A rare clinical case – Solitary fibrous tumor of the pleura

Papadopoulos, Alexis; Porfyridis, Ilias; Christodoulides, Gregorios; Georgiou, Andreas

doi:10.1016/j.rmcr.2015.07.006

Cited by 5 publications

(9 citation statements)

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“…SFTP accounts for 5% of all tumors of the pleura, and is mainly benign. Malignant solitary fibrous tumor of the pleura (MSFTP) is relatively rare and constitute ~20% of all reported SFTPs 8. In this report we present the case of a female patient with a malignant pleural tumor invading adjacent mediastinal structures.…”

Section: Introductionmentioning

confidence: 94%

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

Song¹,

Yang²,

Zhang³

et al. 2018

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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.

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Section: Introductionmentioning

confidence: 94%

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

Song¹,

Yang²,

Zhang³

et al. 2018

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“…SFTP is a very rare tumor [1] , [4] , which has gained appropriate recognition in the last two decades as a discrete pathologic entity [9] . It represents 5% of the tumors of the pleura.…”

Section: Discussionmentioning

confidence: 99%

“…As far as the pleural solitary fibrous tumors are concerned, about 800 cases of SFTP have been described in the literature. Historically several terms have been used to describe this tumor, such as benign mesothelioma, localized mesothelioma, localized fibrous mesothelioma, localized fibrous tumor of the pleura, sub-pleural fibroma, pleural fibroma, localized benign fibroma, and sub-mesothelial fibroma [1] , [4] , [5] . The first description of the tumor is chronologically debatable.…”

Section: Introductionmentioning

confidence: 99%

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Solitary fibrous tumor: A center's experience and an overview of the symptomatology, the diagnostic and therapeutic procedures of this rare tumor

Hohenforst-Schmidt

Grapatsas

Dahm

et al. 2017

Respiratory Medicine Case Reports

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Solitary Fibrous Tumor of the Pleura (SFTP) is a rare tumor of the pleura. Worldwide about 800 patients diagnosed with this oncological entity have been described in the existing literature. We report our center's 13 year experience. During this time three patients suffering from this rare disease have been treated in our department. All patients were asymptomatic and their diagnosis was initially triggered by a random finding in a routine chest x-ray. The diagnosis was set preoperatively through a needle biopsy under computer tomography (CT) guidance. The tumors were resected surgically though video-assisted thoracoscopic surgery (VATS) or thoracotomy. Because of the lack of specific guidelines due to the rarity of the disease a long-term, systematic follow-up was recommended and performed. Parallel an overview of the diagnostic and therapeutic procedures of the rare tumor is made.

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“…Localised malignant pleural mesothelioma (LMPM) is a rare tumour,2–4 which usually appears as solitary circumscribed nodule or mass attached, either in a sessile manner or pedunculated manner to the surface of the pleura 5. LMPM should be distinguished from diffuse malignant pleural mesotheliomas, because a good prognosis may be obtained by surgical resection 2 6. Diagnostic imaging involves: standard X-rays, CT, MRI and positron emission tomography (PET).…”

Section: Introductionmentioning

confidence: 99%

Rare presentation of a localised malignant pleural mesothelioma with cranial metastasis

2016

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Mesothelioma is an uncommon malignant neoplasm and a localised form of the pleura is especially very rare. Diagnosis of localised malignant pleural mesothelioma (LMPM) is very challenging. Histopathological verification is the gold standard, and studies such as CT, positron emission tomography (PET) and thoracoscopy are very valuable tools in assisting diagnosis. We report a case of histopathologically proven LMPM, which was discovered as a well circumscribed solitary subpleural nodule on PET-CT after presentation with cranial metastasis. This case shows that LMPM can present with uncommon radiological and clinical appearances, and imaging tools such as PET-CT have a very important role in diagnosis.

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A rare clinical case – Solitary fibrous tumor of the pleura

Cited by 5 publications

References 9 publications

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

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

Solitary fibrous tumor: A center's experience and an overview of the symptomatology, the diagnostic and therapeutic procedures of this rare tumor

Rare presentation of a localised malignant pleural mesothelioma with cranial metastasis

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