Adrenal angiosarcoma, a rare neoplasm: a case report and a literature review

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 <6 cm was associated with improved survival (P=0.04).

Conclusions: Primary adrenal AS is an aggressive tumor with poor prognosis. Complete surgical resection is the cornerstone of treatment. However, since available evidence is limited, no definitive prognostic factors can be established.