RENAL SCLEROSING PERITUBULAR NODULES IN RENAL ALLOGRAFT RECIPIENT S. Şen 1 , B. Sarsık 1 , Ö Güngör 2 , D. Demir 1 , H. Töz 2 Departments of Pathology 1 , Nephrology 2 , Ege University, School of Medicine, İzmir, Turkey Objectives: A unfamiliar type of renal lesion characterized by the formation of multiple hamartomatous sclerosing peritubular nodules in the cortex was described by Mandbur and Weiss in 1981. The nodules originated as concentric peritubular proliferations of collagen producing spindle cells. The lesion was called renal sclerosing peritubular nodules (RSPN). To the best of our knowledge, our case represents the second case of RSPN in renal allograft recipients. İdentical native renal lesions associated a hereditary renal abnormality in von Recklinghausen disease and neurofibromatosis. Case: A 41-year-old woman underwent cadaveric (age 42) renal transplantation in 2009 for treatment of renal failure secondary to unknown etiology. After an uncomplicated one year posttransplant course, protocol allograft biopsy was performed. Allograft biopsy showed scattered round, elliptical, and fusiform nodules randomly distributed in the cortex. The glomeruli were essentially unremarkable except global sclerosis (%25). There was interstitial fibrosis and tubular atrophy associated with arteriolar intimal hyalinosis. The lesions showed layers of linear staining with Jones methenamine silver and PAS. Trichrome stain showed a smooth muscle staining. Immunohistochemically vimentin, smooth muscle actin and desmin stains were diffusely positive in the spindle cells in the nodules, whereas S100, pancytokeratin (AE1/AE3) and CD31 stain was negative. Including implantation biopsy, previous renal allograft biopsies were reevaluated and similar smaller nodular lesions were observed in the second allograft biopsy. After the one year follow up these nodules do not alter kidney function. Conclusions: We described first renal allograft RSPN cases, although similar case was discussed in nephpath on 2007 by Riopel. They found that their donor had a well established diagnosis of neurofibromatosis. Our findings might be a progression of donor disease.

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Page 1: RENAL SCLEROSING PERITUBULAR NODULES IN RENAL ... · Web viewİdentical native renal lesions associated a hereditary renal abnormality in von Recklinghausen disease and neurofibromatosis

RENAL SCLEROSING PERITUBULAR NODULES IN RENAL ALLOGRAFT RECIPIENT

S. Şen1, B. Sarsık1, Ö Güngör2, D. Demir1, H. Töz2

Departments of Pathology1, Nephrology2, Ege University, School of Medicine, İzmir, Turkey

Objectives: A unfamiliar type of renal lesion characterized by the formation of multiple hamartomatous sclerosing peritubular nodules in the cortex was described by Mandbur and Weiss in 1981. The nodules originated as concentric peritubular proliferations of collagen producing spindle cells. The lesion was called renal sclerosing peritubular nodules (RSPN). To the best of our knowledge, our case represents the second case of RSPN in renal allograft recipients. İdentical native renal lesions associated a hereditary renal abnormality in von Recklinghausen disease and neurofibromatosis.

Case: A 41-year-old woman underwent cadaveric (age 42) renal transplantation in 2009 for treatment of renal failure secondary to unknown etiology. After an uncomplicated one year posttransplant course, protocol allograft biopsy was performed.

Allograft biopsy showed scattered round, elliptical, and fusiform nodules randomly distributed in the cortex. The glomeruli were essentially unremarkable except global sclerosis (%25). There was interstitial fibrosis and tubular atrophy associated with arteriolar intimal hyalinosis. The lesions showed layers of linear staining with Jones methenamine silver and PAS. Trichrome stain showed a smooth muscle staining. Immunohistochemically vimentin, smooth muscle actin and desmin stains were diffusely positive in the spindle cells in the nodules, whereas S100, pancytokeratin (AE1/AE3) and CD31 stain was negative.

Including implantation biopsy, previous renal allograft biopsies were reevaluated and similar smaller nodular lesions were observed in the second allograft biopsy. After the one year follow up these nodules do not alter kidney function.

Conclusions: We described first renal allograft RSPN cases, although similar case was discussed in nephpath on 2007 by Riopel. They found that their donor had a well established diagnosis of neurofibromatosis. Our findings might be a progression of donor disease.