RESUMO
Background: Lipoprotein glomerulopathy is an infrequent glomerular disorder that culminates in nephrotic syndrome and often progresses to kidney failure. Whereas most patients have been reported in Japan and China, limited reports have been documented outside these regions. This patient represents the first report of lipoprotein glomerulopathy in Pakistan. Case Presentation: A 25-year-old male patient, hypertensive for 2 years, presented with progressive body edema, frothy urine, and fatigue. Examination revealed elevated blood pressure, bilateral pedal edema, and positive shifting dullness. Laboratory results showed significant proteinuria and elevated cholesterol and triglyceride levels. Renal biopsy revealed enlarged glomeruli with a dilated capillary lumen filled with pale-staining mesh-like material "lipoprotein thrombi." Mild tubular atrophy and interstitial inflammation were observed. No interstitial fibrosis was evident. Electron microscopy detailed the lipoprotein thrombi with lipid granules and vacuoles of various sizes. A diagnosis of lipoprotein glomerulopathy was rendered. Treatment with fenofibrate, rosuvastatin, and captopril led to notable improvements in symptoms, blood pressure, and lipid levels during a 6-month follow-up. Subsequent biopsy showed complete resolution of the lipoprotein thrombi and a significant reduction in subendothelial granular densities. However, the flocculent subendothelial material persisted to some extent despite the complete resolution of lipoprotein thrombi. Conclusion: This report underscores the rarity of lipoprotein glomerulopathy in Pakistan and contributes valuable insights into its histopathologic features and global epidemiology. This unique instance aims to raise awareness among healthcare professionals, aiding in improved recognition of this rare entity. The favorable response to fenofibrate treatment underscores its effectiveness in managing lipoprotein glomerulopathy.
RESUMO
Primary leiomyosarcoma is a rare malignant kidney tumor. The diagnosis of this disease is usually made on the basis of histological examination because it lacks specific clinical or radiological characteristics. Differentiation between leiomyosarcoma and sarcomatoid renal cell carcinoma can be challenging because spindle cell morphology is observed in both tumors. Therefore, caution should be exercised when making a diagnosis of primary renal leiomyosarcoma. Both renal sarcoma and sarcomatoid renal cell carcinoma have a worse prognosis, and nephrectomy is the treatment of choice in locally resectable tumors. An example of such a tumor is discussed in relation to its diagnostic challenges. We report a case of a 35-year-old female who presented with a left renal mass. A left radical nephrectomy was performed, and a firm, tan-white, lobulated tumor (14x8x7.5 cm) was present on gross examination. A histological diagnosis of high-grade leiomyosarcoma was made on the basis of histology, positivity for caldesmon and desmin, and negative cytokeratin immunostaining. Sarcomatoid renal cell carcinoma was ruled out based on morphological findings after extensive sampling of the tumor along with negativity for CK, CD-10, and carbonic anhydrase IX immunostaining.
