Solitary Fibrous Tumor Of The Pleura Research Articles

Solitary fibrous tumours of the pleura: do we need a different perspective on malignancy?

Solitary fibrous tumours of the pleura (SFTP) are historically considered to be benign soft tissue neoplasms. However, a clinical relevant number of these neoplasms have malignant histological features. The objective of this study was to evaluate the percentage of SFTP presenting unfavourable clinical behaviour in order to predict negative long-term outcome. A retrospective review of 74 patients treated at 4 hospitals between 1990 and 2013 was performed. The median follow-up was 10 years (range: 1-20 years). Risk of tumour recurrence and metastases (unfavourable clinical behaviour) with regard to histology using the Kaplan-Meier and Cox proportional hazards methods. The mean age was 61 years (SD 12.75 years). There were 31 male patients (58%) and 43 female patients (42%). Tumour size ranged from 1 to 30 cm (mean 9.09 cm; SD 6.22 cm). Complete resection (R0) was achieved by minimally invasive thoracoscopic resection in 29% and thoracotomy in 57%; 25% of SFTPs showed histological evidence of malignancy, according to England criteria. Recurrence occurred in 21% and 10% of patients had metastases; 83% of patients with metastases and 39% of patients with recurrence died within 5 years. The median recurrence-free survival for histologically benign SFTP was not reached, compared to 8 years for malignant SFTP. The five-year overall survival rate was 84%. Mitotic rate ≥1/10 HPF, high cellularity, nuclear atypia, Ki-67 level >5% and poorly circumscribed (sessile) growth pattern were associated with poor long-term outcome. Pathological differentiation of SFTP morphology into pedunculated, well circumscribed and poorly circumscribed (sessile) growth pattern is recommended. Due to the misleading classification into histologically benign and malignant, all unpedunculated SFTP should be classified as potentially aggressive. Lifelong follow-up is mandatory.

Solitary fibrous tumor of the pleura with air cystic component

We present an uncommon case of a solitary fibrous tumor of the pleura with the appearance of an air-containing cystic mass. We discuss the differential diagnosis through the imaging findings, the hypothetical origins of the air component, and the possible relationship between the air component and the aggressivity of the tumor.

Solitary fibrous tumor of the pleura: A rare case report

The solitary fibrous tumor of the pleura, first conceptualized in 1870, is a rare mesenchymal neoplasm that represents less than 5% of all tumors originating in the pleura. It is most common between the sixth and seventh decade of life, affecting men and women equally, with no associated hereditary or environmental risk factors.

Pleural malignant fibrosolitary tumor: A rare entity

Solitary fibrous tumors of the pleura represent a rare histological entity, belonging to the groups of pleural tumors of sub-mesothelial origin , therefore some authors evoke the hypothesis of its proliferation from submesothelial tissue of the pleura .The clinical manifestations are varied and the certain diagnostic can only be made by anatomopathological examination which evaluates precise malignancy criteria. Total surgical removal is the only way to ensure a definitive cure. We report the case of 65-year-old woman with a malignant solitary fibrous tumor operated by thoracotomy with complete resection. Through this presentation, we would like to underline the rarity of this entity

A Small And a Large Solitary Fibrous Tumors of the Pleura

Solitary Fibrous Tumor of the Pleura (SFTP) is an exceptionally rare mesenchymal tumor that typically originates from the visceral pleura and constitutes less than 5% of all pleural tumors. While it often displays benign histological features, the tumor’s behavior remains enigmatic and less comprehensively understood, primarily owing to its limited incidence and case studies. We present two cases of SFTP. The First one was a small pleural nodule a nodule in a 61 year old patien, confirmed as a SFTP through CT-guided biopsy. Successful tumor resection was performed, with histopathological confirmation, and the patient remained recurrence-free for two years. The second one was incidental finding in a 68-year-old patient of a huge pleural mass with necrotic regions and microcalcifications on imaging. Surgical removal revealed an encapsulated tumor with myxoid features, classified as an intermediate-risk SFTP. The patient experienced no complications and had no local recurrence 16 months post-surgery. Solitary fibrous tumors within the pleura are uncommon. The preferred treatment approach is surgical resection, with diligent post-operative monitoring.

Solitary Fibrous Tumor of the Pleura: A Case Report

Solitary fibrous tumors of the pleura are rare malignancies, with fewer than 1000 reported cases. They are either discovered incidentally, or in association of non-specific symptoms such as hypoglycemia. The association of paraneoplastic hypoglycemia and solitary fibrous pleural tumors is rare. Hypoglycemia occurs in large tumors and normalizes after surgery, the treatment of choice for pleural solitary fibrous tumors (PSTF). The authors discuss the management of this rare tumor and the association of solitary fibrous tumor of the pleura (SFTP) with hypoglycemia.

Anesthetic management of thoracotomy for massive intrathoracic solitary fibrous tumor of the pleura: a case report

BackgroundsSolitary fibrous tumor of the pleura (SFTP) is a rare thoracic tumor and usually asymptomatic. Massive SFTP may affect adjacent organs and tissues including pulmonary vasculature, bronchus and heart. A thoracotomy for massive SFTP is necessary in severe case. Therefore, it is important for anesthesiologists to understand the condition of patients with massive SFTP and develop an appropriate anesthetic management strategy.Case summaryA 76-year-old woman with massive SFTP presented to our clinical center and was evaluated as requiring thoracotomy. She received multidisciplinary cooperation treatment from the radiology, cardiac, thoracic surgery and anesthetic teams. The perioperative management of anesthesiologists played a crucial role in the great prognosis of this woman.ConclusionsThis case report demonstrates the importance of comprehensive and meticulous perioperative management and provides guidance to the multidisciplinary team on the potential risk and the rational treatment strategy of patients with massive SFTP during the perioperative period.

Surgery for Solitary Fibrous Tumors of the Pleura: A Review of the Available Evidence.

Solitary fibrous tumors of the pleura (pSFT) are a relatively rare neoplasms that can arise from either visceral or parietal pleura and may have different aggressive biological behaviors. Surgery is well known to be the cornerstone of the treatment for pSFT. We reviewed the existing literature, focusing on the role of surgery in the management and treatment of pSFT. All English-written literature has been reviewed, focusing on those reporting on the perioperative management and postoperative outcomes. Surgery for pSFT is feasible and safe in all experiences reported in the literature, but surgical approaches and techniques may vary according to the tumor dimensions, localization, and surgeons' skills. Long-term outcomes are good, with a 10-year overall survival rate of more than 70% in most of the reported experiences; on the other hand, recurrence may happen in up to 17% of cases, which occurs mainly in the first two years after surgery, but case reports suggest the need for a longer follow-up to assess the risk of late recurrence. Malignant histology and dimensions are the most recognized risk factors for recurrence. Recurrence might be operated on in select patients. Surgery is the treatment of choice in pSFT, but a radical resection and a careful postoperative follow-up should be carried out.

Multiple solitary fibrous tumors of the pleura with multicentric and unilateral involvement: a case report

BackgroundSolitary fibrous tumor of the pleura (SFTP) is a mesenchymal tumor. Patients with SFTP generally have only one lesion. We herein report an extremely rare case of multiple SFTPs that were multicentric and unilateral.Case presentationThe patient was a 21-year-old asymptomatic young man who was referred to our hospital due to abnormal shadows on a chest X-ray. Computed tomography showed 6 tumors of heterogeneous sizes in the left thoracic cavity. The tumors were suspected to be multiple benign or low-grade malignant thoracic tumors, and tumor resection was performed. The tumors had almost the same appearance, with uniform fibroblastic spindle cell proliferation, and arose from the pleura in microscopy. Immunohistochemical staining revealed that the tumor cells were positive for CD34, CD99, Bcl-2, and STAT6. Based on these findings, the tumors were diagnosed as multiple SFTPs with multicentricity. At 1 year and 6 months after the first surgery, 2 new lesions were found above the diaphragm, and these were resected. These tumors were arose from the pleura with a fibrous capsule structure. Their pathological findings were identical to the initial tumor without evidence of malignant transformation.ConclusionWe experienced an extremely rare case of multiple SFTPs with multicentric and unilateral lesions.

Solitary fibrous tumor of pleura presenting as respiratory failure: unusual presentation of a rare tumor

Solitary fibrous tumors of the pleura (SFTPs) are rare primary pleural tumors derived from sub-mesothelial mesenchymal tissue that are mostly incidentally diagnosed in asymptomatic subjects. Symptomatic SFTPs usually present with dry cough and chest pain. We report the case of a 55-year-old lady with a 9-month history of shortness of breath, right-sided chest pain, and heaviness, who presented to our institute with hypercapnic respiratory failure and later required invasive mechanical ventilation. Subsequent radiological and histopathological investigations led to the diagnosis of a benign SFTP occupying almost the entire right hemithorax. She was treated with complete surgical resection by thoracotomy resulting in immediate resumption of adequate ventilation in the postoperative period. We emphasize the importance of large pleural tumors as a rare but reversible cause of hypercapnic respiratory failure.

Unexpected malignancy: resection of a large thoracic solitary fibrous tumour of the pleura with preoperative arterial emolisation.

Unexpected malignancy: resection of a large thoracic solitary fibrous tumour of the pleura with preoperative arterial emolisation.

June 2023 Medical Image of the Month: Solitary Fibrous Tumor of the Pleura

No abstract available. Article truncated after 150 words. An 85-year-old man presented to our institution for a second opinion and for management of a recurrent left-sided pleural effusion. The patient has a history of CLL, which was diagnosed with a chest wall biopsy 4 years prior to presentation. Since that time, he has undergone chemotherapy and had a good response. In the past 18 months, the patient has had their left-sided pleural effusion drained 24 times. The patient also has a history of hypothyroidism and has had a cholecystectomy. The patient brought multiple outside imaging studies with him for review. An image capture from a recent ultrasound-guided left thoracentesis (Figure 1A) demonstrated, in retrospect, a pedunculated left lower lobe mass. An outside PET-CT (Figure 1B) was also available, confirming the presence of this mass, which had relatively uniform, low level FDG uptake such that it evade notice on first interpretation. A CT angiogram (Figure 2) demonstrated a large …

190 - Solitary fibrous tumours of the pleura causing mass effect

190 - Solitary fibrous tumours of the pleura causing mass effect

Malignant Solitary Fibrous Tumours of the Pleura Are Not All the Same: Analysis of Long-Term Outcomes and Evaluation of Risk Stratification Models in a Large Single-Centre Series.

Introduction: Malignant solitary fibrous tumours of the pleura (mSFTP) are extremely rare diseases (<5% of all pleural neoplasms) with unpredictable behaviour. Surgery remains the standard of care for these tumours; however, estimating patient prognosis and planning follow-up remain challenging. Several risk stratification models have been proposed, but a classification with diagnostic and prognostic potential has not been well standardised yet. The aim of this study was to analyse the clinicopathological data of mSFTP to investigate their prognostic features and to compare the performance of three risk stratification models proposed in the literature. Methods: Observational retrospective cohort study on all proven cases of mSFTP surgically resected with radical intent between 2000 and 2019 in a single centre. Demographic, surgical and pathological data were examined. All patients were risk-stratified by using three prediction models: modified Demicco, De Perrot and Tapias. Overall survival (OS) and disease-free survival (DFS) were analysed. Results: There were 21 men and 13 women (median age, 67 years, range, 23-83 years). Twenty-one patients (62%) were symptomatic. The median follow-up was 111 months (range, 6-258 months). The 5-year OS and DFS were 81.2% and 77.4%, respectively. Nine patients (26.5%) experimented recurrences. At univariate analysis, the presence of necrosis (p = 0.019), nuclear atypia (p = 0.006), dimension greater than 11.5 cm (median value of our cohort) (p = 0.037) and relapse/disease progression (p = 0.001) were independent prognostic factor of worse OS. The administration of adjuvant treatment was a protective independent factor for survival (p = 0.001). Radicality of resection (p = 0.005); tumour dimension (p = 0.013), presence of necrosis (p = 0.041) and nuclear atypia (p = 0.007) and pleural pattern (p = 0.011) were independent prognostic factors of worse DFS. Analysing the three risk stratification models, the Tapias score was revealed as the best index to predict both OS (p = 0.002) and DFS (p = 0.047) in patients with mSFTP. Conclusions: Using the risk stratification model proposed by Tapias, patients with the highest risk of recurrence could be identified at the time of surgery to establish a more frequent imaging surveillance and longer follow-up. The role of adjuvant treatment in mSFTP therapy has not been established yet, but further analysis on patients with a high risk of recurrence, stratified according to risk models, along with biomolecular panels may tailor future post-surgical therapies.

An incidental finding ‒ Solitary fibrous tumor of the pleura

A 46-year-old female, with no previous history of respiratory complaints, was found to have a lesion in the left lower zone on chest X-ray during preoperative evaluation for undergoing hysterectomy in view of vaginal prolapse. Computed tomography (CT) of the chest showed a well-defined lobulated pleural-based lesion with smooth margins in the left lower lobe measuring 9.3 cm × 5.3 cm. She underwent CT-guided lung biopsy which showed a mesenchymal neoplasm. Hence, uniportal Video assisted thoracoscopic surgery (VATS) was done and a biopsy Histo-pathological examination (HPE) sent showed pleomorphic spindle cells with little cytoplasm and immunohistochemistry showed vimentin, CD34, STAT 6 positivity, and pan-cytokeratin negativity, consistent with solitary fibrous tumor of the pleura.

Solitary fibrous tumor of the pleura with paraneoplastic Doege-Potter syndrome

Solitary fibrous tumor of the pleura with paraneoplastic Doege-Potter syndrome

Giant solitary fibrous tumor of the pleura. A rare clinical case

Giant solitary fibrous tumor of the pleura. A rare clinical case

A case of slow-growing dedifferentiated solitary fibrous tumor of the pleura over a 7-year period

A case of slow-growing dedifferentiated solitary fibrous tumor of the pleura over a 7-year period

Trabectedin Is Active against Two Novel, Patient-Derived Solitary Fibrous Pleural Tumor Cell Lines and Synergizes with Ponatinib.

Solitary fibrous tumor of the pleura (SFT) is a rare disease. Besides surgery combined with radiotherapy in nondisseminated stages, curative options are currently absent. Out of fourteen primo-cell cultures, established from surgical SFT specimens, two showed stable in vitro growth. Both cell models harbored the characteristic NAB2-STAT6 fusion and were further investigated by different preclinical methods assessing cell viability, clone formation, and protein regulation upon single-drug treatment or in response to selected treatment combinations. Both fusion-positive cell models showed-in line with the clinical experience and the literature-a low to moderate response to most of the tested cytotoxic and targeted agents. However, the multi-tyrosine kinase inhibitors ponatinib and dasatinib, as well as the anti-sarcoma compound trabectedin, revealed promising activity against SFT growth. Furthermore, both cell models spontaneously presented strong FGFR downstream signaling targetable by ponatinib. Most interestingly, the combination of either ponatinib or dasatinib with trabectedin showed synergistic effects. In conclusion, this study identified novel trabectedin-based treatment combinations with clinically approved tyrosine kinase inhibitors, using two newly established NAB2-STAT6 fusion-positive cell models. These findings can be the basis for anti-SFT drug repurposing approaches in this rare and therapy-refractory disease.

Malignant solitary fibrous tumor of the pleura: A narrative review of clinical characteristics, diagnosis and therapeutic options

Solitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal neoplasm that originates from CD34+ dendritic stromal cells. While the majority of SFTPs are considered benign, an estimated 10%-20% are malignant. Malignant SFTP (MSFTP) is highly invasive and has propensities for local recurrence and distant metastasis. In recent years, an increasing number of MSFTPs have been reported. However, the diagnosis of MSFTP can be difficult owing to a lack of typical clinical manifestations and imaging features. To facilitate its diagnosis, the clinical, imaging, and histopathological characteristics of MSFTP are reviewed herein, followed by the clinical management such as surgery, radiotherapy, chemotherapy, targeted drugs and immunotherapy. Detection of NAB2-STAT6 fusion and expression of nuclear STAT6 can facilitate the diagnosis of SFTP. Furthermore, the recurrence risk stratification and prediction models of MSFTP as well as progress will be discussed. An improved understanding of these factors may contribute to more efficient diagnosis and management of MSFTP patients.