Benign Solitary Fibrous Tumors Of The Pleura Research Articles

Doege-Potter syndrome due to a solitary fibrous tumor of the pleura: a case report

BackgroundDoege-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 presentationA 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.ConclusionDoege-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.

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.

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

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). 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. 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. 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.

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.

The utility of 18F-FDG PET/CT in solitary fibrous tumors of the pleura

ObjectiveTo demonstrate the utility of 18F-FDG PET/CT in the differentiation of benign and malignant solitary fibrous tumors of the pleura (SFTP). Materials and methodsA retrospective review was performed on the 18F-FDG PET/CT data from 17 patients with histopathologically diagnosed benign or malignant SFTP. The size, side of SFTP, presence of necrosis, calcification, pleural effusion, hilar lymphadenopathy (LAP), density on CT images (Hounsfield unit-HU), and 18F-FDG uptake (SUVmax) were recorded and compared in order to detect malignant SFTP. Statistical significance was set as p<0.05. ResultsThe difference in size, presence of necrosis, and hilar LAP on CT images were statistically significant (p=0.004, p<0.001, p=0.015, respectively) in a comparison of benign and malignant SFTPs. The mean HU of benign SFTP was 46.16±5.52HU, and for malignant SFTP it was 35.03±4.61HU (p=0.003). The mean SUVmax was 3.02±1.02 for benign SFTP and 4.89±2.12 for malignant SFTP (p=0.021). A cut-off value of ≥7cm for size, ≤39.81HU for density, and ≥3.47 for SUVmax was obtained by ROC analysis for detecting malignant SFTP. Conclusions18F-FDG PET/CT may have a limited role in diagnosing malignant SFTP in suspected patients.

An unusual pathology with an undefined etiology:solitary fibrous tumors of the pleura.

The aim of the present study was to evaluate the etiology and clinical and pathological behavior of solitary fibrous tumors of the pleura (SFTPs), as well as the most appropriate surgical approach and the results of long-term follow-up of this condition. Clinical and long-term follow-up records of 14 patients who had surgery for SFTP between 2001 and 2014 were reviewed retrospectively. Etiological factors, diagnostic procedures, and clinical courses and outcomes for these patients were studied. Of the 14 patients, 8 were male (57%) and 6 were female (43%) patients. The mean age was 54.14 ± 10.35 (41-75) years. There was no remarkable common etiological factor. Preoperative diagnosis was achieved only in 2 patients. Predominant symptoms were cough and chest pain. Complete resection was achieved in all patients. Video-assisted thoracic surgery (VATS) was performed in 8 patients. All but one patient were classified as having benign SFTP. The mean follow-up was 58.5 ± 41.4 (10-132) months and no recurrence was noted in the follow-up. These rarely seen tumors of the pleura are usually benign and asymptomatic and their preoperative diagnosis is difficult. Clinical and pathological behavior is still unpredictable and the treatment consists of complete resection. Minimally invasive techniques such as VATS are recommended for surgery if the tumor size is appropriate.

The utility of 18F-FDG PET/CT in solitary fibrous tumors of the pleura.

To demonstrate the utility of (18)F-FDG PET/CT in the differentiation of benign and malignant solitary fibrous tumors of the pleura (SFTP). A retrospective review was performed on the (18)F-FDG PET/CT data from 17 patients with histopathologically diagnosed benign or malignant SFTP. The size, side of SFTP, presence of necrosis, calcification, pleural effusion, hilar lymphadenopathy (LAP), density on CT images (Hounsfield unit-HU), and (18)F-FDG uptake (SUVmax) were recorded and compared in order to detect malignant SFTP. Statistical significance was set as p<0.05. The difference in size, presence of necrosis, and hilar LAP on CT images were statistically significant (p=0.004, p<0.001, p=0.015, respectively) in a comparison of benign and malignant SFTPs. The mean HU of benign SFTP was 46.16±5.52HU, and for malignant SFTP it was 35.03±4.61HU (p=0.003). The mean SUVmax was 3.02±1.02 for benign SFTP and 4.89±2.12 for malignant SFTP (p=0.021). A cut-off value of ≥7cm for size, ≤39.81HU for density, and ≥3.47 for SUVmax was obtained by ROC analysis for detecting malignant SFTP. (18)F-FDG PET/CT may have a limited role in diagnosing malignant SFTP in suspected patients.

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.

Radiology–Pathology Conference: 18F FDG PET-CT imaging of solitary fibrous tumor of the pleura

Solitary fibrous tumors of the pleura (SFTPs) are mesenchymal neoplasms that arise from the pleura. SFTPs can grow slowly and may become big masses with malignant behaviors and metastases. The ratio of benign to malignant is 7:1, and pathology is necessary to differentiate between benign and malignant. We present a case of a 37-year-old man with a presumptive diagnosis of benign SFTP by 18F-fluorodeoxyglucose positron emission tomography/computed tomography, which was confirmed by pathology.

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 the pleura: computed tomography-pathological correlation and comparison with computed tomography of benign solitary fibrous tumor of the pleura

The aim of this study was to determine the computed tomography (CT)-pathological correlation of malignant solitary fibrous tumors of the pleura (MSFP) and to compare these findings with CT findings of benign solitary fibrous tumors of the pleura (BSFTP). We retrospectively identified the clinical, CT, and pathological findings in seven cases of MSFP. There was a CT-pathological correlation for the MSFPs. Additionally, 12 cases of BSFTP from case files were compared with the clinical and CT features of the MSFPs. On CT, the MSFP appeared as a heterogeneously enhancing mass >10 cm (100%). Pleural metastasis (57.1%) and lung metastasis (14.3%) were associated. In the CT-pathological correlation, the enhancing area was mixed cellular and collagenous tissue with hypercellularity, mitosis, and pleomorphism. Hemorrhage, necrosis, cystic, or myxoid degeneration produced areas of intratumoral low attenuation. MSFPs showed a higher incidence of intratumoral low-attenuation areas (P = 0.034) and pleural metastasis (P = 0.009); and on CT, MSFPs tended to be larger than BSFTPs (P = 0.076). MSFPs showed a >10 cm pleural mass with low-attenuation regions on CT, which corresponded to hemorrhage, necrosis, cystic, or myxoid degeneration. MSFPs had a higher incidence of intratumoral low-attenuation areas and pleural metastasis, and on CT they tended to be larger than BSFTPs.

Video-Assisted Thoracoscopic Surgery (VATS) for Patients with Solitary Fibrous Tumors of the Pleura

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. 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). 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. 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 with a minute malignant component and diaphragmatic vascular supply: Report of a case

We resected a fist-sized, solitary fibrous tumor of the pleura (SFTP) with a minute malignant component, following percutaneous embolization of its feeding artery. The tumor had macroscopic characteristics of a benign SFTP, and most parts of it were microscopically benign. However, further careful pathological examination revealed a minute malignant component in its periphery. We report this case to show that large and mostly benign SFTPs may contain malignant components, which can be overlooked. Thus, large SFTPs should be resected in consideration of this possibility.

A Case of a Solitary Fibrous Tumor of the Pleura Presenting as Pneumonia and Acute Respiratory Failure

Solitary fibrous tumors of the pleura (SFTPs) are relatively rare tumors that originate from the mesenchymal cells of the submesothelial tissue of the pleura. Patients with SFTPs are often asymptomatic, while some patients experience pleuritic chest pain, cough and/or dyspnea. We report here on a case of SFTP, and the patient presented with septic shock and respiratory failure that required mechanical ventilation. A 68-year-old woman was admitted for the evaluation of her dyspnea and generalized edema. Chest imaging studies showed an 18 cm-sized voluminous mass occupying the right thoracic cavity with anterior displacement of hilar structures and atelectasis of the right lung. Immediately after admission, she developed pneumonia and septic shock that required antibiotics and mechanical ventilation. She displayed a partial response to medical treatment, and then complete excision of the tumor was performed and the pathologic examination revealed benign SFTP. Afterward, she fully improved without evidence of recurrence until now.