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      Malignant Solitary Fibrous Kidney Tumor with Peritoneal Disease: A Case Report

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          We report a case of a large malignant solitary fibrous kidney tumor. A complete surgical resection of the primary tumor and peritoneal disease was carried out, and a histological examination confirmed the initial diagnosis. We describe and discuss the characteristics of this rare kidney neoplasm.

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          Most cited references 10

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          Solitary fibrous tumor of the pleura.

           John Robinson (2006)
          The solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura. Only about 800 cases have been reported in the medical literature. The tumor appears to be unrelated to malignant pleural mesothelioma, the most common primary tumor of the pleura. In just over half of these cases, the neoplasm presents as an asymptomatic mass, is often quite large, and is benign in 78% to 88% of patients. The initial evaluation and diagnosis, tumor classification, surgical treatment, results of therapy, and long-term prognosis are reviewed, based on a selective review of the literature from MEDLINE beginning 1980. Complete en bloc surgical resection is the preferred treatment of benign and malignant varieties of the tumor. The pedunculated tumors attached to the visceral pleura can be effectively treated with a wedge resection of lung. Sessile tumors arising on the lung require a larger lung resection. Sessile tumors on the chest wall require wide local excision, often with chest wall resection because of their propensity for local recurrence. Adjuvant therapy remains controversial in SFTP. Benign SFTP has a high cure rate and an 8% local recurrence rate that is usually amenable to curative re-excision. Malignant SFTP, especially the more common sessile type, has a 63% recurrence rate even with complete resection. The majority of patients with recurrent disease die of the tumor within 2 years. Nevertheless, the overall long-term cure rate for all patients is 88% to 92%.
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            Malignant solitary fibrous tumor of the kidney: report of a case and comprehensive review of the literature.

            Renal solitary fibrous tumors (SFTs) have been reported infrequently. We report a 76-year-old man with a left renal mass that had previously been shown radiographically to be stable, but was now growing. Grossly, the mass measured 12 cm, was poorly circumscribed, and invaded beyond the renal capsule. Approximately 10% of the neoplasm consisted of haphazardly arranged spindle cells admixed with dense collagenous bands, which is typical of benign SFT. However, the remainder of the mass was composed of pleomorphic, spindled sarcoma cells with frequent mitoses and foci of necrosis. Immunohistochemically, we observed CD34 labeling in the benign SFT component with loss of expression in the sarcomatous component, focal labeling for Bcl-2 protein in both areas, and absence of labeling for cytokeratin, renal cell carcinoma marker, S100 protein, CD117, and muscle markers in both areas. To our knowledge, this is the first reported case of malignant renal SFT, likely representing transformation from a histologically documented benign SFT component.
              • Record: found
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              Malignant Solitary Fibrous Tumor of the Kidney: Report of the First Case Managed with Interferon

              Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.

                Author and article information

                Case Reports in Nephrology and Dialysis
                S. Karger AG
                January – April 2014
                11 April 2014
                : 4
                : 1
                : 70-74
                Departments of aUrology and bPathology, Hospital Nuestra Señora de Sonsoles, Ávila, Spain
                Author notes
                *Dr. Maria Isabel Rodríguez Cruz, Hospital Nuestra Señora de Sonsoles, Vereda de las Mozas 22; 6º 4, ES-05003 Ávila (Spain), E-Mail
                362539 PMC4025056 Case Rep Nephrol Urol 2014;4:70-74
                © 2014 S. Karger AG, Basel

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (, applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Figures: 3, Pages: 5
                Published: April 2014

                Cardiovascular Medicine, Nephrology

                Solitary fibrous tumor, Kidney, Peritoneal disease


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