[Solitary fibrous tumor of pleura (symptoms, diagnosis, treatment)]. / Solitarnaya fibroznaya opukhol' plevry (klinika, diagnostika, lechenie).

OBJECTIVE: Optimization of diagnosis and treatment of patients with solitary fibrous tumor of pleura, analysis of overall survival and disease-free survival, predictors of recurrence. MATERIAL AND METHODS: There were 66 patients with solitary fibrous tumor of pleura (26 men and 40 women) aged 57.6 years (range 26-80 years). Asymptomatic course was found in 29 (44%) patients, various symptoms - in 37 (56%) patients. Thoracotomy was applied in 36 patients, thoracoscopy - in 30 patients. Immunohistochemical examination included analysis of definition of Stat6 expression. RESULTS: Benign variant of SFT was diagnosed in 50 (75.7%) patients, malignant variant - in 16 (24.3%) patients. STAT6 expression was observed in all cases. Postoperative morbidity was 9%, mortality - 1.6%. Recurrence was diagnosed in 2 (4%) patients with benign variant of disease and in 5 (31.2%) patients with malignant variant (2 of them died from progression of disease). Progression-free survival was 89.4%, overall survival - 95.4%. Predictors of recurrence are tumor dimension over 10 cm, necrosis and/or hemorrhagic component of tumor, mitotic count of at least four per 10 high-power fields. CONCLUSION. S: Olitary fibrous tumor of pleura is a rare mesenchymal fibroblastic neoplasm growing from submesothelial layer. Differential and preoperative morphological diagnosis of SFT is difficult and demands a special immunohistochemical examination with analysis of Stat 6 expression. Surgery is preferred for tumor de novo and recurrent neoplasm.

Extrameningeal solitary fibrous tumors-surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal malignancy. Although surgery is potentially curative, the local relapse risk is high after marginal resections. Given the lack of prospective clinical trial data, the objective of the current study was to better define the role of perioperative radiotherapy (RT) in various SFT presentations by location. METHODS: This was retrospective study performed across 7 sarcoma centers. Clinical information was retrieved from all adult patients with extrameningeal, primary, localized SFT who were treated between 1990 and 2018 with surgery alone (S) compared with those who also received perioperative RT (S+RT). Differences in treatment characteristics between subgroups were tested using analysis of variance statistics and propensity score matching. Local control and overall survival rates were calculated from the start of treatment until progression or death from any cause. RESULTS: Of all 549 patients, 428 (78%) underwent S, and 121 (22%) underwent S+RT. The median follow-up was 52 months. After correction for mitotic count and surgical margins, S+RT was significantly associated with a lower risk of local progression (hazard ratio, 0.19: P = .029), an observation further confirmed by propensity score matching (P = .012); however, this association did not translate into an overall survival benefit. CONCLUSIONS: The results from this retrospective study investigating perioperative RT in patients with primary extrameningeal SFT suggest that combining RT with surgery in the management of this patient population is significantly associated with a reduced risk of local failures, especially in patients who have less favorable resection margins and in those who have tumors with a high mitotic count.

Localized malignant mesothelioma, an unusual and poorly characterized neoplasm of serosal origin: best current evidence from the literature and the International Mesothelioma Panel.

Localized malignant mesotheliomas (LMM) is an uncommon and poorly recognized neoplasm. Its pathologic diagnosis is often surprising in patients with serosal/subserosal based localized tumors that are clinically suspicious for metastatic lesions or primary sarcomas. Once a tumor is diagnosed as "mesothelioma", LMM is often mistaken for diffuse malignant mesothelioma (DMM). Best currently available evidence about LMM was collected from the literature and cases diagnosed by members of the International Mesothelioma Panel (IMP). One hundred and one (101) LMM have been reported in the English literature. Patients had localized tumors with identical histopathologic features to DMM. Patients ranged in age from 6 to 82 years; 75% were men. Most (82%) of the tumors were intrathoracic. Others presented as intrahepatic, mesenteric, gastric, pancreatic, umbilical, splenic, and abdominal wall lesions. Tumors varied in size from 0.6 to 15 cm. Most patients underwent surgical resection and/or chemotherapy or radiation therapy. Median survival in a subset of patients was 29 months. Seventy two additional LMM from IMP institutions ranged in age from 28 to 95 years; 58.3% were men. Sixty tumors (83.3%) were intrathoracic, others presented in intraabdominal sites. Tumors varied in size from 1.2 to 19 cm. Median survival for 51 cases was 134 months. Best evidence was used to formulate guidelines for the diagnosis of LMM. It is important to distinguish LMM from DMM as their treatment and prognosis is different. A multidisciplinary approach is needed for the diagnosis of LMM as it shows identical histopathology and immunophenotype to DMM.

Surgical outcomes and clinical courses of solitary fibrous tumors of pleura.

AIM: This study's aim is to define the clinicopathological and surgical outcomes, clinical courses, and results of long-term follow-up of cases that underwent surgical treatment for solitary fibrous tumors of the pleura (SFTp). SUBJECTS AND METHODS: Clinical and long-term follow-up records of 16 consecutive patients who had surgery for SFTp between 2006 and 2016 were reviewed in the retrospective chart review. There were nine males (56%) and seven (44%) females with an average age of 60.5 ± 17.1 (range 30-87 years). Etiological factors, diagnostic procedures, clinical and surgical outcomes, 5-year overall survival (OS), and disease-free survival (DFS) in these patients were researched. RESULTS: There was no remarkable common etiological factor. Nine of the cases were asymptomatic. Other symptoms were chest pain, dyspnea, cough and hypertrophic osteoarthropathy, respectively. Thoracotomy for the removal of pleural mass was carried out in 15 (94%) cases. Additional resection procedures included the chest wall resection in two cases and lobectomy in two. Video-assisted thoracoscopic surgery (VATS) resection was performed in one (6%) case. Complete surgical excision was performed in 74% of cases. Nineteen percent of cases were malignant SFTp (mSFTp). One of the mSFTp cases died in the 19th month after the diagnosis. The mean follow-up time was 50.6 ± 34.2 months (2--114 months). Mean survival of mSFTp patients was 40.6 ± 19.08 months (19--55 months) and that for benign SFTp (bSFTp) was 52.9 ± 37.05 months (2--114 months). Five-year OS--DFS were 93.5% and 74%, respectively. CONCLUSION: SFTp is an uncommon benign neoplasm but it can have malignant features. Even in the case of recurrence, the main treatment is total surgical excision. Oncologic treatments can be tried in unresectable and metastatic cases. VATS can be used in surgical total excision for small diameter and appropriate tumors. Understanding the nature of these tumors, immunohistochemical, and genetic studies may be a guide in future.

A Modern Reaffirmation of Surgery as the Optimal Treatment for Solitary Fibrous Tumors of the Pleura.

BACKGROUND: The objective of this study was to identify the most effective treatment for survival in patients with solitary fibrous tumors of the pleura (SFTP). METHODS: The National Cancer Database was queried for patients with malignant SFTPs. Patients were divided into two groups, those who had surgical treatment and those who did not. The primary outcome was 5-year overall survival, which was assessed by the Kaplan-Meier method and compared using the log rank test. RESULTS: There were 204 patients with SFTPs identified between 2004 and 2014. Of those, 65% of patients (133) had surgical intervention, whereas 18% (37) had no surgical intervention, and 17% (34) had unknown treatment information. Among patients who underwent resection, 42% (56) had sublobar resections, 45% (60) had lobectomies, and 13% (17) had pneumonectomies. The overall 5-year survival for SFTP patients was 56%. Patients who had surgical intervention had a relative survival of 64%, and patients with no surgical intervention had 22% (p < 0.001). There was no difference in 5-year survival among patients who had sublobar resections compared with patients who had greater anatomic resections (65% versus 64%, p = 0.823). CONCLUSIONS: Patients with SFTP who were managed with surgical intervention have better overall survival than patients who are not managed with surgery. Furthermore, similar 5-year survival for sublobar resections compared with greater anatomic resections suggested that the former, when possible, is sufficient.

Comparison of Risk Stratification Models to Predict Recurrence and Survival in Pleuropulmonary Solitary Fibrous Tumor.

INTRODUCTION: Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms. Most follow a benign course, but a subset will recur or metastasize. Various risk stratification schemes have been proposed for SFTs, but none has been universally endorsed and few have focused on pleuropulmonary SFTs. METHODS: Histologic sections from surgically resected pleuropulmonary SFTs were examined, with confirmatory immunohistochemistry. Patients were risk-stratified by using four prediction models as proposed by de Perrot, Demicco (original and modified), and Tapias. Kaplan-Meier analysis was used to estimate overall survival (OS) and progression-free survival (PFS). RESULTS: The 147 study patients included 78 females (53.1%) with a median age of 61.5 years (range 25-87). The median follow-up was 5.5 years (range 0-33). Recurrence or metastasis occurred in 15 patients (10.2%), with five deaths from disease. Significant predictors of worse OS included male sex, age at least 55 years, tumor size at least 10 cm, nonpedunculated growth, severe atypia, necrosis, and mitotic count of at least four per 10 high-power fields. Predictors of recurrence included tumor size of at least 10 cm, severe atypia, necrosis, at least four mitoses per 10 high-power fields, and Ki67 labeling index of at least 2%. All systems predicted PFS, but only the Demicco and Tapias systems significantly predicted OS. The modified Demicco system provided the best discrimination for PFS (C-statistic = 0.80 compared with 0.78). CONCLUSION: The risk scoring systems proposed by Tapias et al. and Demicco et al. were both predictive of OS and PFS. The Demicco system has the advantages of simplicity and applicability to SFTs from other sites, as well as provision of the best discrimination for PFS, and thus may be the best system to apply in general practice.

Prognostic value of MIB-1 proliferation index in solitary fibrous tumors of the pleura implemented in a new score - a multicenter study.

BACKGROUND: Although the majority of solitary fibrous tumors of the pleura (SFTP) follow a benign course, 10-25% of patients suffer from recurrence or metastatic disease. Several scoring models have been proposed to predict the outcome. However, none of these included immunohistochemical (IHC) markers as possible prognosticators. METHODS: In this multicenter study, we collected clinical data and formalin-fixed and paraffin-embedded (FFPE) tissue blocks of patients with histologically proven SFTP which had been surgically resected between 2000 und 2015. After systematic and extensive IHC staining on tissue microarrays, the results were analyzed and compared to histomorphological and clinical data for their possible prognostic value. RESULTS: In total, 78 patients (mean age 61 ± 11 years) were included. Of these, 9 patients (11%) had an adverse outcome including SFTP recurrence (n = 6) or SFTP-related death (n = 3). Mean overall survival was 172 ± 13 months. 1 and 10-year event-free survival rates were 99% and 93%. In the multivariable analysis only MIB-1 proliferation index (Ki-67) ≥10% (HR 12.3, CI 1.1-139.5, p = 0.043), ≥4 mitoses per 10 high power fields (HR 36.5, CI 1.2-1103.7, p = 0.039) and tumor size larger than 10 cm (HR 81.8, CI 1.7-4016.8, p = 0.027) were independently associated with adverse outcome. CONCLUSION: A high proliferation rate by MIB-1 IHC was associated with impaired outcome. Upon this, we established a new score using mitosis, necrosis, size of the tumor and MIB-1, which performed better than the traditional scores in our data set. This prognostic score could help to better evaluate outcome of SFTP, but requires external validation.

Solitary Fibrous Tumor - Less Common Neoplasms of the Pleural Cavity.

PURPOSE: solitary fibrous tumors (SFT) represent a heterogeneous group of primary pleural neoplasms with a low incidence rate and of which the biological origin, which consists of mesenchymal cells, is uncertain. METHODS: The authors present herewith a retrospective analysis of 22 patients with SFTs who were diagnosed and surgically treated between the years 2000-2015. The preoperative tumors were successfully verified morphologically by transthoracic core needle biopsy under CT control in 27.3% of patients. Surgical approaches were either posterolateral thoracotomy or videothoracoscopy. The follow-up median was 45 months (range 1-188 months). RESULTS: Twenty tumors were surgically removed radically, two tumors were found to be unresectable due to the considerable tumor size. From histological point of view 81.8% of tumors were SFT with low malignant potential, 18.2% of tumors with high malignant potential. Despite the radical extirpation of the SFT, it relapsed in two patients. CONCLUSION: The gold standard of SFT treatment is radical surgical removal; however, patients at risk of recurrence require additional follow-ups. The results of adjuvant therapy in recurrent and malignant forms of SFTs are the subject of discussion and further study.

Prognostic factors in solitary fibrous tumors of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura (SFTP) are rare neoplasms originating from submesothelial mesenchymal cells with fibroblastic differentiation. The clinical behavior of SFTPs is mostly benign; however, up to 20% of patients develop local recurrence and/or distant metastasis. Although different risk-stratification models have been described, definitive criteria to predict a malignant clinical course of SFTP are still lacking. METHODS: In a retrospective analysis at a single-institution, 25 patients with histologically proven SFTP were identified. Clinicopathologic and survival data were collected and pathologic sections reviewed. Different markers and risk-stratification models were correlated with disease- and overall-free survival by Kaplan-Meier analysis. RESULTS: Of 25 SFTP, 8 tumors (32%) were classified as malignant according to the World Health Organization criteria. Three patients (12%) developed recurrence. Cohort median follow-up was 28 mo, and median overall survival was 160 mo. Comparison of proliferation markers showed higher mitosis count per high-power field and MIB-1 labeling index (MIB) in malignant compared with nonmalignant SFTP. MIB was identified as a predictor for disease-free survival. Applying the previously reported classifications to categorize SFTP according to the probability to show malignant behavior, significant differences in disease-free survival were also present in our cohort. CONCLUSIONS: In the present analysis of rare SFTP, previously proposed staging systems were applicable for prediction of disease-free survival. Independently of treatment, MIB was the only sole predictive marker. A prospective multi-institutional database could be helpful in establishing detailed predictive criteria in patients diagnosed with SFTP.

Malignant solitary fibrous tumors of the pleura: retrospective review of a multicenter series.

INTRODUCTION: Available data on the malignant solitary fibrous tumor of the pleura (mSFTP), a very rare neoplasm with unpredictable prognosis, are scarce. The aim of this study is to collectively analyze the aggregated data from the largest series in the English literature to date, a multicenter, 10-year study of 50-cases. METHODS: We retrospectively reviewed the clinical records of patients who underwent surgical resection for mSFTP in the period between January 2000 to July 2010. Long-term survival (LTS) and 5-year disease-free survival were analyzed in detail. RESULTS: There were 24 men and 26 women (median age, 66 years; age range, 44-83 years). Thirty-two patients (64%) were symptomatic. A malignant pleural effusion was diagnosed in 12 cases. Surgical resection included isolated mass excision in 13 patients and extended resection in 35. In the remaining two cases only biopsies were undertaken. The resection was complete in 46 cases (92%). Adjuvant treatment was administered to 15 patients. Median follow-up was 116 months (range, 18-311 months). Overall LTS and disease-free survival were 81.1% and 72.1%, respectively. Fifteen patients (30%) experienced a relapse of the disease. Complete resection yielded much better LTS than partial resection (87.1% versus 0%; p < 0.001). At the Cox regression analysis, incomplete resection (hazards ratio [HR]: 39.02; 95% confidence interval [CI]:4.04-380.36; p = 0.002) and malignant pleural effusion (HR: 3.44; 95%CI: 0.98-12.05; p = 0.053) were demonstrated to be risk factors for earlier death. At multivariate analysis, chest-wall invasion and malignant pleural effusion increased the risk of recurrence (HR: 4.34; 95%CI: 1.5%-12.6%; p = 0.007 and HR: 3.48; 95%CI: 1.1%-11.0%; p = 0.038, respectively). CONCLUSIONS: Surgical resection remains the treatment of choice for mSFTP. Relapse is common (approximately 30%). Incomplete resection and malignant pleural effusion at diagnosis impact LTS negatively.

Solitary fibrous tumor of the pleura: outcomes of 157 complete resections in a single center.

BACKGROUND: The purpose of this study was to identify factors affecting long-term outcomes after complete resection of solitary fibrous tumors of the pleura (SFTP). METHODS: This was a single-center retrospective study using data from patients operated on from January 1980 to December 2010. RESULTS: Of the 157 patients (72 men, 85 women; median age, 58 years [13-87 years]), 60 (38%) had symptoms. All patients had complete en bloc resection with wedge lung excision (n=122), lobectomy (n=15), bilobectomy (n=3), segmentectomy (n=1), pneumonectomy (n=4), chest wall resection (n=8), diaphragm resection (n=3), or multilevel hemivertebrectomy (n=1). The tumors were pedunculated (n=89) or sessile (n=68). Definitive histologic examination showed benign tumors (bSFTP) in 90 patients (57%) and malignant tumors (mSFTP) in 67 (43%) patients. Compared with the bSFTP group, the mSFTP group had significantly larger tumors (13.4 cm vs 6.4 cm; p<0.0001) and a nonsignificantly higher proportion of symptomatic patients (58% vs 23%). Overall operative mortality and morbidity rates were 0.6% and 5.7%, respectively, with no significant difference between patients with mSFTP and those with bSFTP. The 5-year survival rate was better in patients with bSFTP than in patients with mSFTP (96% vs 68%; p=0.0003). Tumor recurrence was more common in patients with mSFTP than in those with bSFTP (16% vs 2%; p<0.0001) and was associated with decreased survival (p=0.02). Sessile tumors (p=0.05), CD34-negative tumors (p=0.005), and extensive surgical procedures (p=0.04) were significant risk factors for tumor recurrence. CONCLUSIONS: Complete en bloc resection of SFTP provides good long-term survival. Tumor recurrence is the main risk factor for death and may occur in mSFTP despite en bloc resection and requires multimodal treatment and close follow-up.

Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model.

Solitary fibrous tumor represents a spectrum of mesenchymal tumors, encompassing tumors previously termed hemangiopericytoma, which are classified as having intermediate biological potential (rarely metastasizing) in the 2002 World Health Organization classification scheme. Few series have reported on clinicopathological predictors with outcome data and formal statistical analysis in a large series of primary tumors as a single unified entity. Institutional pathology records were reviewed to identify primary solitary fibrous tumor cases, and histological sections and clinical records reviewed for canonical prognostic indicators, including patient age, tumor size, mitotic index, tumor cellularity, nuclear pleomorphism, and tumor necrosis. Patients (n=103) with resected primary solitary fibrous tumor were identified (excluding meningeal tumors). The most common sites of occurrence were abdomen and pleura; these tumors were larger than those occurring in the extremities, head and neck or trunk, but did not demonstrate significant outcome differences. Overall 5- and 10-year metastasis-free rates were 74 and 55%, respectively, while 5- and 10-year disease-specific survival rates were 89 and 73%. Patient age, tumor size, and mitotic index predicted both time to metastasis and disease-specific mortality, while necrosis predicted metastasis only. A risk stratification model based on age, size, and mitotic index clearly delineated patients at high risk for poor outcomes. While small tumors with low mitotic rates are highly unlikely to metastasize, large tumors ≥ 15 cm, which occur in patients ≥ 55 years, with mitotic figures ≥ 4/10 high-power fields require close follow-up and have a high risk of both metastasis and death.

Video-assisted thoracoscopic surgery (VATS) for patients with solitary fibrous tumors of the pleura.

OBJECTIVES: To present our experience of video-assisted thoracoscopic surgery (VATS) for patients with solitary fibrous tumors of the pleura (SFTPs) and to discuss the treatment of choice of such neoplasms. METHODS: Between June 2000 and September 2008, 21 patients with SFTPs (9 men and 12 women) underwent VATS at our department. The mean age was 52.5 years (range, 33-76 years). RESULTS: Surgical excision was performed in all patients. Surgical excision was performed by VATS in 15 patients (71.4%), by VATS plus a small thoracotomy (<5 cm) in 4 patients (19.0%), and by posterolateral thoracotomy accompanied by VATS in 2 patients (9.5%). Mean chest drain duration was 2.3 days (range, 1-4 days), and the mean hospital stay was 7.2 days (range, 4-15 days). There were 18 pathologically benign SFTP cases (85.7%) and 3 malignant SFTP cases (14.3%). There was no operative morbidity or mortality. No recurrence or metastasis of SFTPs developed during postoperative median follow-up period of 43 months. CONCLUSIONS: Complete resection and close follow-up for years after operation is recommended for SFTPs. VATS may play an important role in reducing the size of the thoracotomy incision in the treatment of SFTPs, which results in less invasive surgery.

Solitary fibrous tumor of the pleura: surgery and clinical course in 18 cases.

Solitary fibrous tumors of the pleura are very rare neoplasms that can sometimes present with malignant features. Between 1984 and 2007, 18 cases were treated in our institution. There were 7 men and 11 women, with a median age of 56 years (range, 33-77 years). All patients underwent surgical treatment. Except for one case with hemangiopericytic features, all tumors were histologically the fibrous type of pleural mesothelioma. Resections were radical and there were no recurrences. There was no perioperative mortality. The outcome was excellent, and all patients have been followed up continuously. Survival rates at 3, 5, and 10 years were calculated as 86.7%, 75%, and 66.7%. One patient died after 18 months (malignant type of solitary fibrous tumor), and 2 died of unrelated disease after 24 and 53 months. Surgery is the treatment of choice, and careful long-term clinical follow-up is required.

Pleuro-pulmonary solitary fibrous tumors: a clinicopathologic, immunohistochemical, and molecular study of 88 cases confirming the prognostic value of de Perrot staging system and p53 expression, and evaluating the role of c-kit, BRAF, PDGFRs (alpha/beta), c-met, and EGFR.

Pleuro-pulmonary solitary fibrous tumor (SFT) is a relatively uncommon mesenchymal neoplasm of uncertain histogenesis, unknown molecular features, and unpredictable clinical behavior. Although complete resection is universally accepted as the most important single prognostic factor, some clinicopathologic characteristics (gross appearance, tumor size, mitotic index, tumor necrosis, hypercellularity, and pleomorphism) are related to patient outcome, and a staging system based on these parameters with practical therapeutical implications has been recently proposed by de Perrot et al. Here, 88 pleuro-pulmonary SFTs were collected and clinicopathologic characteristics including de Perrot classification, patients' follow-up, p53 expression, and several "targetable" kinases [c-kit, v-raf murine sarcoma viral oncogene homolog B1, platelet-derived growth factor receptor (PDGFR)-alpha/beta, c-met, epidermal growth factor receptor (EGFR)] were retrospectively analyzed. Fifty-two cases (59%) had at least 1 clinicopathologic feature related to malignancy, whereas mortality and recurrences occurred in 10.2% and 18.2% of the cases, respectively. de Perrot staging and high p53 expression were significantly related to the conventional clinicopathologic prognostic features as well as to overall survival (OS) and disease-free survival (DFS) (P<0.001). At multivariate analysis, high p53 expression and tumor necrosis were the only parameters significantly associated with OS and DFS (P=0.017 and P=0.012, respectively). Immunohistochemical expression was frequently detected for PDGFR-alpha (97.7%), PDGFR-beta (86.5%), and hepatocyte growth factor receptor (96.6%), whereas missense mutations were only identified in 2 cases both involving PDGFR-beta (exons 18 and 20). We conclude that de Perrot stratification of SFT is a reliable prognostic indicator and merits consideration in view of its suggestions for the management of these tumors in daily practice. p53 expression may represent a valid and easy-to-test prognostic factor significantly related to OS and DFS. Although mutations of the corresponding genes are rare events in SFT, PDGFR-alpha/beta, and hepatocyte growth factor receptor tyrosine kinases should be further investigated given the availability of specific inhibitory molecules which might provide useful and novel therapeutical approaches for SFT patients.

[Solitary fibroma of the pleura - clinical findings and prognosis]. / Solitäre fibröse Tumoren der Pleura - Klinik und Prognose.

Solitary fibrous tumours of the pleura can arise from either the visceral or the parietal pleura. Female and male subjects are affected equally; solitary tumours of the pleura are found at all ages with a main incidence in the sixth and seventh decades. The current literature describes the origin of both benign and malign tumour cells as being from multipotential submesothelial fibrous cells. The clinical appearance is unspecific and characteristic symptoms are lacking; cough, feeling of pressure in the thoracic chest or dyspnoea are the main leading symptoms and correlate with tumour size. A CT scan of the chest is the diagnostic tool of choice. Frequently, a solitary tumour of the pleura is found incidentally. Paraneoplastic syndromes are described and hypertrophic pulmonary osteoarthropathia (HPO) is the most common in cases of a solitary fibrous tumour of the pleura. The therapy of choice is complete surgical resection. There are no data supporting a neoadjuvant or adjuvant therapy. There are no known risk factors and complete resection is the only factor of prognostic relevance. Patients with a benign solitary fibrous tumour of the pleura have a far better survival compared to those with the malignant variant. In two recent reviews, the 5- and 10-year survival rates are described as being 97 and 90 %, respectively, for benign tumours compared with 89 and 88 % for malignant fibromas.