Papillary Solitary Fibrous Tumor/Hemangiopericytoma: An Uncommon Morphological Form With NAB2-STAT6 Gene Fusion

Yao, Zhigang; Wu, Haibo; Hao, Yinghua; Wang, Xingfu; Ma, Genshan; Li, Jia; Li, Jifeng; Chun-hu, Lin; Zhong, Xiuming; Wang, Zhou; Wang, Daizhong

doi:10.1093/jnen/nlz053

Cited by 8 publications

(5 citation statements)

References 25 publications

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“…We included 10 cases from our own retrospective cohort and retrieved 31 cases from the systematic review, as detailed in the Supplementary Flow Chart ( Supplementary Figure S1 ) [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ]. All were primary SFT with a STAT6-positive pathological diagnosis.…”

Section: Resultsmentioning

confidence: 99%

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Apra

Arbi

Montero

et al. 2022

Cancers

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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.

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Section: Resultsmentioning

confidence: 99%

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Apra

Arbi

Montero

et al. 2022

Cancers

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“…SFT has many similar phenotypes with hemangiopericytomas. Specifically, CD-34, CD-99, Bcl-2, and STNT6 are considered as markers of both tumors ( 4 ). STF usually occurs in the pleura.…”

Section: Discussionmentioning

confidence: 99%

“…STF usually occurs in the pleura. Most SFTs progress slowly with relatively good prognosis, but malignant cases occur in 10% of all cases ( 4 ).The probability of SFTs occurring in the kidney is low, but owing to its rarity, the process of diagnosis and treatment is challenging ( 5 ). Some recorded cases show that the pathognomonic gene fusion between NAB2 and STAT6 is highly sensitive and specific for SFTs ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

“…More clinical experiments need to be carried out to find the optimal treatment for each group. According to statistics, SFTs in meninges with NAB2: exon 6—STAT6 have higher recurrence rates (50%) compared with SFTs with NAB2: exon 4—STAT6 (27%) ( 4 ), but the influence of NAB2: exon 6—STAT6 in kidney SFTs on recurrence is still unknown. Due to the lack of a large number of valid data, more related studies are needed to further confirm the fusion mutation site of NAB2: exon 6—STAT6: exon 16 that led to STAT6 in this case.…”

Section: Discussionmentioning

confidence: 99%

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Case report: Solitary fibrous tumor of the kidney with a NAB2-STAT6 fusion gene

Hu²,

Wang³

2022

Front. Oncol.

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BackgroundSolitary fibrous tumor (SFT) is a rare spindle cell neoplasm that mostly originates from the pleura, and accounts for only 2% of all soft tissue tumors. Moreover, the cases of SFT of the kidney are rarely reported. Here, we report a typical case of kidney SFT, which was consistent with other reported cases. This case further expands on existing diagnostic methods of SFT and explains the importance of STAT6 mutations in SFT.Case SummaryWe report a typical case of SFT of the kidney. A 34-year-old woman presented to the urinary surgery department after physical examinations were suggestive of a urologic neoplasm. Further relevant imaging investigations suggested a renal tumor with benign behaviors. The patient was diagnosed with a kidney tumor suspected to be SFT and underwent laparoscopic radical left nephrectomy. Postoperative pathological immunohistochemical tests showed positivity for Signal Transducer and Activator of Transcription 6(STAT6), CD-34, CD-99, and Bcl-2, thus confirming the diagnosis of SFT. Combined with the results of genetic testing of the patient, the tumor was indicated to carry NGFI-A-Binding protein 2(NAB2): exon 6—STAT6: exon 16 mutation sites, which confirmed our diagnosis. The patient recovered quickly without any clinical evidence of incomplete resection. She has been followed-up for more than a year and will continue to be reviewed every three months to observe the final outcomes.ConclusionSolitary fibrous tumor is difficult to differentiate from other renal tumors. CT imaging, STAT6 immunostaining and gene profiling are valid investigations to establish the diagnosis.

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“…SFTs, in particular, can be graded as 1, 2, or 3. Other histologic variants of SFT have also been described in the literature at various sites, including the fat-forming variant that has previously been identified in the orbit [ 11 ], a papillary variant [ 19 ], and a giant cell-rich variant [ 20 ]. The differential diagnosis includes fibrous meningioma, schwannomas, and poorly differentiated synovial sarcoma [ 11 , 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical, Pathologic, and Radiologic Features of Orbital Solitary Fibrous Tumors and Meningiomas

Williams¹,

Ahmad²,

Chin³

et al. 2021

Cureus

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A wide variety of benign and malignant tumors can arise from different structures in the orbital and peri-orbital area, affecting the eye and the optic nerve. This spectrum of tumors includes primary and metastatic carcinomas, lymphomas, melanomas, soft tissue tumors, and primary tumors of the retina, optic disc, and optic nerve. These also extend to relatively rare entities such as solitary fibrous tumor and meningioma of the orbit and optic nerve, which can present with very similar clinical and radiologic features, although the tumor grades, treatment plans, and outcomes can vary widely. In this report, we present two clinical cases of solitary fibrous tumor [central nervous system (CNS) World Health Organization (WHO) grade 2 and 3) and compare their clinical presentation, radiologic and histologic features, treatment, and clinical outcomes to a group of three orbital meningiomas (CNS WHO grade 1 and 2). In the context of these five cases of orbital lesions, we review the current clinical, pathologic, and radiologic literature on orbital tumors, focusing primarily on solitary fibrous tumors and meningiomas, along with an expanded discussion on the diagnostic criteria of both entities, as well as the treatment and prognosis of these lesions.

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Papillary Solitary Fibrous Tumor/Hemangiopericytoma: An Uncommon Morphological Form With NAB2-STAT6 Gene Fusion

Cited by 8 publications

References 25 publications

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Case report: Solitary fibrous tumor of the kidney with a NAB2-STAT6 fusion gene

Clinical, Pathologic, and Radiologic Features of Orbital Solitary Fibrous Tumors and Meningiomas

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