Suprarenal solitary fibrous tumor associated with a NF1 gene mutation mimicking a kidney neoplasm: implications for surgical management

World Journal of Surgical Oncology

Case Report

Daniela Russo¹  Massimo Mascolo¹  Luigi Santini²  Claudio Mauriello²  Giovanni Conzo²  Ernesto Tartaglia²  Claudio Gambardella²  Daniela Esposito²  Gianfranca Stornaiuolo³  Giovan Battista Gaeta³ 

[1] Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131, Naples, Italy;Department of Anesthesiologic, Surgical and Emergency Science VII Division of General Surgery, Second University of Naples Italy, Via Pansini 5, 80131, Naples, Italy;Department of Infectious Diseases, Viral Hepatitis Unit, Second University of Naples, Via Pansini 5, 80131, Naples, Italy;

关键词: Solitary fibrous tumor;    Spindle cells;    Renal neoplasm;    Immunohistochemical assay;    Neurofibromatosis gene mutation;   

DOI  :  10.1186/1477-7819-12-87

 received in 2013-05-24, accepted in 2014-03-15,  发布年份 2014

来源: Springer

【 摘 要 】

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm, usually occurring in the pleura. Pararenal SFT, mimicking an adrenal gland or renal tumor, as here described, is extremely rare. We report a case of a right suprarenal SFT, incidentally discovered by abdominal ultrasound in a 54-year-old woman carrying a point neurofibromatosis 1 (NF1) gene mutation. Preoperative diagnostic work-up was ineffective in evaluating its origin, and an open radical right nephrectomy was therefore undertaken. Immunohistochemical assay showed a positivity for CD34, CD99 and Bcl-2, so suggesting a diagnosis of SFT. According to our knowledge, the association between this type of tumor and NF1 gene mutation has never been described. In cases of pararenal tumors, a more detailed preoperative diagnosis could be useful to better plan the extension of resection, allowing, in selected cases, nephron-sparing surgery. More studies are needed to better analyze the relationship between NF1 gene mutation and SFT.

【 授权许可】

CC BY   
© Conzo et al.; licensee BioMed Central Ltd. 2014