Primary Adrenal Angiosarcoma: A Rare and Potentially Misdiagnosed Tumor

Abstract

A man aged 69 years presented with acute right flank pain secondary to a hemorrhagic large adrenal tumor. En bloc resection was performed to repair the inferior vena cava. Immunoperoxidase levels in the tumor were positive for factor VIII and CD31 and negative for S100, protein Melan-A, CD34, synaptophysin, chromogranin, desmin, muscle specific actin, ETFA (EMA), KRT20 (CK20), CDX2, TTF1, LNPEP (PLAP), inhibin, ?-fetoprotein, CD30, hepatocyte paraffin, and aberrant expression of cytokeratin 7 and pankeratin. The pathological diagnosis was consistent with adrenal angiosarcoma. Obtaining appropriate immunoperoxidase stains and multidisciplinary evaluation helped make the diagnosis of this rare adrenal tumor and determine its management. The patient had an uneventful postoperative course and completed 4 cycles of adjuvant chemotherapy with doxorubicin/ifosfamide and adequately tolerated the treatment. However, positive surgical margins were found, so he was referred to radiation oncology specialists for possible adjuvant radiotherapy to the surgical bed. Weeks after the first initiation of therapy, the patient presented to the emergency department complaining of shortness of breath, fatigue, and generalized weakness for 3 days. He was admitted and found to have new-onset anemia and a new-onset, large, right pleural effusion. Thoracentesis performed showed sanguinolent fluid that, after microscopic evaluation, was suggestive of recurrent malignancy. Thoracic aortography performed with subselective catheterization to several arteries (right bronchial, right phrenic, and right renal arteries) did not show any active bleeding. However, the right inferior intercostal and adrenal arteries were presumed to be the reason for the bleeding event, so they were embolized until stasis. The patient remained hemodynamically unstable but eventually experienced multiorgan failure. In spite of aggressive measures, he died 10 days after admission to the hospital.

Fig 1

A–B. — (A) Axial and (B) coronal views of computed tomography with contrast. Illustrated is a bilobed, right suprarenal, heterogeneous mass 16.4 × 9.1 × 9.5 cm in size centered in the right adrenal gland that has displaced the adjacent right kidney, right hepatic lobe, the right renal vein, and inferior vena cava. This mass demonstrates attenuation greater than simple fluid, with areas of linear and peripheral hyperattenuation seen predominantly inferiorly, suggesting internal complexity.

Fig 2

A–E. —(A) Malignant vessels with cytological atypia and mitotic figures (hematoxylin and eosin stain, × 40). (B) Factor VIII indicates vascular differentiation of the epithelioid cells (× 40). (C) Cytokeratin 7 shows aberrant staining with this keratin antibody (× 20). (D) Groups of atypical epithelioid vessels are visible (hematoxylin and eosin stain, × 10). (E) Highly atypical epithelioid cells can be observed (Romanowsky stain, × 40).

Fig 3

A–B. — (A) Axial and (B) coronal views of computed tomography with contrast. Massive, right-sided pleural effusion measuring 25–32 HU, suggesting complicated fluid such as proteinaceous or hemorrhagic debris. Prominent, associated, compressive atelectasis is present with some aeration to the right upper lobe. A discrete hyperdense sentinel clot is not noted to suggest active hemorrhage and no contrast extravasation.