Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm, usually occurring in the pleura. Pararenal SFT, mimicking adrenal glands or renal tumor, as here described, is extremely rare. We report a case of a suprarenal right SFT, incidentally discovered by abdominal ultrasound in a 54-year-old woman carrying a point neurofibromatosis 1 (NF) gene mutation. Preoperative diagnostic work-up was ineffective to evaluate 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 case of pararenal tumors, a more detailed preoperative diagnosis could be useful to better plan the extension of resection, allowing, in selected cases, a nephron-sparing surgery. More studies are needed to better analyze the relationship between NF1 gene mutation and SFT.

Suprarenal solitary fibrous tumor associated with a NF 1 gene mutation mimicking a kidney neoplasm. Implications for surgical management. World Journal of Surgical Oncology 2014, 12:87 1-5

CONZO, Giovanni;GAMBARDELLA, Claudio
2014

Abstract

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm, usually occurring in the pleura. Pararenal SFT, mimicking adrenal glands or renal tumor, as here described, is extremely rare. We report a case of a suprarenal right SFT, incidentally discovered by abdominal ultrasound in a 54-year-old woman carrying a point neurofibromatosis 1 (NF) gene mutation. Preoperative diagnostic work-up was ineffective to evaluate 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 case of pararenal tumors, a more detailed preoperative diagnosis could be useful to better plan the extension of resection, allowing, in selected cases, a nephron-sparing surgery. More studies are needed to better analyze the relationship between NF1 gene mutation and SFT.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11591/192709
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