Paraneoplastic syndrome associate with solitary fibrous tumor of pleura

Karki, Apurwa; Yang, John; Chauhan, S.

doi:10.4103/lungindia.lungindia_118_17

Cited by 8 publications

(5 citation statements)

References 16 publications

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“…Hypertrophic osteoarthropathy, known as Pierre-Marie-Bamberger syndrome, is characterized by clubbing of the fingers or toes caused by calcification of the bone surface and soft tissue. Also, the increase of human beta chorionic gonadotropin-releasing factor leads to gynecomastia (26), and cerebellar degeneration is also occasionally seen (27). Distant metastasis of SFTP is very rare, but it has been reported in the pancreas, lung, and thyroid (28,29).…”

Section: Discussionmentioning

confidence: 99%

Rapidly growing solitary fibrous tumors of the pleura: a case report and review of the literature

Zuo¹,

Zhou²,

Sun³

et al. 2020

Ann Transl Med

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A solitary fibrous tumor (SFT) is a rare spindle cell tumor-derived from mesenchymal cells. It may be linked to the fusion of the NAB2-STAT6 gene caused by 12q chromosome rearrangement. It can occur in the connective tissue of any part of the body; however, it is most common in the pleura. Solitary fibrous tumors of the pleura (SFTP) are a persistent painless mass with slow growth. With the increase of the tumor, there will be corresponding compression symptoms. Pleural effusion is rare, and the cytology of pleural effusion is mostly negative. Occasionally, SFTP can induce paraneoplastic syndrome, distant metastasis, and malignant transformation. Lung function may have mild to moderate restrictive ventilation dysfunction. CT is a crucial method for the clinical diagnosis of SFTP. The histopathological features of SFTP are the coexistence of sparse and dense areas. CD34, CD99, Bcl-2, and vimentin are the most valuable immunohistochemical markers.The positive expression rate of STAT6 in benign SFT was even 100%. Adhesion or unclear boundary with surrounding tissues, pleural effusion or calcification, tumors with a maximum diameter greater than 10 cm, invasive growth, uneven density, metastasis or recurrence, paraneoplastic syndrome, moderate to severe cell heterogeneity, high Ki67 proliferation index, and low STAT6 expression suggest SFTP may be a malignant tumor. Gene analysis on next generation sequencing may help reveal the mutation characteristics of SFTP. Complete tumor resection is the gold standard of SFTP. Resectability is the most important prognostic factor. Age, size, mitosis, and necrosis are considered risk stratification factors for prognosis. Fortunately, 80% of SFTP are benign and have anexcellentprognosis but need long-term follow-up.We report a case of rapidly growing tumor with pleural effusion within 9 months, who was surgically treated and is currently under follow-up. And the literature is reviewed.

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Section: Discussionmentioning

confidence: 99%

Rapidly growing solitary fibrous tumors of the pleura: a case report and review of the literature

Zuo¹,

Zhou²,

Sun³

et al. 2020

Ann Transl Med

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“…PSFTs are in most cases asymptomatic, but with their growth, they can lead to compression of the nearby organs (lungs, heart, large vessels, and thoracic wall), associated with a diversity of clinical symptoms [9,27,28]. In our case, the proportion of symptomatic and asymptomatic patients was almost equal, with 21 of them (46.66%) being symptomatic and 24 of them (53.33%) being asymptomatic.…”

Section: Discussionmentioning

confidence: 53%

Pleural Solitary Fibrous Tumors—A Retrospective Study on 45 Patients

et al. 2020

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Introduction: The purpose of this paper is to study the type, the clinical presentation, and the best diagnostic methods for pleural solitary fibrous tumors (PSFTs), as well as to evaluate which is the most appropriate treatment, especially as PSFTs represent a rare occurrence in the thoracic pathology. Material and Method: A retrospective study was conducted on a group of 45 patients submitted to surgery between January 2015 and December 2019. In most cases, the diagnosis was established through imaging studies—thoracic computed tomography (CT) scan with or without contrast—but also using magnetic resonance imaging (MRI) or positron emission tomography (PET) scans when data from CT scans were scarce. All patients were submitted to surgery with curative intent. Results: Most patients included in this study were asymptomatic, with this pathology being more common in patients over 60 years of age, and more common in women. The occurrence of malignant PSFT in our study was 17.77% (8 cases). All cases were submitted to surgery with curative intent, with a single case developing further recurrence. In order to achieve complete resection en bloc resection of the tumor with the chest wall, resection was performed in two cases, while lower lobectomy, pneumectomy, and hemidiaphragm resection, respectively, were needed in each case. Postoperative mortality was null. Conclusion: Thoracic CT scan remains the most important imagistic investigation in diagnosing. MRI is superior to thoracic CT, especially in cases that involved the larger blood vessels within the thorax, spinal column, or diaphragm. Complete surgical resection is the gold standard in treatment of PSFT, and the prognosis in benign cases is very good.

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“…Las lesiones grandes pueden provocar compresión de estructuras vecinas y el paciente puede presentar algunos síntomas tales como dolor torácico, tos y disnea 10 . Algunos pacientes debutan con los llamados síndromes paraneoplásicos que incluyen hipoglicemia refractaria y osteoartropatía pulmonar hipertrófica, los cuales mejoran inmediatamente tras la resección del tumor 11 .…”

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Tumor fibroso solitario gigante de pleura

Palenzuela

García

2022

Rev. Cirugia

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Objetivo: Reportar el caso de una masa gigante en hemitórax izquierdo de 19 cm de diámetro en un paciente de 59 años que debutó con disnea, tos y dolor torácico, confirmándose por estudio imagenológico. Material y Método: Registro clínico de un paciente al cual se le diagnostica tumor fibroso solitario de pleura, siendo intervenido quirúrgicamente para exéresis de la lesión. Resultados: Se realiza toracotomía posterolateral izquierda para exéresis de tumor gigante, requiriendo además resección de diafragma y pericardiectomía parcial con evolución favorable. Discusión: El tumor fibroso solitario es una neoplasia rara derivada del mesénquima que afecta más comúnmente a la pleura, típicamente bien circunscrita, pediculada, con vasos dentro del pedículo tumoral, pudiendo llegar a ser de gran tamaño, siendo considerados gigantes cuando tienen más de 15cm de diámetro. Conclusión: El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica es potencialmente curable. El tratamiento quirúrgico puede efectuarse por toracotomía o videotoracoscopia, dependiendo del tamaño del tumor. A pesar del comportamiento benigno, requiere seguimiento a largo plazo debido a la tendencia a la recidiva.

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Paraneoplastic syndrome associate with solitary fibrous tumor of pleura

Cited by 8 publications

References 16 publications

Rapidly growing solitary fibrous tumors of the pleura: a case report and review of the literature

Rapidly growing solitary fibrous tumors of the pleura: a case report and review of the literature

Pleural Solitary Fibrous Tumors—A Retrospective Study on 45 Patients

Tumor fibroso solitario gigante de pleura

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