Renal epithelial and stromal tumor with a multiple cystic lesion localized in the upper portion of the right kidney

Daisuke Ikuma,Junichi Hoshino,Naoki Sawa,Yoji Nagashima,Keiichi Kinowaki,Kenichi Ohashi,Masato Sawamura,Akinari Sekine,Tatsuya Suwabe,Hiroki Mizuno,Yoshifumi Ubara,Kei Kono,Masayuki Yamanouchi

doi:10.1007/s13730-020-00548-9

Daisuke Ikuma, Junichi Hoshino + Show 11 more

Open Access

https://doi.org/10.1007/s13730-020-00548-9

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Abstract

A 60-year-old Japanese woman was admitted because of the polycystic mass with right flank pain localized in the upper portion of the right kidney. Right nephrectomy was performed because the mass lesion had continuously increased in size over the past 10 years. A surgical specimen showed histology consistent with a mixed epithelial and stromal tumor, which is closely related to multilocular cystic nephroma, and was diagnosed by a defined capsule between the cystic mass lesion and normal renal tissue by CT and MRI, and histology. Localized renal cystic disease that does not have a capsule was excluded from differential diagnosis.

Highlights

Localized renal cystic disease (LRCD) has been reported; cysts form in only one kidney and not in other organs, and patients do not have a family history of cystic disease, unlike autosomal dominant polycystic kidney disease (ADPKD)
We encountered a case of multilocular cystic nephroma (MCN)/mixed epithelial and stromal tumor (MEST)/renal epithelial and stromal tumor (REST) but not LRCD
Five patients presented with hematuria, four with flank pain, and one with a palpable abdominal mass; the disease was an incidental finding in eight patients

Summary

Introduction

Localized renal cystic disease (LRCD) has been reported; cysts form in only one kidney and not in other organs, and patients do not have a family history of cystic disease, unlike autosomal dominant polycystic kidney disease (ADPKD). We encountered a polycystic mass only in the upper portion of the right kidney in a 60-year-old female patient, and URCD/LRCD has been considered first diagnosis, but localized multilocular cystic nephroma (MCN) became definitive diagnosis. Macroscopic examination of the resected kidney showed a polycystic mass measuring 17 × 15 × 12 cm (Fig. 2a), and microscopic examination revealed a fibrous capsule forming a border between the lesion and the normal renal tissue (Fig. 2a). We decided that the most reliable diagnosis in our patient was MCN/MEST/ renal epithelial and stromal tumor (REST) but not LRCD. The patient still has good renal function, with a creatinine level of 1.53 mg/dL and eGFR of 27.8 ml/min/1.73 m3

Discussion

Findings

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