OBJECTIVE: Localized fibrous tumors of the pleura are
rare. We report 7 cases and review the literature in order to
define the range of clinical characteristics of these tumors,
treatment options, and prognosis.
METHODS: A retrospective review of 7 cases treated between
1997 and 2003, focusing on clinical presentation, diagnostic tests,
and treatment. The tumor was removed surgically in all cases,
by thoracotomy in 6 cases and by video-assisted thoracoscopic
surgery in 1 patient.
RESULTS: All patients recovered fully after surgery, with no
postoperative complications. One patient experienced recurrence
twice, 33 and 67 months after the initial resection. All are alive
at the time of writing, after a mean follow up period of 19
months (range 1 month to 5 years and 10 months).
CONCLUSIONS: The treatment of choice for a fibrous tumor
of the pleura is full resection and follow up to detect possible
late recurrence

Delgado, M. (M.)

Rodriguez-Spiteri, N. (Natalia)

Sola, J.J. (Jesús Javier)

Torre, W. (Wenceslao)

Universidad de Navarra

Localized Fibrous Tumors of the Pleura: Experience With 7 Recent Cases

Dadun, University of Navarra

IntroductionSolitary fibrous tumors account for 10% of neoplasmsthat arise in the pleura.1 Unlike pleural mesothelioma, inwhich asbestos plays an important role, solitary tumorsof the pleura have not been clearly linked to a particularetiological factor. Another difference between solitarytumors and pleural mesothelioma is that most solitarytumors are benign, only 12% proving malignant.2Solitary fibrous tumors were once thought to arisefrom mesothelial pleural cells,3 but thanks toimmunohistochemistry we now know them to bemesenchymal, with positive CD34 and vimentinexpression and negative expression of cytokeratins andother epithelial antigens—a profile that is the oppositeof the one seen for malignant mesothelioma.2We describe 7 patients with solitary fibrous tumors ofthe pleura treated in our hospital in order to analyze therole of surgery.Case DescriptionSeven patients were treated between 1997 and 2003 in atotal of 9 operations. Three were women and 4 men (Table 1).Ages ranged from 25 to 63 years (mean, 52.5 years).Three patients were asymptomatic at the time of diagnosis.The most relevant symptoms were hypoglycemia andacropachy, lower extremity edema and coughing, ipsilateralarm edema, and moderate pain on the side where the masswas located (Table 1).Arch Bronconeumol 2004;40(7):329-32 329CASE REPORTSLocalized Fibrous Tumors of the Pleura: Experience With 7 Recent CasesW. Torre,a N. Rodríguez-Spiteri,a J.J. Sola,b and M. DelgadoaaDepartamento de Cirugía Torácica, Clínica Universitaria de Navarra, Pamplona, Navarra, Spain.bDepartamento de Anatomía Patológica, Clínica Universitaria de Navarra, Pamplona, Navarra, Spain.OBJECTIVE: Localized fibrous tumors of the pleura arerare. We report 7 cases and review the literature in order todefine the range of clinical characteristics of these tumors,treatment options, and prognosis.METHODS: A retrospective review of 7 cases treated between1997 and 2003, focusing on clinical presentation, diagnostic tests,and treatment. The tumor was removed surgically in all cases,by thoracotomy in 6 cases and by video-assisted thoracoscopicsurgery in 1 patient.RESULTS: All patients recovered fully after surgery, with nopostoperative complications. One patient experienced recurrencetwice, 33 and 67 months after the initial resection. All are aliveat the time of writing, after a mean follow up period of 19months (range 1 month to 5 years and 10 months).CONCLUSIONS: The treatment of choice for a fibrous tumorof the pleura is full resection and follow up to detect possiblelate recurrence.Key words: Pleural neoplasm. Solitary fibrous tumor. Pleura.Mesotelioma fibroso localizado de pleura: nuestraexperiencia quirúrgica en 7 casos recientesOBJETIVO: El tumor fibroso localizado de pleura es una le-sión poco frecuente. El objetivo de este artículo es presentar7 casos propios y revisar la bibliografía existente, con el finde establecer el espectro clínico de estos tumores, el trata-miento y su pronóstico.MÉTODOS: Revisión retrospectiva de una serie de 7 casosintervenidos entre 1997 y 2003, analizando el modo de pre-sentación, las pruebas diagnósticas y el tratamiento. En to-dos los casos se realizó una resección quirúrgica de la masa,que se llevó a cabo por toracotomía en 6 de los pacientes ypor videotoracoscopia en el paciente restante.RESULTADOS: Tras la cirugía todos los pacientes se recupe-raron completamente, sin la presencia de complicacionespostoperatorias. Un caso recidivó en 2 ocasiones, a los 33 y alos 67 meses de la resección inicial. Todos ellos están vivosen la actualidad tras un período de seguimiento medio de 19meses (desde 1 mes a 5 años y 10 meses).CONCLUSIONES: El tratamiento de elección del tumor fibro-so de pleura es la resección completa con seguimiento poste-rior, por la posibilidad de recidivas tardías de la enfermedad.Palabras clave: Neoplasia pleural. Tumor fibroso solitario. Pleura.Correspondence: Dr. W. Torre.Departamento de Cirugía Torácica. Clínica Universitaria de Navarra. Avda. Pío XII, 36. 31008 Pamplona. Navarra. España.Correo electrónico: wtorre@unav.esManuscript received 11 November, 2003. Accepted for publication 23 December, 2003.All patients had computed tomography (CT) scans of thechest (Figure 1) showing a mass with well-defined borders.Pleural effusion was present in 3 cases. The lesion wasbiopsied before resection in 4 cases and the results wereconsistent with a diagnosis of localized fibrous tumor of thepleura in 3 and adenocarcinoma in 1. Mean tumor sizemeasured at the widest point was 9.8 cm (range, 2.5 to 15 cm).The treatment of choice was complete surgical resection ofthe mass by thoracotomy in 6 cases and video-assistedthoracoscopy in 1 case (Figure 2). No complementarytreatments were given (Table 1).Histology of the excised tissue confirmed the diagnosis oflocalized fibrous tumor of the pleura in all cases. Cellpolymorphism and a high number of mitoses in 1 patientsuggested the possibility of malignant progression. The mostrepresentative results of antigen marker studies in the patientsfor whom they were ordered were positive vimentin and Bcl-2expression and negative cytokeratin expression in all patientsand CD34 positivity in 66% (Table 2).One patient experienced recurrence even though histologysuggested the tumor was benign. The first recurrence was 33months after resection of the first tumor and the secondrecurrence occurred 33 months after the second tumorresection. Both recurrences were treated by surgicalresection.DiscussionLocalized fibrous tumors of the pleura are fairly rare.Although they have been reported in patients as youngas 5 years of age and as old as 87 years, most appear inthe sixth and seventh decades of life, with fairly equalfrequency in men and women.4 Clinical presentationvaries and diagnosis is fortuitous in 50% of cases.1Three of our 7 patients were asymptomatic at the timeof diagnosis. Symptoms arise mainly because localspread of the mass causes coughing (33%), chest painTORRE W, ET AL. LOCALIZED FIBROUS TUMORS OF THE PLEURA: EXPERIENCE WITH 7 RECENT CASES330 Arch Bronconeumol 2004;40(7):329-32Figure 1. Computed tomography scan of the chest in which a large,heterogeneous mass with well-defined borders can be seen occupyingnearly the entire left side of the thorax, displacing mediastinal structures.Figure 2. Videothoracoscopic view. The endostapler (E) can be seenstarting to section the pedicle of the tumor (PT). The arrow shows themacroscopic border between the tumor (T) and its pedicle. The lungparenchyma (P) is located to the left of the mass. TABLE 1Clinical Findings*Case Sex/Age, Years Signs/Symptoms Computed Tomography Size, cm Biopsy1 Male/63 Asymptomatic Mass, left lower quadrant 15×9×8 Consistent with SFTPMicrocalcificationsPleural effusion2 Female/25 Edema, upper left arm Mass, left upper quadrant 13×12×10 Consistent with SFTP and benign pleural tumorCalcifications, cystic areas3 Male/63 Asymptomatic Nodule, right upper lobe 2.5×2Pleural effusion4 Female/50 Moderate pain, right side Mass, right lower quadrant 11×10×5,5 Adenocarcinoma5 Male/52 Edema, lower extremities; Mass, right lower quadrant 7.5×7×3.5cough6 Male/58 Asymptomatic Mass, juxtadiaphragmatic, left 4.77 Female/57 Hypoglycemia Mass, left lower quadrant 15×15×10 Consistent with SFTP Pleural effusion*SFTP indicates solitary fibrous tumor of the pleura.(23%), breathlessness (19%), fever (17%), and lessoften hemoptysis and obstructive pneumonitis. Pleuraleffusion occurs in 10% of cases,2 but was seen on theCT scans of 3 of our patients. Systemic signs are lesscommon: acropachy occurs in 10% to 20%,5 and insulingrowth factor II is secreted by the tumor in 5%, leadingto refractory paraneoplastic hypoglycemia thatdisappears 3 or 4 days after surgery.6 One of ourpatients, who also had acropachy, debuted with loss ofconsciousness caused by an episode of severehypoglycemia. Weight loss2 and galactorrhea1 are otherpossible signs of the tumor.The diagnostic process remains complex. Simpleradiographs will reveal an intrathoracic tumor that isround, homogeneous, solitary, and that has well-definedborders.4 Such findings are not specific to the diagnosis,however. A CT scan will reveal a lobulated mass withheterogeneity that varies according to the grade ofnecrosis and the presence or not of calcifications.Magnetic resonance imaging is used to establish theborders between the lesion and adjacent structures andto confirm the intrathoracic location.7 The role ofpositron emission tomography is currently beingdebated and more experience is needed beforeconclusions can be taken.8 The differential diagnosis iswide-ranging and complex in many cases, given thatbronchogenic cysts, pulmonary abscesses, other lungand pleural neoplasms, lipomas, sarcomas, andmalignant fibrous histiocytomas, among other entities,can have similar clinical and radiographic signs.9Transthoracic CT-guided biopsy does not seem to be areliable diagnostic method10: given the heterogeneousdistribution of cellular density within the tumor, thematerial obtained by this method may be insufficient.11However, some authors consider it to be of greatdiagnostic value.12 In our experience, in 3 of the 4biopsies performed, the diagnosis was congruent withthe study of the excised tissue. Many authors insist thatsurgery is the only method for reaching a firm diagnosisthrough histology of the resected tissue.One of the main concerns after diagnosing a localizedfibrous pleural mass is to try to predict how it willbehave. Currently, no specific test is available forachieving that goal. Traditionally, radiographic findingshave been described as suggesting the tumor is benign ormalignant. Likewise, cytogenetic studies have attemptedto determine the alterations that are responsible for thetumor’s greater or lesser capacity to invade surroundingtissue; among the markers suggested, p53 and Ki-67have been found to be particularly important.13 Theimportance of differentiating between benign andmalignant localized fibrous tumors lies in the differencein recurrence rates for the 2 types. Only 2% of benigntumors recur locally,3 although recurrence has beenreported as long as 17 years after resection of the primarytumor.6 Metastasis is very rare.3 However, the tumor’sbehavior does not always correlate with histology, giventhat a lesion may degenerate as time passes, to becomemalignant.4 In one of the cases we report, in whichhistology suggested that the mass would not behaveaggressively, it nevertheless recurred twice. Briselli et al2described tumor size, the presence or absence of necrosisin the excised tissue, cell polymorphism, the number ofmitoses, and resectability as being useful prognosticfactors, although the growth pattern may be the mostimportant predictor, even stronger than histologicfeatures. Egland et al4 affirmed that whether the tumorcould be fully resected or not is the factor that indicatesmagnitude of risk. De Perrot et al9 reviewed 185 casesand proposed a system of staging based on morphologicand histologic features of the tumor, divided in 5 stages(0 to IV), each with a distinct prognosis.The consensus is that full resection of the tumor isthe treatment of choice in all cases and, if possible, witha margin of safety of 1 to 2 cm.12 Adjuvant treatmentwith chemo- and radiotherapy is controversial, partlybecause experience is still limited. Chemo- andradiotherapy have been reported as prolonging survivalin patients whose tumor was not fully resectable atpresentation.15 De Perrot et al9 established a treatmentTORRE W, ET AL. LOCALIZED FIBROUS TUMORS OF THE PLEURA: EXPERIENCE WITH 7 RECENT CASESArch Bronconeumol 2004;40(7):329-32 331TABLE 2Interventions and Clinical Course*Case Approach Immunohistochemistry Clinical Course1 Left posterolateral thoracotomy Vimentin +, CD34 –, S100 –, LCA–, cytokeratin – Local recurrence, 2.8 years; second local recurrence, 5.6 years2 Left, extended axillary thoracotomy Vimentin +, S100 +, CD34 –, pancytokeratin – Asymptomatic3 Right posterolateral thoracotomy CD34 +, S100 –, CD68 –, keratin –, actin – Asymptomatic4 Right posterolateral thoracotomy Vimentin +, CD99 +, Bcl-2 +, CD68+, CAM 5.2 – Asymptomatic5 Right posterolateral thoracotomy Vimentin +, CD34 + Asymptomatic6 Left video-assisted thoracoscopy Bcl-2 +, CD34 +, cytokeratin AE3/AE1 – Asymptomatic7 Left posterolateral thoracotomy Ki-67 (10%), CD34 +, Bcl-2 +, Asymptomaticthrombomodulin +, calretinin –, MOC31 –, carcinoembryonic antigen–, TTF1 –*LCA indicates leukocyte common antigen; CAM, cell adhesion molecule, TTF, thyroid transcription factor.algorithm based on the aforementioned 5-stage criteria,with indications that complementary treatments may bebeneficial for the patient after resection of malignantand sessile tumors. Based on our experience, we haveconcluded that the treatment of choice for these tumorsat present is surgery and that more evidence is neededbefore more precise conclusions can be reachedregarding complementary treatments.REFERENCES1. Mezzetti M, Panigalli T, lo Giudice F, Cappelli R, Giuliani L,Raveglia F, et al. Surgical experience of 15 solitary benign fibroustumor of the pleura. Crit Rev Oncol Hematol 2003;47:29-33.2. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of thepleura: eight new cases and review of 360 cases in the literature.Cancer 1981;47:2678-89.3. Klemperer P, Rabin CB. Primary neoplasm of the pleura: a reportof five cases. Arch Pathol 1931;11:385-412.4. Egland DM, Hochholzer L, McCarthy MJ. Localized benign andmalignant fibrous tumors of the pleura: a clinicopathologic reviewof 223 cases. Am J Surg Pathol 1989;13:640-58.5. Okike N, Bernatz PE, Woolner LB. Localized mesothelioma ofthe pleura: benign and malignant variant. J Thorac CardiovascSurg 1978;75:363-72.6. Luque-Sánchez F, Ruiz Zafra J, Sánchez-Palencia A, Ramos A.Hipoglucemia grave secundaria a un mesotelioma fibroso pleuralrecidivado. Arch Bronconeumol 1999;35:102-3.7. de Perrot M, Kurt AM, Robert JH, Borisch B, Spiliopoulos A.Clinical behavior of solitary fibrous tumors of the pleura. AnnThorac Surg 1999;67:1456-9.8. Cortés J, Rodríguez J, García-Velloso MJ, Rodríguez-Espiteri N,Boan JF, Castellano JM, et al. [(18)F]-FDG PET and localizedfibrous mesothelioma. Lung 2003;181:49-54.9. de Perrot M, Fischer S, Brüdler MA, Sekine Y, Keshavjee S.Solitary fibrous tumors of the pleura. Ann Thorac Surg 2002;74:285-93.10. Altinok T, Topçu S, Tastepe AI, Yazici U, Çetin G. Localizedfibrous tumors of the pleura: clinical and surgical evaluation. AnnThorac Surg 2003;76:892-5.11. Ali SZ, Hoon V, Syed H, et al. Solitary fibrous tumor. Cancer1997;81:116-21.12. Weynand B, Noel H, Goncette L, Noirhomme P, Collard P. Solitaryfibrous tumor of the pleura. A report of five cases diagnosed bytransthoracic cutting needle biopsy. Chest 1997;112:1424-8.13. Yokoi T, Tsuzuki T, Yatabe Y, Suzuki M, Kurumaya H, KoshimaT, et al. Solitary fibrous tumour: significance of p53 and CD34immunoreactivity in its malignant transformation. Histopathology1998;32:423-32.14. Veronesi G, Spaggiari L, Mazzarol G, de Pas M, Leo F, Solli P, etal. Huge malignant localized fibrous tumor of the pleura. JCardiovasc Surg 2000;41:781-4.15. Suter M, Gebhard S, Boumghar M, Peloponisios N, Geton CY.Localized fibrous tumours of the pleura: 15 new cases and reviewof the literature. Eur J Cardiothorac Surg 1998;14:453-9.TORRE W, ET AL. LOCALIZED FIBROUS TUMORS OF THE PLEURA: EXPERIENCE WITH 7 RECENT CASES332 Arch Bronconeumol 2004;40(7):329-32

Localized Fibrous Tumors of the Pleura: Experience With 7 Recent Cases

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