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Background: Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumors of the pleura (SFTPs). It is characterized by the presence of severe, sustained, and treatment-refractory hypoglycemia. Hypoglycaemia, which may be the sole symptom at disease onset, is mediated by the secretion of high-molecular-weight insulin-like growth factor (IGF-2). Most tumors exhibit benign behavior, with a 100% survival rate at 5 years. However, 10% of these tumors may display aggressive behavior with local or metastatic recurrence. We present a clinical case of a patient with a benign solitary fibrous tumor of the pleura who presented with symptomatic hypoglycemia and required pulmonary and pleural surgical resection to control the paraneoplastic phenomenon., Case Presentation: A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence., Conclusion: Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring., (© 2024. The Author(s).)

A woman in her 30s, non-smoker, presented at the emergency department two times because of spontaneous pneumothorax. The first episode was treated with small bore catheter drainage, while during the second episode-occurring only 1 week later-thoracoscopic talcage was attempted. The postoperative course was characterised by slow clinical and radiological resolution, and recurrence 3 days after discharge. Eventually, multiportal video-assisted thoracoscopic exploration identified an interfissural solid mass. Resection and further work-up revealed the diagnosis of 'low-risk' solitary fibrous tumour (SFT) stage pT1N0M0. The interdisciplinary tumour board advised no adjuvant therapy. A CT thorax was scheduled in 1 year for follow-up. The patient was discharged without complications and has had no recurrences of pneumothorax at 6 months of follow-up. This report shows that SFT can easily be missed on initial presentation and should be considered in the differential diagnosis of pneumothorax, especially when frequently recurring., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Refractory hypoglycaemia in a patient with a solitary fibrous tumour (SFT) is very rare and was first reported in 1930 independently by Doege and Potter, leading to it being named 'Doege-Potter syndrome'. Here, we report the unusual case of a 77-year-old woman with a giant solitary fibrous pleural tumour who presented with complicating pulmonary hypertension and associated heart failure with hypoglycaemia, and subsequently underwent curative resection of the pleural mass with clinical improvement., (© Royal College of Physicians 2023. All rights reserved.)

The article reports on three patients with a solitary fibrous tumor of the chest. The first patient had a tumor in the area of the dome of the right pleural cavity which was radically resected together with the chest wall around its origin. In the second case, the tumor was attached by a vascular pedicle to the lower lobe of the right lung. This tumor was resected atypically, via thoracotomy, along with a margin of healthy lung tissue at the base of its pedicle. The last patient had a tumor of the lower lobe of the right lung, surrounding the lower pulmonary vein, which did not have a clear margin of healthy lung tissue. This finding required right lower lobectomy via posterolateral thoracotomy. The presented cases describe rare types of tumors in the chest area which at the time of detection often reach large dimensions, necessitating extensive surgical procedures. Due to the biological nature of these tumors, long-term patient follow-up is advisable.

A woman in her early 70s was found to have incidental finger clubbing at a fracture clinic consultation for an unrelated problem. She reported no associated respiratory symptoms and was referred back to her General Practitioner for further investigation. A chest radiograph revealed a large left-sided mass. This was characterised as a pleural-based mass on CT, resulting in localised atelectasis and mediastinal shift. A CT guided biopsy revealed histology consistent with a solitary fibrous tumour of the pleura and the patient was referred for thoracotomy and resection., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Solitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal tumor that arises at various sites and typically originates from the pleura. Most patients with SFTPs are asymptomatic, unless the tumor is large. Approximately 20% of SFTP cases are malignant. There are few reports on imaging diagnoses and interventional treatments of SFTP. Here, we report a case of a giant SFTP that exhibited malignant behavior and underwent successful resection after embolization of the main supply artery of the tumor., Case Presentation: We report a clinical case of a giant SFTP in a 66-year-old Chinese female patient complaining of chest tightness and cough for more than 2 months. Ten years ago, the patient had undergone a chest CT scan at a local hospital for cough. Computed tomography (CT) had revealed a mass in the right thoracic region, which was misdiagnosed as a pulmonary abscess by CT-guided biopsy. Therefore, the patient did not receive appropriate/complete treatment at that time. She was hospitalized again, because CT showed significant enlargement of the right thoracic mass, which caused her obvious symptoms of discomfort. The pathological results of CT-guided biopsy at our hospital confirmed SFTP. Considering the large size of the tumor and the rich blood supply, some of the main blood vessels were treated with embolization before surgical resection. A large tumor, about 23 cm × 16 cm × 15 cm in size, was then successfully removed by thoracic surgery. The diagnosis of malignant SFTP was confirmed by surgical pathology and immunohistochemistry., Conclusion: Imaging findings of SFTPs are not characteristic, especially when a tumor is large, the diagnosis is difficult, and the final diagnosis still depends on histological and immunohistochemical examinations. The two-stage surgical treatment described here, which involves first embolization of the main supplying artery of the large tumor and then complete surgical resection, is effective and safe for SFTPs. Whether needle biopsy or vascular embolization is performed, intervention plays a crucial role in the diagnosis and treatment of patients with SFTPs., (© 2022. The Author(s).)

Complete surgical resection of a giant solitary fibrous tumour of the pleura can be challenging. We describe our technique for dealing with one such tumour. A clamshell incision proved inadequate for visualising the extent of the tumour. An additional lower sternotomy incision was then made, which significantly improved exposure. With the aid of cardiopulmonary bypass, it was then possible to mobilise and completely excise the tumour. Complete resection remains the mainstay of treatment for such pleural-based tumours. Every attempt must be made to achieve this, including any modification of the traditional approach if required.

An 84-year-old male presented with bloody stool. On digital rectal examination, a large and firm tumor was palpated in the anterior wall of the rectum at 2 cm from the anal verge. The colonoscopy revealed an ulcerated mass with smooth margins in the anterior wall of the rectum. Enhanced computed tomography showed a huge tumor in the pelvis, invading rectum, urinary bladder and the prostate, with signs of splenic and peritoneal metastases. Findings from an endoscopic biopsy and endoscopic ultrasound-guided fine needle aspiration suggested spindle cell carcinoma. We tentatively diagnosed as spindle cell carcinoma of the rectum and administered panitumumab as palliative chemotherapy. He eventually died at 4 months after the first visit to our institution. The autopsy findings resulted in the confirmed diagnosis as biphasic malignant peritoneal mesothelioma. There are only four previous reports on malignant peritoneal mesothelioma presenting as a colorectal tumor. Although rare, malignant peritoneal mesothelioma should be considered in differential diagnosis of colorectal tumors.

Introduction : The purpose of this paper is to study the type, the clinical presentation, and the best diagnostic methods for pleural solitary fibrous tumors (PSFTs), as well as to evaluate which is the most appropriate treatment, especially as PSFTs represent a rare occurrence in the thoracic pathology. Material and M ethod: A retrospective study was conducted on a group of 45 patients submitted to surgery between January 2015 and December 2019. In most cases, the diagnosis was established through imaging studies-thoracic computed tomography (CT) scan with or without contrast-but also using magnetic resonance imaging (MRI) or positron emission tomography (PET) scans when data from CT scans were scarce. All patients were submitted to surgery with curative intent. Results : Most patients included in this study were asymptomatic, with this pathology being more common in patients over 60 years of age, and more common in women. The occurrence of malignant PSFT in our study was 17.77% (8 cases). All cases were submitted to surgery with curative intent, with a single case developing further recurrence. In order to achieve complete resection en bloc resection of the tumor with the chest wall, resection was performed in two cases, while lower lobectomy, pneumectomy, and hemidiaphragm resection, respectively, were needed in each case. Postoperative mortality was null. Conclusion : Thoracic CT scan remains the most important imagistic investigation in diagnosing. MRI is superior to thoracic CT, especially in cases that involved the larger blood vessels within the thorax, spinal column, or diaphragm. Complete surgical resection is the gold standard in treatment of PSFT, and the prognosis in benign cases is very good., Competing Interests: The authors have no conflicts of interest to declare regarding this study.

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.

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., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Accurate distinction of benign mesothelial proliferations from malignant mesothelioma remains a diagnostic challenge. Sequential use of BAP1 immunohistochemistry and CDKN2A fluorescence in situ hybridization is specific for diagnosis of mesothelioma, but fluorescence in situ hybridization is both costly and time-consuming. Early data indicate that mesothelioma shows extensive loss of nuclear 5-hydroxymethylcytosine (5-hmC). We studied 49 cases of mesothelioma (17 epithelioid mesothelioma, 22 biphasic mesothelioma, and 10 sarcomatoid mesothelioma) and 23 benign mesothelial proliferations using a 5-hmC single immunohistochemical stain, CAM5.2/5-hmC double immunohistochemical stain, and BAP1 immunohistochemistry. Estimations of extent of 5-hmC loss were made using the 5-hmC single stain and CAM5.2/5-hmC double stain, and extent of nuclear 5-hmC loss was definitively quantitated in at least 1000 cells per case. Mean nuclear 5-hmC loss in mesothelioma (84%) was significantly greater than in benign mesothelial proliferations (4%) (p < 0.0001). Using 5-hmC loss in > 50% of tumor nuclei to define the diagnosis of mesothelioma, 5-hmC immunohistochemistry showed sensitivity of 92% and specificity of 100%. An immunopanel including 5-hmC and BAP1 immunohistochemistry achieved sensitivity of 98% and specificity of 100%. Extensive nuclear 5-hmC loss is sensitive and specific for mesothelioma in the differential diagnosis with benign mesothelial proliferations. In challenging mesothelial lesions, immunohistochemical studies showing either extensive 5-hmC loss or BAP1 loss indicate a diagnosis of mesothelioma, precluding the need for CDKN2A fluorescence in situ hybridization in a considerable number of cases.

Background: This 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)., Methods: A 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., Results: A 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., Conclusion: Larger 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., (© 2018 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd.)

Objectives: The separation of benign from malignant mesothelial proliferations and exact subclassification of mesothelioma subtypes is crucial to determining patient care and prognosis but morphologically can be very difficult., Methods: This session of the 2018 IMIG meeting addressed these problems., Results: A new immunohistochemical marker, methylthioadenosine phosphorylase, was shown to correlate well with CDKN2A FISH and is cheaper and faster to run. A 117 gene expression panel also provided good separation on both tissue biopsy and cytology samples. Review of a series of mesotheliomas thought to be biphasic produced only a moderate level of agreement among expert pathologists with some cases being classified as purely epithelioid or sarcomatoid; these classifications had prognostic significance. The entity called transitional mesothelioma was found to behave exactly like sarcomatoid mesothelioma. RNA-seq analysis of a large series of mesotheliomas from a public database showed that, genetically, the morphologic breakdown into epithelioid, sarcomatoid, or biphasic mesotheliomas is artificial because there is a continuous spectrum of genomic changes. There are now criteria for the diagnosis of mesothelioma in situ and this is potentially important, since such cases might be curable., Conclusions: This session documented new morphological and molecular approaches to separating benign from malignant mesothelial proliferations and to subclassifying malignant mesoteheliomas in clinical relevant ways., (Copyright © 2018 Elsevier B.V. All rights reserved.)

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., (Copyright © 2018 International Association for the Study of Lung Cancer. Published by Elsevier Inc. All rights reserved.)

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that was originally described to be localized in the pleura, but thereafter, this has been reported in several anatomic sites. Although the etiology of the neoplasm remains largely unknown, the pathogenesis seems to be related to an NAB2-STAT6 fusion gene due to paracentric inversion on chromosome 12q13. The diagnosis of extrapleural SFT is challenging, owing to its rarity, and requires an integrated approach that includes specific clinical, histological, immunohistochemical, and even molecular findings. Histologically, extrapleural SFT shares morphological features same as those of the pleural SFT because it is characterized by a patternless distribution of both oval- and spindle-shaped cells in a variable collagen stroma. In addition, morphological variants of mixoid, fat-forming, and giant cell-rich tumors are described. A correct diagnosis is mandatory for a proper therapy and management of the patients with extrapleural SFT, as extrapleural SFT is usually more aggressive than pleural form, particularly cases occurring in the mediastinum, retroperitoneum, pelvis, and meninges. Although SFT is usually considered as a clinically indolent neoplasm, the prognosis is substantially unpredictable and only partially related to morphological features. In this context, cellularity, neoplastic borders, cellular atypias, and mitotic activity can show a wide range of variability. We review extrapleural SFT by discussing diagnostic clues, differential diagnosis, recent molecular findings, and prognostic factors., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A solitary fibrous tumor originates in the pleura with variable degrees of invasion. Hypertrophic osteoarthropathy, known as Pierre-Marie-Bamberger syndrome, is characterized by clubbing of the fingers due to bone surface and soft tissue calcification, historically known as a bronchogenic carcinoma paraneoplastic syndrome; however, a few cases have been associated with solitary fibrous tumors. We describe the case of a 38-year-old woman who presented with clubbing of the fingers. Studies revealed an intrathoracic fibrous tumor that was successfully treated with improvement in symptoms.

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.

We describe a case of resection of a solitary fibrous tumour of the pleura using video-assisted thoracic surgery and removal of the giant tumour using a subxiphoid incision without the need for minithoracotomy. Use of the subxiphoid approach as a retrieval port is simple and feasible., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Solitary fibrous tumors of the pleura (SFTPs) are relatively rare tumors that originate from mesenchymal cells of submesothelial tissue of the pleura. Most patients with SFTPs are asymptomatic; however, pleuritic chest pain, cough, and dyspnea can develop. If hypoglycemia is associated with a solitary fibrous tumor, it is referred to as the Doege-Potter syndrome., Case Presentation: A 70-year-old man had visited our hospital with a chief complaint of dyspnea, and he was diagnosed as having a solitary fibrous tumor. A few years later, he developed hypoglycemia, and he underwent excision of the mass., Conclusion: Occasionally, SFTPs induce several paraneoplastic events, such as hypertrophic osteoarthropathy. We described here a patient with an SFTP with Doege-Potter syndrome who was successfully treated with complete resection. Although lesions can be histologically benign, they can clinically present with malignant features.

Solitary fibrous tumours (SFTs) are relatively rare neoplasms thought to originate from the submesothelial connective tissue. SFTs have been described in a variety of sites, including the pleura, orbit, lower respiratory tract, peritoneal cavity and heart. These neoplasms are usually benign, though locally aggressive, and metastatic behaviour has been observed in some cases. We describe a case of a 61-year-old man presenting with weight loss, poor appetite, malaise, worsening dyspnoea on exertion and lower extremity oedema, who was found to have a gigantic-21×21 cm-tumour occupying the entire right hemithorax causing compression and displacement of the mediastinum and liver. Transthoracic CT-guided biopsy revealed SFT of the pleura. The patient underwent preoperative angiography and embolisation of the tumour followed by successful surgical resection via thoracotomy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Solitary fibrous tumour of the pleura (SFTP) is a rare primary tumour of the pleura associated with 4% of cases with a paraneoplastic hypoglycaemia, termed Doege-Potter syndrome (DPS). We report a case of DPS presenting with severe coma in a 90-year-old woman. The cause was a malignant SFTP treated with surgical resection, from which the patient made a full recovery with prevention of recurrent hypoglycaemia. Surgical resection of the SFTP presenting with symptomatic hypoglycaemia should be considered even in elderly patients., (© The Author 2016. Published by Oxford University Press on behalf of the British Geriatrics Society. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Solitary fibrous tumors of the pleura (SFTP) are rare neoplasia of the chest. A subset of SFTP follows a malignant course, sometimes several years after complete resection. Traditional scoring systems based on clinical and histological features are poor predictors of biological behavior. This study aimed to investigate tumor-associated miRNAs expression as novel biomarkers to predict the clinical behavior of SFTP., Methods: Formalin-fixed and paraffin-embedded SFTP tissues blocks from patients surgically resected between 1992 and 2013 at two tertiary care teaching hospitals were included. SFTP tumors were categorized as either malignant or benign variants according to the WHO classification. Following miRNAs levels were measured: let-7a, miR-16b, miR-17, miR-21, miR-31, miR-34a, miR-92a, miR-125a, miR-125b, miR-195-5b, miR-203a, and miR-223. Differential gene expressions which were calculated with the threshold cycle (C t ) method were compared among the two variants., Results: Thirty-eight patients (40% male, mean age 62.2 (±10.9) years) were included. Expression levels of miR-125b showed a significant difference between benign compared to malignant variants (-3.08 ± 0.93 vs. -2.22 ± 1.36, p = 0.0068). Furthermore, lower levels of miR-125b were found to be associated with increased tumor size (p = 0.0414). Thus, downregulation of miR-125b indicates malignant transformation. All other investigated miRNAs were not associated with grading of SFTP., Conclusions: Our data suggest a potential role of miR-125b in the pathogenesis of tumor growth and malignant transformation of SFTP, respectively. Further studies have to address the potential use of miRNA-125b as a biomarker or therapeutic target in SFTP.

Doege-Potter syndrome is a paraneoplastic syndrome characterized by tumor-associated hypoglycemia secondary to a solitary fibrous tumor of the pleura. We present a case of an 84-year-old man, who presented with acute mental confusion and therapy-resistant hypoglycemia. Diagnostic imaging revealed a large sharply defined pleural tumor based on the left diaphragm, after surgical resection the diagnosis was made of a malignant solitary fibrous tumor of the pleura and restoration of the glucose homeostasis was observed.

Purpose: Solitary fibrous tumor (SFT) of the pleura is a rare mesenchymal neoplasm arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura and accounting for less than 5% of primary pleural tumors. SFTs are generally benign and asymptomatic, with 10-year survival rates of up to 98%. Unfortunately, approximately 10% have malignant potential, leading to local recurrence after radical surgery and/or metastatic spread. Of note, giant pleural SFT, which consists of a tumor occupying at least 40% of the affected hemithorax, is even less common with only anecdotal cases reported in the medical literature., Methods: We describe a unique case of giant SFT of the pleura that metastasized to the thyroid gland 1 year after complete resection, focusing on its clinical and pathological features of presentation., Results: En bloc resection remains the mainstay of therapy with curative intent. Patients with large tumors may undergo preoperative angiography with percutaneous embolization of the tumor, which allows to reduce perioperative bleeding. In case of local recurrence, surgery still remains the best treatment option. However, surgery can also be considered in patients with isolated metastatic spread., Conclusions: Every suspected and proven SFT of the pleura should undergo surgical resection, as clinical and radiological criteria cannot accurately distinguish benign from malignant forms. Moreover, the peculiar histological features of SFT should not be neglected when planning clinicoradiological follow-up. Additionally, suspicious clinical findings during follow-up should always be thoroughly investigated in order to exclude or confirm the diagnosis of recurrent disease.

Ectopic pleural thymoma is an exceedingly uncommon clinical entity that has only been described sporadically. Because of their peculiar location and variety of histologic patterns manifested, pleural thymomas may be confused with other neoplasms and may cause diagnostic problems clinically, radiologically, and morphologically. We describe the case of a giant left-sided ectopic pleural thymoma, preoperatively suspected to be a solitary fibrous tumor. A complete surgical resection was achieved and a postoperative diagnosis of WHO Type AB, modified Masaoka stage I tumor was attained. Subsequent thymectomy demonstrated unremarkable thymic tissue. The possibility of ectopic thymoma should be considered when the morphology of the lesion reveals a dual population of epithelial cells without significant nuclear atypia and lymphoid cells. Immunohistochemical studies are helpful in supporting the morphologic impression, both by characterizing the epithelial component as thymic in origin and by demonstrating the immature T-cell phenotype of the admixed reactive lymphocytes., (Copyright © 2016 Elsevier GmbH. All rights reserved.)

Solitary fibrous tumor (SFT) is a derived mesenchymal tumor from spindle cells, mostly occurred in the pleura. To analyze the clinical features of the SFT, data for a patient with SFT that involved in the pleura were retrospectively analyzed by assisted thoracoscope in the Affiliated Hospital of Zunyi Medical College in August 2015. The male patient was 45 years old, who showed the main clinical symptoms of chest pain, cough, sputum, and dyspnea. Large amount of right pleural effusion, chest space-occupying lesions were found by chest CT, suggesting a malignant tumor with metastasis at the 2nd and 3rd right rib. Immunohistochemical results showed: CD34 (+), cytokeratin (-), cytokeratin 5/6 (-), calretinin (-), epithelial membrane antigen(-), mesothelial cell (-), vimentin (++), Wilm's tumor-1 (+), Bcl-2 (+), CD56 (-), CD99 (+), desmin (-), and thyroid transcription factor-1 (-). It was diagnosed as SFT at right side wall layer pleura. SFT is a rare disease and it may occur at any site in the body. It lacks characteristic clinical symptoms and can be asymptomatic, or displays symptoms such as cough, chest pain, dyspnea, and hemoptysis. SFTs can only be conclusively diagnosed based on histopathologic and immunohistochemical characteristics of the tumor, and they are mostly benign. The main treatment for SFTs is the complete surgical resection. The prognosis for this disease is relatively good.

Background: A solitary fibrous tumor of the pleura is a rare but usually benign mesenchymal tumor arising from the pleura. Patients are often asymptomatic, resulting in the majority of tumors being detected incidentally on chest imaging. We present a case of a large solitary pleural tumor and review the typical radiographic and pathologic findings associated with this finding., Case Report: A 63-year-old white man with chronic obstructive pulmonary disease (COPD) was found to have a large pleural mass on chest radiography during a pre-operative assessment. The tumor was biopsied and findings were consistent with solitary fibrous tumor of the pleura., Conclusions: SFTPs are generally considered benign tumors although there is a risk of malignant transformation and recurrence. Imaging studies play an important role in identifying the tumor and planes of resection, and histologic diagnosis is critical in differentiating SFTP from other type of pleural masses. Surgical resection is main therapy of choice.

SFT is a rare spindle cell neoplasm arising mostly at pleural and in rare cases at extrapleural sites. Histology and immunohistology are diagnostic tools. It is crucial to consider SFT as differential diagnosis in pulmonary nodules since they often remain clinically silent until they reach large dimension and to proceed with curative resection without delay. It is essential to follow up patients for a long period of time as recurrence may occur.

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as a hypoinsulinaemic hypoglycaemia from the ectopic secretion of a prohormone of insulin-like growth factor II (IGF-II) from a solitary fibrous tumour. Surgical resection is curative in the majority of cases. If, however, the diagnosis is not suspected and treatment is delayed, it can lead to hypoxic cerebral injury or death. The underlying tumour can be a benign or malignant pleural tumour but may be present in extrapleural sites. For a diagnosis of Doege-Potter syndrome, symptoms attributable to hypoglycaemia and low blood glucose levels should be present along with the secretion of prohormone IGF-II. We report a case of severe hypoglycaemia in a 76-year-old inpatient admitted for resection of a recurrent left-sided pleural tumour. Investigation revealed true hypoglycaemia and Doege-Potter syndrome was diagnosed. The tumour was completely resected and the patient made a full recovery with no further hypoglycaemic episodes.

Solitary fibrous tumor of pleura (SFTP) is uncommon, accounts for less than 5% of all pleural tumors. Pulmonary artery aneurysm (PAA) is also not common, 80% of which often occurs in the main pulmonary trunk and peripheral PAA is rare. SFTP combined with PAA in one patient has not been reported. This paper reports a case of SFTP combined with peripheral PAA, and SFTP maybe accelerate PAA formation.

Background: The aim of this study was to evaluate the clinical characteristics and factors that influence the long-term outcomes of solitary fibrous tumors of the pleura., Methods: We conducted a retrospective study in 2 centers and reviewed 80 patients who underwent surgery between May 1984 and April 2011., Results: Of the 80 patients (29 male; median age, 60 years [33 to 85 years]), 47 were symptomatic (59%). The tumors originated from the visceral pleura in 62 cases (79%) and from the parietal pleura in 18 cases (22%). The tumors were pedunculated in 66 cases (83%) and sessile in 20 cases (17%). Surgical resection with histologically free margins was accomplished in 76 of 79 patients (93%). The tumors were classified as benign in 51 cases (65%) and as malignant in 28 (35%). The factors that were significantly associated with malignant tumors were the presence of symptoms (p = 0.03), a mean diameter 10 cm or greater (p = 0.0004), fibrous adherences (p = 0.003), pleural effusion (p = 0.003), and a Ki67 10% or greater (p = 0.003). The median follow-up was 69 months (range, 1 to 315). Local recurrence occurred in 3 cases. The overall 5- and 10-year survival rates were 90% and 86%, respectively, and the mean survival time was 255 ± 15 months. There were no differences between the benign and malignant tumors., Conclusions: The recurrence rates are low after surgeries for both benign and malignant solitary fibrous tumors of the pleura. However, the factors that are predictive of recurrence have yet to be specified and require additional immunohistochemical and genetic investigations., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Solitary fibrous tumors of the pleura (SFTPs) are infrequent neoplasms with no standardized criteria to predict risk of recurrence after curative surgery. The aim of the present study is to validate a recently proposed recurrence score in a large European cohort of patients with SFTP., Methods: Validation of a previously published scoring system was assessed in a population of 113 patients who underwent complete resection of SFTPs. Patients were scored according to the pleural origin, morphology, size, hypercellularity, presence of necrosis or hemorrhage, and number of mitoses in their SFTPs. Receiver operating characteristic curves were plotted for the score. Time to recurrence analysis was performed using the Kaplan-Meier and Cox proportional hazards methods., Results: After a mean follow-up of 13.2 ± 7.3 years, there were nine recurrences (8.0%). Score performance to predict recurrence was as follows: sensitivity = 78%, specificity = 74%, positive likelihood ratio = 3.0, and negative likelihood ratio = 0.3. A cutoff of 3 points was used to classify 79 patients (69.9%) at low risk and 34 patients (30.1%) at high risk for recurrence. A high-risk classification was significantly associated with more recurrences during follow-up (P = .004), worse overall survival (P = .0008), more extensive lung resections (P = .001), and the use of adjuvant therapies (P = .009). The present score outperformed England's criteria (P = .049) and de Perrot classification (P < .001) when predicting SFTP recurrence., Conclusions: The proposed scoring system, which combines common clinical and histologic features of resected SFTPs, remains predictive of recurrence in a separate patient population. The simple score may guide the postoperative surveillance of this uncommon tumor.