNAC.

Schwannoma of the uterine cervix: a rare case report.

Schwannomas are neurogenic tumors, which are among the most varied tumors with respect to morphology, clinical associations, and presentations; they occur in a wide variety of sites. The uterine cervix is a rare site of occurrence and only 15 cases of schwannomas of the uterine cervix, including 5 benign and 10 malignant cases, have been reported to date. Thus, schwannomas of the uterine cervix may pose diagnostic difficulty. Here, we report a case of benign schwannoma of the uterine cervix in a 37-year-old female, who presented with vaginal spotting.

Primary Extraskeletal Osteosarcoma Arising from the Optic Nerve: A Rare Case Report.

Orbital location of extraskeletal osteosarcoma is extremely rare with only 4 cases reported so far in the English literature. We present the case of a 32-year-old female who presented with proptosis and complete loss of vision of the left eye. Contrast-enhanced computed tomography scan showed a densely calcified lobulated lesion in the left optic nerve showing strong enhancement. A left fronto-occipito-zygomatic osteotomy was conducted and a greyish brown tumor was identified. Histopathological and immunohistochemical examination of the curetted material revealed it to be extraskeletal osteosarcoma. A left partial maxillectomy with ethmoidectomy and left orbital exenteration was done and the patient was advised chemotherapy with radiotherapy.

Lower urinary tract obstruction in male children - a report of three cases.

Urinary retention in young children and infancy is relatively rare. The commonest malignancy cause of bladder outlet obstruction in infancy is prostatic rhabdomyosarcoma; the commonest cause of congenital bladder outlet obstruction in a male infantis posterior urethral valve and the commonest cause of urethral obstruction in male children is urethral calculi. We present here a report on three cases of urethral obstruction in male children.

Acute pancreatitis due to ascariasis: a sonographic pictorial essay on four cases.

Ascariasis is the commonest helminthic disease to infect humans. Due to their wandering nature, the roundworms from the second part of the duodenum migrate through the biliary opening into the hepatobiliary and pancreatic ducts. Ascariasis is the most common parasitic cause of pancreatitis in endemic region. Pancreatitis can result due to pancreatic ascariasis, biliary ascariasis or both. Pancreatitis due to ascariasis can be severe and life-threatening. We present a pictorial essay of acute pancreatitis due to ascariasis on four cases.