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Extremely rare case of intravascular solitary fibrous tumour in the inferior vena cava with review of the literature.

ABSTRACT: 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.

SUBMITTER: Fan J

PROVIDER: S-EPMC6686241 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Extremely rare case of intravascular solitary fibrous tumour in the inferior vena cava with review of the literature.

Fan Jingyuan J Qiu Jinfeng J Wei Qingjun Q

Diagnostic pathology 20190807 1

<h4>Background</h4>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.<h4>Case presentation</h4>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 ve ...[more]

PMID: 31391089

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Project description:Introduction Kidney and liver cysts in autosomal dominant polycystic kidney disease (ADPKD) can compress the inferior vena cava (IVC), but IVC compression prevalence and its risk factors are unknown. Methods Patients who have ADPKD (n = 216) with abdominal magnetic resonance imaging (MRI) studies and age-/sex-matched controls (n = 216) were evaluated for IVC compression as well as azygous vein diameter (a marker of collateral blood flow) and IVC aspect ratio (left-to-right dimension divided by anterior-to-posterior dimension with a value of 1 corresponding to a circular (high pressure) IVC caudal to compression. Results Severe IVC compression (?70%) was observed in 33 (15%) ADPKD subjects and mild compression (?50% to <70%) was observed in 33 (15%) subjects; whereas controls had no IVC compression (P < 0.001). Severe IVC compression was associated with larger azygous vein (4.0 ± 1.3 mm versus 2.3 ± 0.8 mm without IVC compression; P < 0.001) and a more circular IVC cross-section upstream (mean IVC aspect ratio: 1.16 ± 0.27 vs. 1.69 ± 0.67, P < 0.001), suggesting higher pressure upstream from the compression. IVC compression was associated with older age, lower estimated glomerular filtration rate (eGFR), greater height-adjusted total kidney volumes, greater height-adjusted liver volume (ht-LV), and greater liver and renal cyst fractions (P < 0.001). No subject younger than 30 years had IVC compression, but ADPKD subjects ?40 years old had 12-fold higher risk of IVC compression (95% confidence interval [CI]: 4.2–42.4), with highest predicted probability for Mayo Clinic classes 1D (59%; 95% CI: 39%–76%) and 1E (74%; 95% CI: 49%–90%) after adjustment (P < 0.001). Women with ht-LV ? 2000 ml/m had 83% (95% CI: 59%–95%) prevalence of IVC compression. Complications of IVC compression included deep vein thrombosis (DVT) and symptomatic hypotension. Conclusions IVC compression is common in ADPKD patients >40 years old, with Mayo Clinic class 1D/E, and in females with ht-LV > 2000 ml/m. Graphical abstract

Dataset Information

Extremely rare case of intravascular solitary fibrous tumour in the inferior vena cava with review of the literature.

Publications

Extremely rare case of intravascular solitary fibrous tumour in the inferior vena cava with review of the literature.

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