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

World Journal of Surgical Oncology

Luigi Santini²  Giovan Battista Gaeta¹  Gianfranca Stornaiuolo¹  Daniela Russo³  Massimo Mascolo³  Daniela Esposito²  Claudio Mauriello²  Claudio Gambardella²  Ernesto Tartaglia²  Giovanni Conzo² 

[1] Department of Infectious Diseases, Viral Hepatitis Unit, Second University of Naples, 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 Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy

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

Others  :  820006
DOI  :  10.1186/1477-7819-12-87

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

【 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】

   
2014 Conzo et al.; licensee BioMed Central Ltd.

【 图 表 】

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