Malignant Solitary Fibrous Tumor Of The Pleura Research Articles

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.

P36.02 A Case of Recurrent Giant Malignant Solitary Fibrous Tumour of the Pleura: An Elephant in the Room?

Solitary fibrous tumor of the pleura (SFTP) is a rare soft tissue neoplasm, accounting for 4% of chest tumors. The biological behavior of SFTP is generally benign, with 10-15% of SFTP that show malignant features with unpredictable long-term behavior. A 59-year-old Caucasian woman with no history of previous malignancies or comorbidities arrived at our institution with cough, increasing dyspnoea, and chest pain. A chest CT scan showed a giant intra-thoracic mass (25x16x16 cm) partially occupying the right hemithorax (fig.1A). Total body 18F-FDG/PET-CT showed a very inhomogeneous mass pattern, with necrotic areas and areas with various patterns of enhancement. A transthoracic FNAB revealed pulmonary SFTP. After cardio-respiratory complete assessment and tumor board evaluation, the patient underwent surgical excision of the mass en-bloc with right upper lobe by thoracotomy. Microscopically, the tumor showed solid spindle cells with high mitotic activity (5 mitoses/10 HPF), high Ki67 expression, and CD34, bcl2, and STAT6 positive. According to England’s criteria, the diagnosis was indicative of a giant malignant SFTP, and the tumor had a Tapias score of 5, which indicated a high risk of recurrence. The postoperative course was uneventful, and the patient was discharged on 8th postoperative day. Due to R0 resection, no adjuvant radiotherapy or chemotherapy was administered, and a strict follow-up was conducted. After 72 months of follow-up, the patient experimented local pleural recurrences (fig.1B) traded with surgical excision in re-thoracotomy. The histopathological analysis showed eight lesions with a median diameter of 1.5 cm, microscopically composed by spindle cells with 10 mitoses/10 HPF, STAT6+, CD34+. Although R0 resection was achieved, adjuvant chemotherapy was administered. A genome sequencing with the next generation approach was performed on the primary lesion and the recurrent disease. SFTP is a rare neoplasm with unpredictable biological behavior. The surgical resection represents the gold standard if complete resection can be reached. The recently introduced Tapias score can be helpful to assess the risk of recurrence: a strict and long-term follow-up should be conducted in case of a high score (>3). In case of local relapse, surgical excision remains the best therapeutic option.

Solitary Fibrous Tumors of the Chest: An Analysis of Fifty Patients.

BackgroundA solitary fibrous tumor of the chest (SFTC) is a subtype of solitary fibrous tumor (SFT) with a low incidence rate. The purpose of this study is to analyze the diagnosis and treatment of SFTC and the difference between benign and malignant solitary fibrous tumor of the pleura (SFTP) to improve the understanding of this rare disease.MethodsA retrospective analysis of fifty patients with SFTC (33 cases in the pleura and 17 in the lung) was performed. Clinical and imaging characteristics, pathological features, and treatment follow-up outcomes were analyzed.ResultsThe common symptoms of the 50 patients included a cough, expectoration, chest tightness, fever, and chest pain. Space occupying lesions were found via plain computed tomography (CT) and enhanced CT was used for enhancement of the tumors. It was also found that 18 cases had necrosis, and 5 cases had calcification. The histopathology results showed that frequent nuclear division, obvious morphological variation, necrosis, and the high expression of Ki-67 cells are markers of malignant SFTC. There were significant differences in age, chest tightness, necrotic foci in CT, and expression of Ki-67 between the benign and malignant SFTP cases. All the patients who received treatment were given an excellent prognosis.ConclusionA combination of enhanced CT, histopathology, and immunohistochemistry can be used for the accurate diagnosis of SFTC. Advanced age, chest tightness, necrotic foci in CT, and a high Ki-67 index were more likely to be malignant SFTP. Operation and radiofrequency ablation can provide favorable outcomes for both benign and malignant SFTC.

Solitary fibrous tumors of the pleura: A single center experience at National Cancer Center, China.

BackgroundThis study explored the clinicopathological features, predictive factors of malignancy, effectiveness of video assisted thoracic surgery (VATS), and prognosis of solitary fibrous tumor of the pleura (SFTP).MethodsA single‐center retrospective study of the data of 82 patients with SFTP who were surgically treated in our department between January 2003 and December 2015 was conducted.ResultsA total of 82 SFTPs (70 benign, 12 malignant) were included and all patients underwent complete en bloc resection. SFTPs originated from the visceral pleura in 47 (57%) and the parietal pleura in 35 (43%) patients. In our cohort, malignant tumors were often symptomatically large, and the patients with malignant SFTPs (mSFTPs) often had a family history of neoplasms. Patients in the VATS group (n = 22) had tumors with significantly smaller diameters, required a shorter surgical duration and shorter hospital stay, and experienced less intraoperative blood loss and less postoperative chest tube drainage compared to the thoracotomy group (n = 60). No tumor recurrence was found in benign SFTP (bSFTP) patients. The long term survival and disease‐free survival rates of mSFTP patients were 76% and 53%, respectively.ConclusionLarger tumor diameter and a family history of neoplasm may be predictive factors for mSFTP; however, this conclusion needs to be verified in large cohort. VATS is safe and reliable for treating selected SFTP patients. Local recurrence is associated with mSFTP patient death, thus close follow‐up of such patients is crucial.

Surgical therapy and next-generation sequencing-based genetic alteration analysis of malignant solitary fibrous tumor of the pleura.

BackgroundSolitary fibrous tumor of the pleura (SFTP) is a rare pleural neoplasm arising from mesenchymal cells, accounting for <5% of pleural neoplasms. Approximately 10% of cases of SFTP demonstrate malignant potential, leading to local recurrence after radical surgery and subsequent metastasis.MethodsA large malignant-like mass was found in the left thoracic cavity of a 61-year-old woman. Following radical resection of the mass, the patient was diagnosed with malignant SFTP by histologic and immunohistochemical analyses. In addition, a next-generation sequencing-based mutation test was used to reveal the mutational profile of the tumor. The genetic alteration panel was analyzed with reference to public data on the ClinVar and COSMIC databases, after which the public SFTP data were analyzed for frequency of altered genes. Finally, through overlay of the abovementioned two sets, the genetic alteration accounting for SFTP initiation was anticipated to be identified.ResultsIn the mutation panel of our malignant SFTP group, kinase insert domain receptor (KDR) and fms-related tyrosine kinase 1 (FLT1) scored high in pathogenesis but had only a medium frequency; the NAB2–STAT6 fusion appeared to be the dominant genetic alteration in public SFTP samples.ConclusionThe high frequency of NAB2–STAT6 fusion indicates its prominent role in SFTP, while somatic mutations such as FLT1-R593W and KDR-V297I may also contribute to the malignant angiogenic phenotype. The present study affirmed the heterogeneity of SFTP, and more sophisticated classification methods will be needed to explore its underlying mechanisms.SummaryWe believe that improvement in the prognosis of SFTP relies on early diagnosis, margin-free resection, and long-term follow-up. Through genetic analysis, it appears that both NAB2–STAT6 fusion and somatic mutations such as FLT1-R593W and KDR-V297I contribute to SFTP development.

Solitary Fibrous Tumors of the Pleura of the Thorax: CT and FDG PET Characteristics in a Tertiary Referral Center.

This study was conducted to describe the characteristics of a solitary fibrous tumor of the pleura (SFTP) on chest CT, and FDG PET. Furthermore, we analyze the prognosis of SFTP using large data confirmed in a tertiary referral hospital.From January 1997 to March 2012, 41 patients (21 males and 20 females; median age: 59 yr; age range: 27–85 yr) who were pathologically diagnosed with SFTP were consecutively examined. The CT findings, including the size, shape, homogeneity, and anatomic location (chest wall, intrapulmonary/fissure space, diaphragm, and mediastinum) of the SFTP, the 18F-fluorodeoxyglucose positron emission tomography (FDG PET) findings, and the histopathology findings were evaluated.Most of the patients had a mass-type (70.7%), oval/elliptical (80.5%), and homogeneous (70.7%) SFTP with a median diameter of 6.0 cm (range: 1–17). The most common anatomic location was the chest wall (43.9%), followed by the intrapulmonary/fissure space (22.0%), diaphragm (22.0%), and mediastinum (12.3%). For all the 9 patients, the mean maxSUV was 2.9 (SD = 1.16; range: 1.2–4.9) on FDG PET. The malignant SFTP (median: 3.6, range: 2.5–4.9) showed more hypermetabolic than benign SFTP (median: 2.0, range: 1.2–3.1) (P = 0.049).Through familiarity with the various features of the SFTP with regard to its size and location on the preoperative CT and FDG PET, we can add this rare pleural neoplasm to the differential diagnosis of other more common conditions. Moreover, an appropriate treatment choice can be made.

Giant solitary fibrous tumor of the pleura requiring left pneumonectomy.

A 57-year-old man was referred to our department from another hospital after major bleeding following thoracoscopic biopsy of an undiagnosed giant mass in the posterior mediastinum. The bleed had been controlled by emergency posterolateral thoracotomy and diagnosis of low grade malignant solitary fibrous tumor of the pleura (SFTP) then obtained, but radical resection was not performed. A chest computed tomography (CT) scan showed a large hypervascular posterolateral mediastinal mass adherent to the mediastinal pleura, the left lung, the thoracic aorta, and close to the esophagus (Figs 1, ​,22). Figure 1 Axial post-contrast computed tomography (CT) images at four different levels of the chest (shown here at the mediastinum window level), disclosed a large left-posterior mediastinal lesion characterized by many intralesional and surrounding vessels, strictly ... Figure 2 Sagittal reformatted computed tomography (CT) image shows the craniocaudal extension of the mass and its extensive contact with the thoracic aorta. We performed redo posterolateral thoracotomy. The mass was then dissected and effectively separated from the esophagus and pericardium. However, the mediastinal pleura of the left lung was widely infiltrated and intrapericardial left pneumonectomy was required to achieve radical en bloc resection (Fig 3). No residual disease was observed at the end of the procedure. Figure 3 Surgical specimen disclosing the radically resected solitary fibrous tumor of the pleura involving the mediastinal surface of the left lung completely resected en bloc by left intrapericardial pneumonectomy. Histological examination confirmed malignant SFTP CD 34 and CD 99 positive, cytokeratin negative, weighing 985 grams without the left lung. Postoperative paramedian left vocal cord paralysis resulting in moderate dysphonia was documented by video laryngoscopy and a speech therapy program was instituted. The patient was discharged two weeks after surgery without any major complication. Two years of follow up, performed by chest CT scan every four months, did not disclose any local or distant relapse. SFTP is a rare, slow-growing tumor whose origin is widely recognized as the mesenchymal cells of submesothelial tissues of the pleura, rather than mesothelial cells.1 The tumor tends to grow into a huge mass before local compression symptoms develop. Angiography or CT scan frequently discloses multiple feeding vessels, including several intercostal, internal mammary, inferior phrenic, and bronchial arteries.2 The ideal treatment of SFTP is based on radical surgical excision that is generally curative in all benign cases and in approximately half of malignant ones.1 Complete resection of giant SFTP may be challenging because of poor exposure, significant blood supply, strong adhesions, and often the direct involvement of nearby structures, however, there is little evidence for alternative management of this particular tumor (radiotherapy and chemotherapy).3 The prognosis for patients with benign SFTP is generally favorable with a five-year overall survival rate ranging from 79 to 100%, however, the risk of recurrence is high after the resection of a malignant sessile SFTP, although long-term survival is possible, ranging from 46 to 100%.4 Adequate pre-operative blood storage is recommended in cases of planned giant SFTP resection, together with a detailed explanation to acquaint patients with details of the therapeutic schedule, including major lung and mediastinal resection.

Diagnostic pitfalls in the preoperative 18F-FDG PET/CT evaluation of a case of giant malignant solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura (SFTP) is an uncommon entity, generally with an indolent behavior. Nevertheless, some malignant forms have been rarely reported. These, often have an aggressive biological behavior with pathological findings of invasiveness. The preoperative diagnosis and evaluation of the grade of malignancy are extremely challenging. Herein we report a case of a 64-year-old man who presented with a left giant intra-thoracic mass imaged with fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG/PET-CT) and sampled via fine-needle aspiration biopsy (FNAB). Imaging and FNAB findings showed suspicion of a benign form of SFTP. Surgical radical resection of the giant mass was performed. The definitive histological diagnosis showed a malignant SFTP. Based on this report, we take the opportunity to briefly discuss the insidious pitfalls concerning the radiological and (18)F-FDG/PET-CT features as well as cyto/histological findings in the pre-operative diagnostic work-up examination of this rare entity.

Solitary fibrous tumors of the pleura

Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm of the pleura. Although an increasing number of case series have been reported in recent years, so far clinical behavior is still unpredictable. The following article presents a detailed review of the recent larger series of SFTP with particular attention to the clinical presentation, pathologic characteristics and surgical features in order to define more precisely the long-term outcome after treatment of this rare entity. Usually, after a complete surgical resection a very long survival could be expected in almost all cases. However, the preoperative detection and management of malignant SFTPs are still challenging issues. SFTP remains an enigmatic tumor. Clinical and radiological assessment is often unsatisfactory to obtain a definitive diagnosis. Benign SFTPs are almost always cured with complete surgical resection, and in cases of recurrence the re-do resection of benign recurrences is usually curative. Long-term survivals are also possible for malignant SFTPs, although a higher recurrence rate and higher tumor-related mortality can be expected in these cases. Histologic characteristics distinguishing benign from malignant variants are crucial in estimating the risk of recurrence and planning adjuvant therapies.

Malignant Solitary Fibrous Tumor of Pleura Accompanied with First Symptoms of Chest Pain and Hemoptysis: A Case Report

Solitary fibrous tumor of the pleura (SFTP) is a rare tumor especially presents malignant features. Such symptoms of hemoptysis and dyspnea were rarely seen and take 5% and 4% respectively in malignant SFTP. A 26-year-old Chinese man, presenting with hemoptysis in the emergency room, was hospitalized because of dyspnea. The X-ray examination revealed a tumor in the right chest cavity. The patient refused treatment, and the tumor grew rapidly, which complicated the symptoms of the patient. En-bloc excision of tumor plus the involved lung was performed. There was at least a 5000-ml mixture of blood and tumor tissue in the right chest cavity because of continuous bleeding, leading to a tumor capsule split. Histopathology and immunohistochemistry identified the tumor as malignant SFTP, but CD34 was negative. In this case, the tumor grew rapidly and aggressively in two months, indicating that close follow-up and active treatment are needed.

Contralateral recurrence of a malignant solitary fibrous tumor of the pleura

We present an unusual case of a contralateral recurrence of malignant solitary fibrous tumor of the pleura (SFTP) nine years after a complete resection. Recurrence of malignant SFTP has already been described, but is usually localized. In our case the patient underwent surgical resection for a malignant SFTP of the left upper lobe in 2000. Nine years later computed tomography (CT)-scans showed lesions that were suspicious of tumor recurrence in the right lung. Thoracoscopy, wedge-resections and pathological findings revealed four nodules of a malignant SFTP of the right middle and lower lobe, histopathologically identical to the tumor, which had been resected nine years ago. A coincidental mucinous bronchioloalveolar carcinoma of the left lower lobe was resected by thoracotomy. To our knowledge this is the first report of contralateral recurrence of a malignant SFTP years after complete resection in the literature. The possibility of a new primary tumor on the right with local metastasis could not be excluded in the clinical and histopathological examinations. Therefore, contralateral recurrence of malignant SFTP should be considered in the postoperative follow-up even years after complete resection.

Hypoglycemia in a patient with a huge malignant solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura (SFTP) is rare. A 73-year-old man, without past history of diabetes mellitus, was admitted to hospital with profound hypoglycemia, loss of consciousness and respiratory failure. CT indicated a huge heterogenous mass lesion (12 x 12 x 17 cm) at the right thoracic cavity with total atelectasis of the right lower lung. He underwent a standard thoracotomy with complete excision of the tumor. Pathology indicated malignant SFTP with insulin-like growth factor-binding protein-2 production. There were no further incidences of hypoglycemia or respiratory failure after excision of the tumor. Hypoglycemia resulting from a solitary fibrous tumor is uncommon. Standard thoracotomy and complete resection of this giant tumor provided a good result for the present patient.

Solitary Fibrous Tumor of the Pleura

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%.