Solitary Fibrous Tumor: Integration of Clinical, Morphologic

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Solitary Fibrous Tumor: Integration of Clinical, Morphologic International Journal of Molecular Sciences Article Solitary Fibrous Tumor: Integration of Clinical, Morphologic, Immunohistochemical and Molecular Findings in Risk Stratification and Classification May Better Predict Patient Outcome Isidro Machado 1,2,*, María Gema Nieto Morales 3 , Julia Cruz 1, Javier Lavernia 4, Francisco Giner 5, Samuel Navarro 6, Antonio Ferrandez 6 and Antonio Llombart-Bosch 6 1 Pathology Department, Instituto Valenciano de Oncología, 46009 Valencia, Spain; jcruz@fivo.org 2 Patologika Laboratory, Pathology Department, Hospital Quiron-Salud, 46009 Valencia, Spain 3 Zoology Department, University of Valencia, 46009 Valencia, Spain; [email protected] 4 Department of Oncology, Instituto Valenciano de Oncología, 46009 Valencia, Spain; [email protected] 5 Pathology Department, University Hospital “La Fe”, 46009 Valencia, Spain; [email protected] 6 Pathology Department, University of Valencia, 46009 Valencia, Spain; [email protected] (S.N.); [email protected] (A.F.); [email protected] (A.L.-B.) * Correspondence: [email protected] Abstract: Although solitary fibrous tumors (SFTs) have an unpredictable evolution, some specific Citation: Machado, I.; Nieto Morales, clinicopathologic factors have been associated with the final outcome. We retrieved clinical, patholog- M.G.; Cruz, J.; Lavernia, J.; Giner, F.; ical and molecular data of 97 patients with a histological diagnosis of SFT and Signal transducer and Navarro, S.; Ferrandez, A.; activator of transcription 6 (STAT6) positivity. We retrospectively studied the pathological factors Llombart-Bosch, A. Solitary Fibrous predictive of recurrence/metastasis and compared them with the clinical outcome. A wide immuno- Tumor: Integration of Clinical, histochemical study and molecular analysis to detect NAB2/STAT6 gene fusion, tumor protein-53 Morphologic, Immunohistochemical (TP53) and/or (telomerase reverse transcriptase) TERT promotor mutation were performed. The and Molecular Findings in Risk risk of metastasis was calculated using the Demicco risk stratification system (RSS). The results were Stratification and Classification May combined and examined to assess the accuracy of risk stratification and classification. The most Better Predict Patient Outcome. Int. J. common location was in non-extremities; 66% were located in soft tissue or subcutaneous areas and Mol. Sci. 2021, 22, 9423. https:// 92.8% in deep locations. On microscopic analysis, 38.1% of tumors revealed hypercellularity with a doi.org/10.3390/ijms22179423 predominant patternless and/or hemangiopericytic growth pattern; 13.4% had ≥4 mitoses/10HPF; Academic Editor: Maria 16.5% showed necrosis, and almost half the tumors showed at least focal myxoid areas. Dedif- Grazia Cifone ferentiation was observed in three tumors. Immunomarker expression in SFTs was as follows: CD34 92.9%, CD99 57.1%, Bcl2 67.9%, neuroendocrine markers (at least 1) 25.7%, Desmin 14.3%, Received: 12 July 2021 CK(AE1/AE3) 3%, Apoptotic Protease Activating Factor (APAF-1) 87% and finally Ki-67 ≥10% Accepted: 26 August 2021 in 14.4%. The NAB2/STAT6 gene fusion was detected in 50 tumors. After a median follow-up of Published: 30 August 2021 90 months, 9.3% recurred, 11.3% metastasized, 10.3% died of disease and 76.2% were free of disease. TERT mutations were detected in 40.6% of the SFTs; the TP53 mutation was detected in 17%, and Publisher’s Note: MDPI stays neutral only 9.3% showed both mutations. According to the Demicco RSS, 6.1%, 11.3% and 82.4% of the with regard to jurisdictional claims in tumors were classified as high, intermediate or low-risk of metastasis, respectively. All high-risk published maps and institutional affil- tumors had ≥4 mitoses/10HPF, necrosis, Ki-67 ≥ 10, HTER and/or TP53 mutation and poor evo- iations. lution. The intermediate risk SFTs with worse evolution displayed the HTER mutation. Almost all low-risk tumors had a favorable evolution, although four showed at least one adverse factor (Ki-67 ≥ 10, ≥4 mitoses/10HPF or high tumor size) and had a worse evolution. An integration of clinical, morphologic, immunohistochemical and molecular findings may improve risk stratification and Copyright: © 2021 by the authors. classification and better predict patient outcome. The unfavorable course seems to be more frequent Licensee MDPI, Basel, Switzerland. in high-risk SFTs, although it is not exceptional in low-risk SFTs either; hence, a long-term follow-up This article is an open access article is required independently of the assigned risk stratification score. The inclusion of molecular findings distributed under the terms and in risk stratification systems could improve the precision in the classification of SFTs, especially conditions of the Creative Commons Attribution (CC BY) license (https:// those of intermediate risk. Future studies will be required to determine the most effective way to creativecommons.org/licenses/by/ incorporate molecular analyses into RSS on SFTs. The coexistence of several adverse factors such 4.0/). Int. J. Mol. Sci. 2021, 22, 9423. https://doi.org/10.3390/ijms22179423 https://www.mdpi.com/journal/ijms Int. J. Mol. Sci. 2021, 22, 9423 2 of 14 as ≥4 mitoses/10HPF, necrosis, Ki-67 ≥ 10%, mutations in HTER and/or p53 may suggest a closer clinical follow-up regardless of the histological appearance of the tumor. Keywords: solitary fibrous tumor; risk stratification systems; STAT6; APAF-1; HTER mutation; p53 mutation 1. Introduction Solitary fibrous tumors (SFTs) are mesenchymal neoplasms that can occur at any location, especially pleural, meningeal or extrapleural sites. Location in limbs is infre- quent [1–40]. The evolution of SFTs is often uncertain [2–10]. Although most cases evolve favorably, a small group can progress towards dissemination, generally pulmonary, as well as other locations, and will lead to patient death in the absence of effective treatments [1–19]. Thus, valid and reproducible tools should be developed in order to design risk-tailored treatment and risk-adjusted follow-up. A patternless and hemangiopericytic growth with variably fibrosis and collagenous de- posits are characteristic, but SFTs can show many faces, including round cells, giant-cells, myx- oid areas, pleomorphic pattern, fat-forming tumors and dedifferentiated forms [1–5,15–25]. Strong and diffuse nuclear immunoreactivity for Signal transducer and activator of transcription 6 (STAT6) is almost always diagnosed in SFTs, and CD34, Bcl-2 and CD99 immunoexpression is common [1–25]. Aberrant epithelial, muscular or neuroendocrine marker expression has been described which may lead to confusion with other tumors that share a similar morphology [15–25,36]. The fusion gene NAB2/STAT6 and its variants confirm a diagnosis of SFT in those cases with unconvincing STAT6 immunoreactivity, and specific gene fusions have been related to prognosis and tumor location [6–14,26–40]. STAT6 nuclear expression is not exclusive to SFTs and has also been reported in dedifferen- tiated liposarcoma, glioma-associated oncogene homolog 1 (GLI1)-amplified tumor, Kaposi sarcoma and Hodgkin as well non-Hodgkin lymphomas [6,12–25,36–40]. Several risk-stratification systems (RSS) have been described [1–5], although the Demicco et al. RSS seems to be the most widely implemented [1,2,14]. While most SFTs categorized as low-risk by the Demicco system follow an apparently benign course, some cases may have late relapse or metastases; hence, the RSS is not perfectly specific in predicting the evolution in all cases [1,2,14]. In addition, molecular results have not so far been included in any of the current RSS [1,2,14]. Recently, loss of Apoptotic Protease Activating Factor (APAF-1) expression has been associated with poor prognosis in SFTs, but these findings need to be confirmed in larger series [41]. We have previously published a small series of SFTs with at least one histological factor associated with aggressive behavior and explored the correlation between the adverse histological findings and molecular profile with tumor behavior [42]. The main goal of the present study was to confirm our previous results including additional tumors, mainly SFTs categorized as low-risk by Demicco et al. [1,2] and correlate the clinical, histological and immunohistochemical findings, TP53 mutational status and TERT promotor mutational status with clinical outcome. 2. Results 2.1. Clinicopathological and Histopathological Findings The clinicopathological and follow-up data are shown in Tables1 and2. The histologic findings are shown in Figure1 and summarized in Table3. The median follow-up was 90 months. In the microscopic analysis, 38.1% of tumors revealed hypercellularity with a predominant patternless and/or hemangiopericytic growth pattern (Figure1); 13.4% had ≥4 mitoses/10HPF; 16.5% showed necrosis and myxoid areas were frequent (Figure1). Int. J. Mol. Sci. 2021, 22, 9423 3 of 14 Dedifferentiation was observed in three tumors (Figure1). Rare histological patterns were found in sporadic cases (Figure1). 2.2. Immunohistochemistry Results The representative status of the immunohistochemical profile is shown in (Figure2 ) and summarized in Table4. All tumors were diffusely positive for STAT6 (Figure2C), CD34, CD99 (Figure2A,B) and Bcl2 which were frequent, and neuroendocrine markers (at least 1), Desmin, CK(AE1/AE3) were detected sporadically. APAF-1 immunoexpression was detected in 87% of the SFTs, and Ki-67 ≥ 10 was found in 14.4% of the tumors. Table 1. Solitary fibrous tumor clinical data (N = 97). Variables N % Age <55 52 53.6 ≥55 45 46.4 Gender Male 46 47.4 Female 51 52.6 Tumor location Limbs 6 6.2 Not limbs 91 93.8 Tumor location Soft tissue and/or subcutaneous 64 66 tissues Pleural 19 19.6 Visceral 12 12.3 Meningeal 2 2.1 Tumor size <5 cm 38 39.1 5–<10 cm 36 37.1 10–<15 cm 12 12.4 ≥15 cm 11 11.4 Location Deep 90 92.8 Superficial 7 7.2 Surgery Yes 94 97 No 3 3 Radiotherapy Yes 4 4 No 93 96 Chemotherapy Yes 7 7.2 No 90 92.8 Int. J. Mol. Sci. 2021, 22, 9423 4 of 14 Table 2.
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