Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics?

Jamshid Abdul-Ghafar,Arsalan Ahmed,Nasir Uddin,Zubair Ahmad,Khurram Minhas

doi:10.1186/s13000-019-0790-8

Jamshid Abdul-Ghafar, Arsalan Ahmed + Show 3 more

Open Access

https://doi.org/10.1186/s13000-019-0790-8

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Abstract

BackgroundClear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric renal neoplasm with a heterogeneous histological appearance which often results in misdiagnosis. There are no specific immunohistochemical markers which can help in differentiating CCSK from other pediatric renal neoplasms. Recently Cyclin D1 has been investigated as a possible marker in this regard. In this study, we aim to determine the usefulness of Cyclin D1 in differentiating between CCSK and other pediatric renal neoplasms and to compare our results with those of recently published studies.MethodsA total of 48 cases of CCSK, Wilms tumor (WT), renal rhabdoid tumor, mesoblastic nephroma, renal Ewing sarcoma and neuroblastoma were included in the study. All cases were stained with cyclin D1. Extent of Cyclin D1 staining was graded according to percentage of positive tumor cells as diffuse (> 70%), focal (5 to 70%), and negative (< 5%). Intensity of Cyclin D1 staining was graded as strong or 3+, moderate or 2+ and weak or 1 + .ResultsMost or all cases of CCSK, neuroblastoma and renal Ewing sarcoma demonstrated diffuse and strong positivity for Cyclin D1. Most cases of Wilms tumor (epithelial component) also demonstrated diffuse and often strong positivity for Cyclin D1. In most cases of WT, blastemal component was negative.ConclusionsCyclin D1 is a sensitive but not specific immunohistochemical marker for CCSK and many other pediatric renal malignant neoplasms as well as for neuroblastoma. Hence, careful examination of histological features is important in reaching an accurate diagnosis in CCSKs. However, Cyclin D1 is very helpful in distinguishing between blastema-rich WT and CCSK.

Highlights

Clear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric renal neoplasm with a heterogeneous histological appearance which often results in misdiagnosis
Study cohort The surgical pathology files of the Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital and cases were submitted from French Medical Institute for Mothers and Children were searched for cases of CCSK, Wilms tumor (WT), renal rhabdoid tumor (RT), congenital mesoblastic nephroma, Ewing sarcoma of kidney as well as retroperitoneal neuroblastoma reported over a ten year period i.e. January 1, 2008 and December 31, 2017
All 4 cases of congenital mesoblastic nephroma (CMN) in our study showed markedly intense (3+) focal to diffuse staining for Cyclin D1

Summary

Introduction

Clear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric renal neoplasm with a heterogeneous histological appearance which often results in misdiagnosis. Clear cell sarcoma of kidney (CCSK) is an uncommon mesenchymal renal tumor of uncertain histogenesis which comprises approximately 3 to 4% of malignant pediatric renal neoplasms. It occurs in young children and mean age at diagnosis is approximately 36 months. It is more common in males (male to female ratio is 2:1), is centered in the renal medulla and is almost always unifocal.

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