Immunoglobulin A (IgA) dominant membranoproliferative glomerulonephritis (MPGN) is rare, described only as case reports. We report a rare case of an elderly man presenting with rapidly progressive renal failure and nephrotic range proteinuria with histological, immunofluorescence, and ultrastructural findings supporting a diagnosis of IgA dominant MPGN. Autoimmune disease, cryoglobulinemia and infection-associated glomerulonephritis were excluded. Remission was achieved within 3 months of treatment. This case highlights an uncommon diagnosis with a good response to therapy. The differential diagnosis of IgA nephropathy with MPGN-like pattern is discussed.
BACKGROUND Urine cytology when combined with cystoscopy remains a gold standard in screening and surveillance of urothelial carcinoma. Paris system for reporting urine cytology (PSRUC) gives seven well defined diagnostic criteria. We aimed to analyse utility of urine cytology in patients with urogenital symptoms, compare existing institutional system (EIS) with PSRUC and assess the performance of both reporting systems in predicting subsequent high-grade urothelial carcinoma on histopathology. METHODS A five year retrospective study included a total of 146 urine samples from 74 patients. Each case was assigned a category according to both EIS and PSRUC system. After cyto-histological correlation, sensitivity, specificity and diagnostic accuracy of urine cytology in detecting malignancy using PSRUC and EIS were determined. Performance of urine cytology in predicting subsequent high grade urothelial carcinoma (HGUC) was assessed for both reporting systems. RESULTS PSRUC resulted in reduction in number of cases assigned to atypical category (10.5 % vs. 3.4 %) and increase in low grade carcinomas assigned to NGUC category (66 % vs. 100 %). Positive predictive value (PPV) for predicting subsequent high grade urothelial carcinoma for HGUC and SHGUC category remained the same (100 %). Sensitivity (66.67 % vs. 55.5 %), specificity (100 % vs. 85.71 %) and diagnostic accuracy (81 % vs. 68.75 %) was improved with application of PSRUC when compared to EIS. Two cases of genitourinary tuberculosis were diagnosed. CONCLUSIONS PSRUC improves predictive accuracy of subsequent high-grade urothelial carcinoma on histopathology and it ensures uniformity in reporting. Judicious use of urine cytology might aid in early diagnosis of infectious conditions like tuberculosis. KEYWORDS PSRUC, Urine Cytology, High Grade Urothelial Carcinoma
A 28-year-old woman who is a resident of an endemic goitre region presented with anterior neck swelling of 2-year duration with rapid progression over preceding 6 months. The patient had associated voice change and dysphagia; however, there were no respiratory symptoms. She also had significant weight loss for the last 6 months. There was no history of radiation exposure or thyroid cancer in the family.On examination, a large, firm, smooth surfaced swelling was palpable in the region of thyroid (Fig. 1). Trachea was pushed anteriorly and was felt throughout its course suggesting retrotracheal nature. Fine needle aspiration cytology (FNAC) from the swelling was reported as poorly differentiated/medullary carcinoma thyroid. However, serum calcitonin was within normal limits. Neck X-ray showed retrotracheal widening (Fig. 2a) and neck contrast-enhanced computed tomography revealed a heterogeneously enhancing solid cystic lesion 12.5 × 9.5 cm with peripheral calcification in retropharyngeal space presumably arising from the left lobe of thyroid.With the diagnosis of thyroid carcinoma with retrotracheal extension, patient underwent total thyroidectomy with excision of retrotracheal tumour. Intra-operatively both lobes of thyroids appeared normal and the left lobe was connected with few fibrous bands to a large well-capsulated mass in retrotracheal location that had minimal adhesion to oesophagus and pharyngeal wall without any evidence of tumour metastasis into lateral neck nodes (Fig. 2b). Patient made an uneventful recovery. Final histopathology was suggestive of synovial sarcoma showing immunostaining for cytokeratin, bcl-2 and vimentin (Fig. 3). In view of histopathology, patient received adjuvant radiotherapy and is free of disease.Head and neck synovial sarcoma is an extremely rare malignancy representing approximately 0.1% of all head and neck cancers. 1 These tumours arise from pluripotent mesenchymal cells with no definitive relationship to articular surfaces, and are named as such for their appearance and not of their origin. 2 The histopathological diagnosis of synovial cell sarcoma is challenging as a result of the variable cellular and architectural morpholFig. 1. Neck swelling with tattooing.Fig. 2. (a) Lateral X-ray neck showing retrotracheal widening. (b) Intraoperative photograph showing relation of mass with adjacent structures.
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