{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T20:36:21Z","timestamp":1785875781801,"version":"3.56.0"},"reference-count":39,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T00:00:00Z","timestamp":1661126400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,10,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that can arise at various anatomic locations. It is characterized by inv12(q13q13)-derived NAB2::STAT6 fusion, resulting in the nuclear expression of STAT6. Primary SFT of the adrenal gland is rare. We launched a multi-institutional collaboration to comprehend the overarching demographics, clinical and follow-up, macroscopic, microscopic, IHC, and FISH features of 9 patients with SFT of the adrenal gland.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We added a series of 9 patients to the collection of adrenal SFTs where the clinicopathologic parameters, including clinical presentation, imaging, histopathology, IHC, molecular profiles, and management and follow-up data, were analyzed comprehensively. A modified 4-variable risk stratification model, including age, tumor size, and necrosis, was applied.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Our series consisted of 6 male and 3 female patients, ranging in age from 19 to 64 years (mean, 49.3 years). Abdominal pain (4) and fever with abdominal pain (1) were the presenting symptoms in 5 patients. In the remaining 4 patients, the tumors were detected by abdominal imaging for hypertension and diabetes. The size of the tumor ranged from 2 cm to 10.5 cm in maximum dimension. All tumors exhibited the morphology of a spindle cell SFT with a patternless architecture; 3 had a focal storiform arrangement. STAT6 positivity was observed in all tumors, and 7 were positive for CD34. Surgical resection was the primary modality of treatment. No adjuvant therapy was administered. Follow-up ranging from 7 months to 23 months was available for 7 patients. All were alive without disease recurrence or metastasis. Risk stratification placed 8 (88.9%) patients into a low-risk category and 1 into an intermediate-risk category.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>This series is the largest of adrenal SFTs to date. These tumors of the adrenal gland are predominantly spindle cell neoplasms with indolent behavior, with a wide age distribution and a slight male preponderance. Combining our cohort with the previously published cases, the majority of tumors fall into the low-risk category for the propensity to develop metastases. Owing to the rarity and age distribution associated with these tumors, the differential diagnosis is wide and requires a systematic approach for ruling out key differential diagnoses aided by STAT6 IHC.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ajcp\/aqac088","type":"journal-article","created":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T09:57:49Z","timestamp":1661162269000},"page":"546-554","source":"Crossref","is-referenced-by-count":7,"title":["Solitary Fibrous Tumor of the Adrenal Gland"],"prefix":"10.1093","volume":"158","author":[{"given":"Shilpy","family":"Jha","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Prolife Diagnostics , Bhubaneswar , India"},{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5094-2424","authenticated-orcid":false,"given":"Sambit K","family":"Mohanty","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Prolife Diagnostics , Bhubaneswar , India"},{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"},{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nakul Y","family":"Sampat","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Subhasini","family":"Naik","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Prolife Diagnostics , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manas R","family":"Baisakh","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Prolife Diagnostics , Bhubaneswar , India"},{"name":"Department of Pathology and Laboratory Medicine, Apollo Hospital and Apollo Cancer Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niharika","family":"Pattnaik","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anandi","family":"Lobo","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sudhasmita","family":"Rauta","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Prolife Diagnostics , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shivani","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gauri","family":"Munjal","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepika","family":"Jain","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samriti","family":"Arora","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vipra","family":"Malik","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekta","family":"Jain","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biswajit","family":"Sahoo","sequence":"additional","affiliation":[{"name":"Department of Radiodiagnosis, All India Institute of Medical Sciences , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohit","family":"Bhardwaj","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, CORE Diagnostics , Haryana , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saurav K","family":"Mishra","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Apollo Cancer Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunil","family":"Jaiswal","sequence":"additional","affiliation":[{"name":"Department of Surgical Oncology, Apollo Cancer Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lalit M","family":"Barik","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Advanced Medical Research Institute , Bhubaneswar , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5136-6617","authenticated-orcid":false,"given":"Bonnie L","family":"Balzer","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center , Los Angeles, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indranil","family":"Chakrabarti","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, North Bengal Medical College , Siliguri , India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil V","family":"Parwani","sequence":"additional","affiliation":[{"name":"Department of Pathology, Wexner Medical Center, The Ohio State University , Columbus , OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,8,22]]},"reference":[{"key":"2022100608584995300_CIT0001","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1097\/PAP.0000000000000284","article-title":"The 2020 WHO Classification of Tumors of Soft Tissue: selected changes and new entities","volume":"28","author":"Choi","year":"2021","journal-title":"Adv Anat 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An update on clinical, molecular and diagnostic features","volume":"34","author":"Ronchi","year":"2018","journal-title":"Ann Diagn Pathol."},{"key":"2022100608584995300_CIT0003","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/ajim.4700220103","article-title":"Primary neoplasms of the pleura: a report of five cases","volume":"22","author":"Klemperer","year":"1992","journal-title":"Am J Ind Med"},{"key":"2022100608584995300_CIT0004","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1038\/modpathol.2012.83","article-title":"Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model","volume":"25","author":"Demicco","year":"2012","journal-title":"Mod Pathol."},{"key":"2022100608584995300_CIT0005","doi-asserted-by":"crossref","first-page":"1979","DOI":"10.1093\/annonc\/mdx250","article-title":"Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database","volume":"28","author":"Salas","year":"2017","journal-title":"Ann Oncol"},{"key":"2022100608584995300_CIT0006","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.1038\/modpathol.2016.126","article-title":"TERT promoter mutations and prognosis in solitary fibrous tumor","volume":"29","author":"Bahrami","year":"2016","journal-title":"Mod Pathol."},{"key":"2022100608584995300_CIT0007","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1038\/modpathol.2017.54","article-title":"Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model","volume":"30","author":"Demicco","year":"2017","journal-title":"Mod Pathol."},{"key":"2022100608584995300_CIT0008","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1136\/jclinpath-2016-204088","article-title":"Solitary fibrous tumor of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2::STAT6 fusion gene","volume":"70","author":"Kouba","year":"2017","journal-title":"J Clin Pathol"},{"key":"2022100608584995300_CIT0009","first-page":"nlz053","article-title":"Papillary solitary fibrous tumor\/hemangiopericytoma: an uncommon morphological form with NAB2::STAT6 gene fusion","author":"Yao","year":"2019","journal-title":"J Neuropathol Exp Neurol"},{"key":"2022100608584995300_CIT0010","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1038\/ng.2522","article-title":"Whole-exome sequencing identifies a recurrent NAB2::STAT6 fusion in solitary fibrous tumors","volume":"45","author":"Chmielecki","journal-title":"Nat Genet."},{"key":"2022100608584995300_CIT0011","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1038\/ng.2509","article-title":"Identification of recurrent NAB2::STAT6 gene fusions in solitary fibrous tumor by integrative sequencing","volume":"45","author":"Robinson","year":"2013","journal-title":"Nat 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retroperitoneal solitary fibrous tumor in the suprarenal region","volume":"188","author":"Kakihara","year":"2007","journal-title":"AJR Am J Roentgenol."},{"key":"2022100608584995300_CIT0016","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1007\/s12022-010-9113-z","article-title":"Solitary fibrous tumor of the adrenal gland with unusual immunophenotype: a potential diagnostic problem and a brief review of endocrine organ solitary fibrous tumor","volume":"21","author":"Ho","year":"2010","journal-title":"Endocr Pathol."},{"key":"2022100608584995300_CIT0017","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1007\/s12020-013-0082-0","article-title":"A very rare bilateral adrenal tumor","volume":"45","author":"Toniato","year":"2014","journal-title":"Endocrine."},{"key":"2022100608584995300_CIT0018","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1186\/1477-7819-12-87","article-title":"Suprarenal solitary fibrous tumor associated with a NF1 gene mutation mimicking a kidney neoplasm: implications for surgical management","volume":"12","author":"Conzo","year":"2014","journal-title":"World J Surg Oncol"},{"key":"2022100608584995300_CIT0019","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1097\/RLU.0b013e31828e9752","article-title":"A rare case of solitary fibrous tumor of the adrenal gland detected by (18)F-FDG PET\/CT","volume":"39","author":"Treglia","year":"2014","journal-title":". Clin Nucl Med."},{"key":"2022100608584995300_CIT0020","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1186\/s13256-019-2163-z","article-title":"A giant solitary fibrous tumor of the adrenal gland in a 13-year old: a case report and review of the literature","volume":"13","author":"Gebresellassie","year":"2019","journal-title":"J Med Case Rep"},{"key":"2022100608584995300_CIT0021","doi-asserted-by":"crossref","first-page":"100919","DOI":"10.1016\/j.eucr.2019.100919","article-title":"Adrenal solitary fibrous tumor: a case report","volume":"27","author":"Yonli","year":"2019","journal-title":"Urol Case Rep"},{"key":"2022100608584995300_CIT0022","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1177\/1066896915627485","article-title":"The current status of solitary fibrous tumor: diagnostic features, variants, and genetics","volume":"24","author":"Thway","year":"2016","journal-title":"Int J Surg Pathol."},{"key":"2022100608584995300_CIT0023","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.prp.2009.02.012","article-title":"Sarcomatoid carcinoma of the adrenal gland: a case report and review of literature","volume":"206","author":"Coli","year":"2010","journal-title":"Pathol Res Pract"},{"key":"2022100608584995300_CIT0024","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1038\/modpathol.2013.164","article-title":"Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics","volume":"27","author":"Doyle","year":"2014","journal-title":"Mod Pathol."},{"key":"2022100608584995300_CIT0025","first-page":"95","article-title":"Solitary fibrous tumor of the pleura as a rare cause of severe hypoglycemia: Doege-Potter syndrome","volume":"99","author":"Pavl\u00edkov\u00e1","journal-title":"Rozhl 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spectrum of malignant progression in solitary fibrous tumors: a study of 8 cases with a discrete anaplastic component\u2014is this dedifferentiated SFT?","volume":"33","author":"Mosquera","year":"2009","journal-title":"Am J Surg Pathol."},{"key":"2022100608584995300_CIT0035","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1177\/1066896916650257","article-title":"STAT6 loss in dedifferentiated solitary fibrous tumor","volume":"25","author":"Schneider","year":"2017","journal-title":"Int J Surg Pathol."},{"key":"2022100608584995300_CIT0036","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1038\/modpathol.2015.70","article-title":"Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation","volume":"28","author":"Dagrada","year":"2015","journal-title":"Mod Pathol."},{"key":"2022100608584995300_CIT0037","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1038\/modpathol.2015.90","article-title":"NAB2::STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors","volume":"28","author":"Tai","year":"2015","journal-title":"Mod Pathol"},{"key":"2022100608584995300_CIT0038","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1111\/his.13703","article-title":"TERT promoter mutations in solitary fibrous tumour","volume":"73","author":"Demicco","year":"2018","journal-title":"Histopathology."},{"key":"2022100608584995300_CIT0039","first-page":"313","article-title":"Solitary fibrous tumor: an evolving and unifying entity with unsettled issues","volume":"34","author":"Huang","year":"2019","journal-title":"Histol Histopathol."}],"container-title":["American Journal of Clinical 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