Work

Primary Adrenal Sarcomas: Diagnostic Challenges and Therapeutic Insights From a Case Series Including Leiomyosarcoma and Epithelioid Angiosarcoma

Year: 2025

Type: article

Abstract: Primary adrenal leiomyosarcoma and primary adrenal epithelioid angiosarcoma are exceptionally rare mesenchymal tumors of the adrenal gland. Both typically present as unilateral, nonfunctional adrenal masses and may closely resemble other adrenal or metastatic tumors, thus making diagnosis challenging. Immunohistochemical analysis is essential for accurate classification and clinical decision-making. We report two primary adrenal leiomyosarcomas and one primary adrenal epithelioid angiosarcoma, all occurring in elderly female patients without initial evidence of extra-adrenal disease. The leiomyosarcomas demonstrated spindle cell morphology with strong expression of smooth muscle markers and variable proliferative activity, including one patient that progressed to hepatic metastasis. The epithelioid adrenal angiosarcoma exhibited epithelioid features, extensive necrosis, diffuse CD31 and ERG positivity, and focal keratin expression. Complete surgical resection was achieved in all patients. These patients highlight the diagnostic complexity and prognostic variability of adrenal sarcomas. Documenting such rare tumors remains critical to improving diagnostic precision and guiding optimal management strategies.

Source: International Journal of Surgical Pathology

DOI: 10.1177/10668969251345715

Authors: Busra Yaprak Bayrak, Levente Kuthi, Isa Cam, Cigdem Vural, Mahmut Akgul

Institutions: Kocaeli Üniversitesi, Semmelweis University, National Institute of Oncology, Brigham and Women's Hospital

Language: English

Topic: Sarcoma Diagnosis and Treatment

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,150