Soft tissue perineurioma involving the kidney: a report of two cases with an emphasis on differential diagnosis

Abstract Background Soft tissue perineurioma of the kidney is rare, with only a few reported cases. We report two additional cases with histologic, immunohistochemical and genetic analyses. Case presentation Both tumors were from adults (1 female aged 49 years and 1 male aged 42 years) and grossly h...

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Main Authors: Tian-shi Ma (Author), Ling Zhou (Author), Quan Zhou (Author), Xiang-lei He (Author), Ming Zhao (Author)
Format: Book
Published: BMC, 2021-09-01T00:00:00Z.
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042 |a dc 
100 1 0 |a Tian-shi Ma  |e author 
700 1 0 |a Ling Zhou  |e author 
700 1 0 |a Quan Zhou  |e author 
700 1 0 |a Xiang-lei He  |e author 
700 1 0 |a Ming Zhao  |e author 
245 0 0 |a Soft tissue perineurioma involving the kidney: a report of two cases with an emphasis on differential diagnosis 
260 |b BMC,   |c 2021-09-01T00:00:00Z. 
500 |a 10.1186/s13000-021-01149-5 
500 |a 1746-1596 
520 |a Abstract Background Soft tissue perineurioma of the kidney is rare, with only a few reported cases. We report two additional cases with histologic, immunohistochemical and genetic analyses. Case presentation Both tumors were from adults (1 female aged 49 years and 1 male aged 42 years) and grossly had maximum diameters of 6.5 and 10 cm, respectively. The tumors were overall well circumscribed but unencapsulated, with focally entrapped benign native renal tubules in one case; both tumors seemed to arise in the capsular areas. The tumors had histologic and immunohistochemical profiles consistent with soft tissue perineurioma. Fluorescence in situ hybridization analyses demonstrated that the tumors were negative for amplification of MDM2 and rearrangements of ESWR1, FUS, and KMT2A. Targeted next-generation sequencing revealed a low tumor mutation burden and likely pathogenic mutations (CYP2B6 and FLT1 mutations for 1 each). Follow-up data were available for both patients; neither had tumor recurrence or metastasis. Conclusions In conclusion, renal perineurioma is rare, usually arises in the capsular areas, and is cured by resection. Low-grade dedifferentiated liposarcoma and low-grade fibromyxoid sarcoma as well as other spindle cell lesions should be considered in the differential diagnosis. 
546 |a EN 
690 |a Soft tissue perineurioma 
690 |a kidney 
690 |a molecular genetics 
690 |a dedifferentiated liposarcoma 
690 |a low-grade fibromyxoid sarcoma 
690 |a Pathology 
690 |a RB1-214 
655 7 |a article  |2 local 
786 0 |n Diagnostic Pathology, Vol 16, Iss 1, Pp 1-8 (2021) 
787 0 |n https://doi.org/10.1186/s13000-021-01149-5 
787 0 |n https://doaj.org/toc/1746-1596 
856 4 1 |u https://doaj.org/article/9b2c856957a8468f801b9c8dc6d25bca  |z Connect to this object online.