Uncommon presentation of a rare tumour - incidental finding in an asymptomatic patient: case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours
| Autor: | Dávid Becker, Hajnalka Vágó, Ibolya Csecs, Miklós Pólos, Béla Merkely, E Bartha, Attila Tóth, Nikolette Szücs, Zoltán Sápi, Pál Maurovich-Horvat, Csilla Czimbalmos, Zoltán Szabolcs |
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| Rok vydání: | 2017 |
| Předmět: |
Adult
Cancer Research Pathology medicine.medical_specialty Solitary fibrous tumor Solitary fibrous tumour CD99 CD34 Case Report 030204 cardiovascular system & hematology lcsh:RC254-282 Pericardial effusion Asymptomatic Pericardial Effusion Multimodality imaging Long term follow-up Heart Neoplasms 03 medical and health sciences 0302 clinical medicine Genetics medicine Humans Pericardium medicine.diagnostic_test business.industry Intrapericardial localization Magnetic resonance imaging lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens Image Enhancement medicine.disease Magnetic Resonance Imaging medicine.anatomical_structure Oncology Ventricle Solitary Fibrous Tumors 030220 oncology & carcinogenesis Female Radiology Neoplasm Recurrence Local medicine.symptom Tomography X-Ray Computed business |
| Zdroj: | BMC Cancer BMC Cancer, Vol 17, Iss 1, Pp 1-8 (2017) |
| ISSN: | 1471-2407 |
| DOI: | 10.1186/s12885-017-3574-0 |
| Popis: | Background A 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 presentation A 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. Conclusion Intrapericardial 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. Electronic supplementary material The online version of this article (10.1186/s12885-017-3574-0) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
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