Solitary fibrous tumor of the adrenal gland – its biological behavior and report of a new case

Surgical and Experimental Pathology

P. van Battum¹  I. Verlinden²  J. W. A. Leijtens³  S. E. Huisman⁴ 

[1] Department of Internal Medicine, Zuyderland Medical Center, Henri Dunantstraat 5, 6419PC, Heerlen, The Netherlands;Department of Pathology, Laurentius Hospital, Monseigneur Driessenstraat 6, 6043CV, Roermond, The Netherlands;Department of Surgery, Laurentius Hospital, Monseigneur Driessenstraat 6, 6043CV, Roermond, The Netherlands;Maastricht University Medical Center, Maastricht University, P. Debyelaan 25, 6229HX, Maastricht, The Netherlands;Department of Surgery, Laurentius Hospital, Monseigneur Driessenstraat 6, 6043CV, Roermond, The Netherlands;

关键词: Solitary fibrous tumor;    Adrenal gland;    Incidentaloma;    Case report;    Adrenalectomy;   

DOI  :  10.1186/s42047-021-00088-1

来源: Springer

【 摘 要 】

IntroductionA solitary fibrous tumor (SFT) is an uncommon neoplasm of mesenchymal and probably fibroblastic origin, occurring mainly in the extremities, and pleura. However, a primary involvement of endocrine organs is rare and even exceptional when found in the adrenal gland. Hereby, we describe the 10th report of an adrenal SFT.Case presentationA 77-year old man was diagnosed with a lesion in the right adrenal gland during a urologic indicated computed tomography (CT). No symptoms and laboratory anomalies were reported indicating any endocrine activity. Follow up CT-scans showed progressive growth of the nodule for which the patient underwent laparoscopic right adrenalectomy. Histological examination showed a hypercellular spindle cell neoplasm with elongated nuclei and a low mitotic index. The vessels were arranged in a hemangiopericytoma-like pattern with a slight sclerosing appearance. Immunohistochemistry showed a positive staining of neoplastic cells for STAT6, CD-34 and Bcl-2. Translocation analysis using RT-PCR showed no NAB2-STAT6 fusion. The specimen was confirmed as a hypercellular variant of an adrenal SFT.DiscussionSFT is a rare neoplasm when occurring in the adrenal gland. Differential diagnosis can be broad because of no defined pathognomonic morphological characteristics. However, NAB2-STAT6 gene fusions are considered a molecular hallmark of SFTs. Therefore, STAT6 immunohistochemistry is a valuable diagnostic tool in differentiating between SFT and histologic mimics. After diagnosing SFT, its biological behavior is difficult to predict. SFTs are mostly benign tumors. Nonetheless, a histological benign-appearing SFT can show malignant clinical characteristics impeding assessment of proper follow up. However, malignancy has not been previously reported in any adrenal SFT case report.

【 授权许可】

CC BY