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

Author:

Huisman S. E.,Verlinden I.,van Battum P.,Leijtens J. W. A.ORCID

Abstract

Abstract 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.

Publisher

Springer Science and Business Media LLC

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