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Management of solitary fibrous tumours of the pleura: a systematic review and meta-analysis.

ABSTRACT: Background:Solitary fibrous tumours of the pleura (SFTP), or pleural fibromas, are rare tumours that generally, but not universally, follow a benign course. Surgical resection is the standard treatment, but there are no evidence-based guidelines regarding the management of these tumours. Methods:Five databases were searched from inception to April 1, 2019 for studies reporting on SFTP management. Results:Twenty-seven studies met the inclusion criteria (1542 patients, all non-comparative case series); 98% of these patients underwent resection and all SFTP included were pathologically diagnosed. 394 out of 1299 cases (30.5%, 95% CI 27.8-32.8%) were malignant with recurrence rates of between 0% and 42.9%. A pleural effusion was always associated with a negative outcome, but no other features were consistently reported to have negative associations. Preoperative biopsies incorrectly reported malignant histology in two studies. Over 25% of cases of recurrence occurred when a complete (R0) resection had been achieved. The first recurrence occurred >5?years after the initial resection in at least 23% of cases. Conclusions:There is strong evidence to support long-term surveillance after surgical resection of SFTP, even where a complete (R0) resection has been achieved; however, there is no clear evidence to inform clinicians regarding the selection of patients who should undergo resection. The rates of malignant SFTP and SFTP recurrence are higher than previously reported. Only those that were pathologically diagnosed or resected were included, which may bias the data towards more aggressive tumours. Data collection on radiologically diagnosed SFTP is required to draw conclusions regarding the timing and need for intervention.

SUBMITTER: Mercer RM

PROVIDER: S-EPMC7430150 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature

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Management of solitary fibrous tumours of the pleura: a systematic review and meta-analysis.

Mercer Rachel M RM Wigston Charlotte C Banka Radhika R Cardillo Giuseppe G Benamore Rachel R Nicholson Andrew G AG Asciak Rachelle R Hassan Maged M Hallifax Robert J RJ Wing Louise L Bedawi Eihab O EO Maskell Nick A NA Harriss Elinor K EK Miller Robert F RF Rahman Najib M NM

ERJ open research 20200701 3

<h4>Background</h4>Solitary fibrous tumours of the pleura (SFTP), or pleural fibromas, are rare tumours that generally, but not universally, follow a benign course. Surgical resection is the standard treatment, but there are no evidence-based guidelines regarding the management of these tumours.<h4>Methods</h4>Five databases were searched from inception to April 1, 2019 for studies reporting on SFTP management.<h4>Results</h4>Twenty-seven studies met the inclusion criteria (1542 patients, all no ...[more]

PMID: 32832532

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Project description:Simple Summary Solitary fibrous tumors are rare benign or cancerous tumors that develop in all tissues, including close to the spinal cord. These cases are exceptional and we describe their presentation and outcome based on 31 published cases and 10 patients on whom we operated. The tumors can develop in any portion of the spine and cause back pain, associated with neurological deficits, such as compression of a nerve or the spinal cord, in 66% of patients. Surgical removal is the first step towards diagnosis and treatment, but complete removal could be achieved in only 70% of patients, due to bleeding or spinal cord invasion. Tumors were found to recur after a mean 5.8 years (1 to 25), without identified risk factors. However, in patients with subtotal removal, radiotherapy significantly improves the rate of recurrence. In total, spinal solitary fibrous tumors are treated by neurosurgeons on the front line but discussion in a multidisciplinary team will provide general treatments, especially radiotherapy after subtotal removal. Abstract All solitary fibrous tumors (SFT), now histologically diagnosed by a positive nuclear STAT6 immunostaining, represent less than 2% of soft tissue sarcomas, with spinal SFT constituting a maximum of 2% of them, making these tumors extremely rare. We provide an up-to-date overview of their diagnosis, treatment, and prognosis. We included 10 primary STAT6-positive SFT from our retrospective cohort and 31 from a systematic review. Spinal pain was the most common symptom, in 69% of patients, and the only one in 34%, followed by spinal cord compression in 41%, radicular compression, including pain or deficit, in 36%, and urinary dysfunction specifically in 18%. Preoperative diagnosis was never obtained. Gross total resection was achieved in 71%, in the absence of spinal cord invasion or excessive bleeding. Histologically, they were 35% grade I, 25% grade II, and 40% grade III. Recurrence was observed in 43% after a mean 5.8 years (1 to 25). No significant risk factor was identified, but adjuvant radiotherapy improved the recurrence-free survival after subtotal resection. In conclusion, spinal SFT must be treated by neurosurgeons as part of a multidisciplinary team. Owing to their close relationship with the spinal cord, radiotherapy should be considered when gross total resection cannot be achieved, to lower the risk of recurrence.

Dataset Information

Management of solitary fibrous tumours of the pleura: a systematic review and meta-analysis.

Publications

Management of solitary fibrous tumours of the pleura: a systematic review and meta-analysis.

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