Suprarenal solitary fibrous tumor associated with a NF1 gene mutation mimicking a kidney neoplasm: implications for surgical management.
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.
Department of Anesthesiologic, Surgical and Emergency Science VII Division of General Surgery, Second University of Naples Italy, Via Pansini 5, 80131 Naples, Italy. giovanni.conzo@unina2.it.
Giovanni
G
Conzo
Ernesto
E
Tartaglia
Claudio
C
Gambardella
Claudio
C
Mauriello
Daniela
D
Esposito
Massimo
M
Mascolo
Daniela
D
Russo
Gianfranca
G
Stornaiuolo
Giovan Battista
GB
Gaeta
Luigi
L
Santini
D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D009392:Nephrectomy; D025542:Neurofibromin 1 / Q000235:genetics*; D011379:Prognosis; D054364:Solitary Fibrous Tumors / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery
D025542:Neurofibromin 1