Validation of nuclear STAT6 immunostaining as a diagnostic marker of meningeal solitary fibrous tumor (SFT)/hemangiopericytoma

Berghoff, Anna Sophie; Kresl, Philip; Bieńkowski, Michał; Koelsche, Christian; Rajky, Ursula; Hainfellner, Johannes A.; Preusser, Matthias

doi:10.5414/np300993

Cited by 23 publications

(13 citation statements)

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“…Literature from the past 5 years was searched, and we found that STAT6 immunohistochemistry has high specificity for SFT and that the diagnostic value of strong and diffuse nuclear expression of STAT6 in SFT has been proven. [18][19][20] It was also found that STAT6 is a dependable marker to differentiate SFT from myofibromas. 21 At the same time, follicular lymphomaassociated STAT6 mutations facilitate nuclear residency of STAT6, 22 while nuclear localization of STAT6 is also dramatically increased in Kaposi's sarcoma tissue.…”

Section: Discussionmentioning

confidence: 95%

A case of solitary fibrous tumor/hemangiopericytoma in the central nervous system with papillary morphology

et al. 2019

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This report describes the clinicopathological findings of a solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) of the central nervous system in a 59‐year‐old man with space‐occupying lesions on both the left anterior basicranial and on the top tail of falx cerebri. The tumor showed small quantities of solid papillary areas and a prominent papillary structure, where atypical cells were compactly arranged along the fibrovascular core. The tumor cells of both components showed nuclear relocalization of the signal transducer and activator of transcription 6 protein, with very high specificity and sensitivity for the diagnosis of SFT/HPC. In the literature, only three cases of SFTs with a papillary pattern have been reported, but this case showed a complete papillary pattern. “Papillary” SFT/HPC is a rare morphological variant of SFTs/HPCs, and its differential diagnosis among intracranial tumors is an important factor which clinicians should bear in mind during diagnosis.

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Section: Discussionmentioning

confidence: 95%

A case of solitary fibrous tumor/hemangiopericytoma in the central nervous system with papillary morphology

et al. 2019

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“…Sarcomatoid carcinomas typically show positivity for CK [7], which was not the case in our patient. Absence of STAT-6 goes against a diagnosis of meningeal hemangiopericytoma [8]. Chromogranin and synaptophysin studies which are usually positive in paragangliomas were not available; however, imaging was not suggestive of that [9].…”

Section: Discussionmentioning

confidence: 99%

Malignant Epithelioid Neoplasm of the Brain

Jog¹,

Ronderos²,

Ali

et al. 2020

Cureus

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Malignant epithelioid tumors have been described in various organ systems, but are rarely seen in the brain. They are aggressive tumors and have high mortality. In certain cases, the immunohistochemistry (IHC) findings may not be sufficient to clarify the diagnosis. In these cases, next-generation genetic sequencing may play a role in clarifying the diagnosis. In addition to lab testing, a thorough history and physical exam are necessary to rule out other sources of the tumor such as melanoma. Patients presenting with neurological symptoms are cared for by a wide variety of physicians, hence it is important to raise awareness of rare tumors in order to provide timely and appropriate management and referral for these patients. We present the case of a middle-aged woman who was diagnosed with a 'malignant epithelioid neoplasm' of the brain, a rare variety of tumors. We also give the clinical course of this illness.

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“…IHC for STAT6 is a reliable marker to confirm the SFT/HPC destination. 12,13 Our study, included the SFT/HPC cases that were confirmed by STAT6, IHC, or next generation sequencing to avoid the diagnostic ambiguity. NAB2-STAT6 fusion is the driver mutation of SFT/HPC and the fusion constitutively activates EGR1.…”

Section: Discussionmentioning

confidence: 99%

High p16 Expression Is Associated with Malignancy and Shorter Disease-Free Survival Time in Solitary Fibrous Tumor/Hemangiopericytoma

Liang

Heller

et al. 2018

J Neurol Surg B

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Objective Solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are now classified along a single spectrum of fibroblastic mesenchymal tumors with NAB2–STAT6 fusion. This fusion acts as a driver mutation that constitutively activates EGR1, which is known to be involved in the p16 pathway. Overexpression of p16 is associated with malignancy and worse prognosis in multiple mesenchymal tumors. The authors sought to investigate p16 immunoexpression in association with malignancy and prognosis of SFT/HPC tumors. Design Twenty-three SFT/HPC tumors (central nervous system [CNS]: 12, non CNS: 11) diagnosed at our institution from 2002 to 2016 were assigned into 3 grades. Data from microarray immunohistochemistry for STAT6, synaptophysin, CD56, chromogranin, SST2A, EGR1, Ki67, and p16, grade and survival were analyzed. Results CNS SFT/HPCs tend to be malignant (grade 3; 67 vs. 18%, p = 0.036) and more likely to express synaptophysin (33 vs. 0%, p = 0.035) than non CNS tumors. Overexpression of p16 (immunopositivity ≥ 50% tumor cells) was associated with malignant (grade 3) tumors, and has a sensitivity of 70% (7/10), and a specificity of 77% (10/13), as a predictive marker for malignancy. SFT/HPC patients with low p16 expression demonstrated significantly longer disease-free survival time (median survival > 113 months) than those with high p16 expression (median survival = 30 months, p = 0.045). Conclusions SFT/HPCs in the CNS are more likely to be malignant than the tumors in other sites. High p16 expression is also associated with malignancy and shorter disease-free survival time in SFT/HPC tumors in our study cohort. Clinically, p16 overexpression can be used as predictive marker for malignancy and prognosis and a possible therapeutic target.

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Validation of nuclear STAT6 immunostaining as a diagnostic marker of meningeal solitary fibrous tumor (SFT)/hemangiopericytoma

Cited by 23 publications

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A case of solitary fibrous tumor/hemangiopericytoma in the central nervous system with papillary morphology

A case of solitary fibrous tumor/hemangiopericytoma in the central nervous system with papillary morphology

Malignant Epithelioid Neoplasm of the Brain

High p16 Expression Is Associated with Malignancy and Shorter Disease-Free Survival Time in Solitary Fibrous Tumor/Hemangiopericytoma

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