A rare presentation of a solitary fibrous tumour.
Ontology highlight
ABSTRACT:
SUBMITTER: Costa GNF
PROVIDER: S-EPMC10140206 | biostudies-literature | 2023 May
REPOSITORIES: biostudies-literature
Publications
A rare presentation of a solitary fibrous tumour.
Costa Gonçalo N Ferraz GNF Franco Fátima F Teixeira Rogério P C RPC
Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation 20230405 5
Similar Datasets
Project description:BackgroundA solitary fibrous tumour is a rare, mainly benign spindle cell mesenchymal tumour most commonly originating from the pleura. An intrapericardial location of a solitary fibrous tumour is extremely unusual. We present a case of an asymptomatic patient with a slow-growing massive benign cardiac solitary fibrous tumour.Case presentationA 37-year-old asymptomatic female patient was referred to our hospital with an enlarged cardiac silhouette found on her screening chest X-ray. The echocardiographic examination revealed pericardial effusion and an inhomogeneous mobile mass located in the pericardial sac around the left ventricle. Cardiac magnetic resonance (MRI) examination showed an intrapericardial, semilunar-shaped mass attached to the pulmonary trunk with an intermediate signal intensity on proton density-weighted images and high signal intensity on T2-weighted spectral fat saturation inversion recovery images. First-pass perfusion and early and late gadolinium-enhanced images showed a vascularized mass with septated, patchy, inhomogeneous late enhancement. Coronary computed tomography angiography revealed no invasion of the coronaries. Based on the retrospectively analysed screening chest X-rays, the mass had started to form at least 7 years earlier. Complete resection of the tumour with partial resection of the pulmonary trunk was performed. Histological evaluation of the septated, cystic mass revealed tumour cells forming an irregular patternless pattern; immunohistochemically, the cells tested positive for vimentin, CD34, CD99 and STAT6 but negative for keratin (AE1-AE3), CD31 and S100. Thus, the diagnosis of an intrapericardial solitary fibrous tumour was established. There has been no recurrence for 3 years based on the regular MRI follow-up.ConclusionIntrapericardial SFTs, showing slow growth dynamics, can present with massive extent even in completely asymptomatic patients. MRI is exceedingly useful for characterizing intrapericardial masses, allowing precise surgical planning, and is reliable for long-term follow up.
| S-EPMC5581469 | biostudies-literature
Project description:BackgroundSolitary fibrous tumour (SFT) is a rare mesenchymal malignancy that lacks robust prognostic and predictive biomarkers. Interferon-stimulated gene 15 (ISG15) is a ubiquitin-like modifier, associated with tumour progression, and with poor survival of SFT patients, as previous published by our group. Here, we describe the role of ISG15 in the biology of this rare tumour.MethodsISG15 expression was assessed by immunohistochemistry in tissue microarrays from SFT patients and tested for correlation with progression-free survival and overall survival (OS). The effects of ISG15 knockdown or induction were investigated for cancer stem cell (CSC) characteristics and for drug sensitivity in unique in vitro models of SFT.ResultsThe prognostic value of ISG15 for OS was validated at protein level in malignant SFT patients, prospectively treated with pazopanib and enrolled in GEIS-32 trial. In SFT in vitro models, ISG15 knockdown lead to a decrease in the expression of CSC-related genes, including SOX2, NANOG, ALDH1A1, ABCB1 and ABCC1. Likewise, ISG15 downregulation decreased the clonogenic/ tumoursphere-forming ability of SFT cells, while enhancing apoptotic cell death after doxorubicin, pazopanib or trabectedin treatment in 3D cell cultures. The regulation of CSC-related genes by ISG15 was confirmed after inducing its expression with interferon-β1; ISG15 induction upregulated 1.28- to 451-fold the expression of CSC-associated genes.ConclusionsISG15 is a prognostic factor in malignant SFT, regulating the expression of CSC-related genes and CSCs maintenance. Our results suggest that ISG15 could be a novel therapeutic target in SFT, which could improve the efficacy of the currently available treatments.
| S-EPMC9304060 | biostudies-literature
Project description:BACKGROUND:Solitary fibrous tumour (SFT) is a mesenchymal tumour of fibroblastic type, and it develops in almost any part of the human body. However, according to previous studies, the occurrence of intravascular SFTs is extremely rare. CASE PRESENTATION:We reported a case of intravascular SFT in a 67-year-old woman who has been experiencing swelling and pain in the right leg for 2?months. Computed tomography venography scan revealed a well-defined mass obstructing the inferior vena cava (IVC). Surgical resection was performed, and histopathologic and immunohistochemical results were consistent with SFT. Further, next-generation sequencing (NGS) analysis was performed, and results revealed two tumour-related gene mutations (deletion of PMS2 and variation of ESR1 [L536P]). The patient did not receive any adjuvant therapy, and no signs of tumour progression were observed during the 6-month follow-up. CONCLUSION:To the best of our knowledge, this study first presented about SFT arising from the IVC and carried out an NGS analysis to validate the molecular mechanism of such condition.
| S-EPMC6686241 | biostudies-literature
Project description:BackgroundIntracranial solitary fibrous tumour (iSFT) is an exceptional mesenchymal tumour with high recurrence rates. We aimed to analyse the clinical outcomes of newly diagnosed and recurrent iSFTs.MethodsWe carried out a French retrospective multicentre (n = 16) study of histologically proven iSFT cases. Univariate and multivariate Cox models were used to estimate the prognosis value of the age, location, size, WHO grade, and surgical extent on overall survival (OS), progression-free survival (PFS), and local recurrence-free survival (LRFS).ResultsEighty-eight patients were included with a median age of 54.5 years. New iSFT cases were treated with gross tumour resection (GTR) (n = 75) or subtotal resection (STR) (n = 9) and postoperative radiotherapy (PORT) (n = 32, 57%). The median follow-up time was 7 years. The median OS, PFS, and LRFS were 13 years, 7 years, and 7 years, respectively. Forty-two patients experienced recurrence. Extracranial metastasis occurred in 16 patients. Median OS and PFS after the first recurrence were 6 years and 15.4 months, respectively. A higher histological grade was a prognosis factor for PFS (p = 0.04) and LRFS (p = 0.03). GTR influenced LRFS (p = 0.03).ConclusionGTR provided benefits as a first treatment for iSFTs. However, approximately 40% of patients experienced relapse, which remains a challenging state.
| S-EPMC9913492 | biostudies-literature
Project description:Introduction and importanceA solitary fibrous tumour (SFT) is a rare neoplasm that commonly arises in the pleura and can occur in other extrathoracic sites. Extrapleural SFT, particularly in the sinonasal cavity, is extremely rare. There are no definite diagnostic criteria for sinonasal SFT as it is rare. Histologic analysis with immunohistochemistry plays an important role in diagnosing SFT.Case presentationWe report herein a case of SFT of the sinonasal cavity, which later spread to the oral cavity in a 67-year-old male with underlying papillary thyroid carcinoma (PTC) stage IV. He complained of recurrent epistaxis from a mass in his left nasal cavity for two weeks. The mass grew bigger, and spread to the oral cavity, causing dysphagia and upper airway obstruction. Tracheostomy was done under local anaesthesia and a biopsy of the mass was taken to rule out metastasis from the PTC. However, histopathological examination revealed a mesenchymal tumour of fibroblastic type, consistent with an SFT. He was planned for surgical resection of the tumour. However, he refused the operation and was lost to follow-up.Clinical discussionWe describe the clinical presentation of this rare tumour of the sinonasal and oral cavity, including upper airway obstruction, and the importance of immunohistochemical markers such as CD34 and BCL-2 in diagnosing SFT. Complete resection of the tumour is the definitive treatment for SFT.ConclusionSFT of the sinonasal and oral cavity is extremely rare. Upper airway obstruction may occur due to the location of the tumour in the airway region. Immunohistochemistry is crucial to distinguish this tumour from other mesenchymal tumours.
| S-EPMC8609029 | biostudies-literature
Project description:Solitary Fibrous Tumors (SFT) is a relatively rare occupying lesion, which often originated from the pleura and has been reported in a series of reports. However, there are few reports on retroperitoneal SFT. To better understand its biological behavior, clinical diagnosis and follow-up strategy, this case studied the manifestations, diagnosis and treatment of retroperitoneal SFT in a 57-year-old woman, and reviewed the relevant literature.
| S-EPMC6796658 | biostudies-literature
Project description:Solitary fibrous tumors (SFT) is a rare type of soft tissue tumors and there have been only a few reported cases of SFTs at the perineum region. We report a SFT below the pubic bone in a 35-year-old male causing compression on his corpus spongiosum. The patient underwent en bloc tumor resection with preservation of spermatic vessels and the bladder neck. Pathological evaluation and immunohistochemistry (IHC) staining with CD34(+), CD99(+), STAT6(+), S100(-) and Desmin(-) confirmed the diagnosis of SFT. Surgery plays a key role in treatment strategies and pathological examination with IHC is important in the diagnosis of SFTs.
| S-EPMC7005559 | biostudies-literature
Project description: Not available
| S-EPMC8078432 | biostudies-literature
Project description:This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base.
| S-EPMC4899415 | biostudies-literature
Project description:The expression of TFE3 (transcription factor E3) in solitary fibrous tumours (SFTs) and their histologic mimickers was investigated, and the diagnostic value and clinical significance of TFE3 nuclear expression in SFTs were explored. Immunohistochemical analysis for TFE3 was performed on 50 cases of SFTs that were surgically resected. The controls were sample tissues from malignant peripheral nerve sheath tumour, synovial sarcoma, dedifferentiated liposarcoma, spindle cell lipoma, and dermatofibrosarcoma protuberans. The survival of patients with TFE3-positive and TFE3-negative expressions was assessed through the Kaplan-Meier analysis. In 44 of 50 (88%) SFTs, nuclear immunoreactivity for TFE3 was detected. The TFE3 expression was negative in all samples of synovial sarcoma, malignant peripheral nerve sheath tumour, dermatofibrosarcoma protuberans, and spindle cell lipoma and weakly positive in 2 of 10 cases of dedifferentiated liposarcoma. Fluorescence in situ hybridization (FISH) confirmed that the expression of the TFE3 protein is not caused by gene translocation. There was no statistical significance between the association of the TFE3 expression and SFT patient prognosis. Therefore, TFE3 is capable of enhancing the differential diagnosis of SFTs and their histologic mimickers and can be potentially used as a diagnostic marker. The findings also offer valuable insights into SFT diagnosis, aetiology, and associated molecular mechanisms.
| S-EPMC7275214 | biostudies-literature