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

Introduction A 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 t...

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Veröffentlicht in:Surgical and Experimental Pathology 2021-02, Vol.4 (1), p.1-5, Article 6
Hauptverfasser: Huisman, S. E., Verlinden, I., van Battum, P., Leijtens, J. W. A.
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Sprache:eng
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Zusammenfassung:Introduction A 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 presentation A 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. Discussion SFT 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. Keywords: Solitary fibrous tumor, Adrenal gland, Incidentaloma, Case report, Adrenalectomy
ISSN:2520-8454
2520-8454
DOI:10.1186/s42047-021-00088-1