@article{GS155462,
author = {Gaia Cicioni and Immacolata Iannone and Cristina De Padua and Enrico Spalice and Daniele Crocetti and Mariarita Tarallo and Luigi Petramala and Claudio Letizia and Paolo Sapienza},
title = {Adrenal angiosarcoma, a rare neoplasm: a case report and a literature review},
journal = {Gland Surgery},
volume = {15},
number = {6},
year = {2026},
keywords = {},
abstract = {Background: Angiosarcoma (AS) is a rare malignant tumor of vascular endothelial origin, accounting for approximately 1% of soft tissue sarcomas. Primary adrenal AS represents an exceptionally rare entity, with very limited cases reported in the literature, resulting in significant diagnostic and therapeutic challenges.Case Description: A 67-year-old male with arterial hypertension presented with an incidentally detected left adrenal mass on abdominal ultrasound. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large heterogeneous lesion measuring up to 90 mm × 82 mm × 88 mm, with hemorrhagic components and progressive enlargement, raising suspicion for an aggressive malignancy. Endocrine workup was unremarkable. The patient underwent robotic left adrenalectomy with complete (R0) resection. Histological examination showed a malignant proliferation of pleomorphic epithelioid cells; immunohistochemistry confirmed positivity for CD31, CD34, and podoplanin, with a Ki-67 index of ~80%, establishing the diagnosis of adrenal AS. No adjuvant therapy was administered. At 4-month follow-up, the patient was alive and disease-free. A systematic review of the PubMed database identified 51 additional published cases, totaling 52 patients. The disease predominantly affects males (67%), mean age 58 years. Overall survival was 41% and disease-free rate 33% at 36 months; tumor size },
issn = {2227-8575}, url = {https://gs.amegroups.org/article/view/155462}
}