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Letter to the Editor
Solitary fibrous tumor of the renal hilum: A rare a diagnosis for a not so rare clinical picture
Tumor fibroso solitario del hilio renal: un diagnóstico poco común de un cuadro clínico no tan raro
J.F. Rodríguez-Morenoa, E. Condeb, I. Durana,c,
Corresponding author
a División de Oncología Médica, Centro Integral Oncológico Clara Campal, Madrid, Spain
b Laboratorio de Dianas Terapéuticas, Hospital Universitario San Chinarro, Madrid, Spain
c Facultad de Medicina, Universidad San Pablo CEU, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Imaging studies such as computed tomography &#40;CT&#41; or ultrasound are becoming more and more frequent in our routine clinical practice&#46; Often these techniques identified masses in the kidney but cannot always distinguish between renal cell carcinomas&#44; metastases&#44; carcinomas of the upper urinary tract&#44; or benign renal tumors&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Surgical resection&#44; which provides tissue for accurate diagnosis as well as definitive therapy&#44; remains the standard approach for most patients who present with renal masses&#46; However&#44; a common clinical problem is the management of patients with small lesions or those of uncertain malignant potential&#46; Also&#44; elderly patients with multiple comorbidities represent a subgroup in which a customized approach is frequently required&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Percutaneous biopsy or fine needle aspiration with image guidance may be an alternative in selected cases&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Yet&#44; these techniques have not been widely utilized&#44; because of concern about haemorrhage or tumor seeding along the needle track&#46; Nevertheless&#44; percutaneous biopsy may be an alternative in selected patients in whom surgery may entail significant risk&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In this regard&#44; we saw in our institution a 47-year-old female in whom an abdominal CT scan performed as a study of unspecific abdominal pain revealed an ovoid mass of 26<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>17<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>28<span class="elsevierStyleHsp" style=""></span>mm&#46; compressing the right renal pelvis with avid contrast enhancement and intra-lesion calcifications and no signs of disease spread &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46; A radical nephrectomy was performed and histopathological analysis showed a solitary fibrous tumor &#40;SFT&#41;&#46; The renal hilum contained a 2&#46;3-cm mass of adipose tissue&#46; Microscopically&#44; the neoplasm was separated from the kidney and the renal pelvis parenchyma and it was composed of round to spindle-shaped tumour cells arranged in hypercellular and hypocellular areas separated by thick bands of collagen&#46; No necrosis or atypia were observed and the mitotic index was low&#46; Tumor cells stained positive for CD34&#44; CD99&#44; Vimentin and Bcl2 and no staining was observed for AE1&#47;AE3&#44; MelanA&#44; HMB45&#44; smooth muscle actin&#44; CD10&#44; Desmin and S-100 protein &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">SFT is an unusual mesenchymal neoplasm that shows a prominent hemangiopericytoma &#40;HPC&#41;-like branching vascular pattern&#44; and in fact SFT and HPC share histological features and have almost identical gene expression profiles&#44; so they are now grouped as one entity&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">SFT&#47;HPC is more common in middle-aged adults and it occurs most frequently in the pleura&#46; Surgical resection is the gold standard and it is curative in many cases&#46; Nevertheless&#44; some reports reveal a metastatic potential even early in the course of the disease&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">SFT&#47;HPC is rarely diagnosed in genitourinary &#40;GU&#41; oncology&#46; It presents as well circumscribed masses that histologically consist of tightly packed spindle cells separated by bands of hyalinized collagen and branching blood vessels&#46; Most SFT&#47;HPC are indolent although some can behave as high-grade sarcomas&#46; There is a rare form &#40;i&#46;e&#46; Doege-Potter syndrome&#41; where patients present with very large masses and hypoglycemia induced by tumor cells production of IGF-2&#46; Radiological findings in the diagnosis of SFT usually reveal large tumors&#44; with discrete margins and avid contrast enhancement&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Resection of these tumors is curative in most cases&#44; but rare recurrences might occur mostly in tumors larger than 10<span class="elsevierStyleHsp" style=""></span>cm&#46; Doxorubicin-based chemotherapy has scarce activity in the metastatic or unresectable setting&#46; Targeted therapy inhibiting platelet-derived growth factor receptor or vascular endothelial growth factor has recently shown clinical activity in these patients and can represent an alternative treatment&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">This case illustrates a rare entity in GU oncology and attempts to summarize its clinical presentation&#44; pathology&#44; and management&#46; It also shows how accurate diagnosis of renal masses remains a challenge for the physicians involved in GU oncology&#46;</p></span>"
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Article information
ISSN: 21735786
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos