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

Sep 29, 2026

Primary adrenal leiomyosarcoma and primary adrenal epithelioid angiosarcoma are exceptionally rare mesenchymal tumors that commonly present as unilateral, nonfunctional adrenal masses and can mimic other adrenal or metastatic lesions. Accurate classification requires immunohistochemical analysis to guide clinical decisions. The report describes two leiomyosarcomas and one epithelioid angiosarcoma in elderly women without initial extra-adrenal disease. Surgical resection was achieved in all cases, highlighting diagnostic complexity and prognostic variability.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages14

Reading Time35 min

AccessFree to download

Background

Clinical presentation and diagnostic challenges

Histopathology and immunohistochemistry

Case-focused findings (from abstract)

Leiomyosarcoma features and progression

Epithelioid angiosarcoma features

Surgical management and outcomes

Imaging and differential diagnosis considerations

Primary Adrenal Sarcomas: Diagnostic Challenges and Therapeutic Insights From a Case Series Including Leiomyosarcoma and Epithelioid Angiosarcoma Busra Yaprak Bayrak, MD1 , Levente Kuthi, MD2,3 , Isa Cam, MD4 , Cigdem Vural, MD1, and Mahmut Akgul, MD5 International Journal of Surgical Pathology 2026, Vol. 34(1) 125–138 © The Author(s) 2025 Article reuse guidelines: [sagepub.com/journals-permissions](sagepub.com/journals-permissions)[ ](sagepub.com/journals-permissions)[DOI:](DOI: 10.1177/10668969251345715)[ 1](DOI: 10.1177/10668969251345715)[0.](DOI: 10.1177/10668969251345715)[11](DOI: 10.1177/10668969251345715)[77/](DOI: 10.1177/10668969251345715)[1](DOI: 10.1177/10668969251345715)[066896925](DOI: 10.1177/10668969251345715)[1](DOI: 10.1177/10668969251345715)[3457](DOI: 10.1177/10668969251345715)[1](DOI: 10.1177/10668969251345715)[5](DOI: 10.1177/10668969251345715)[ ](DOI: 10.1177/10668969251345715)[journals.sagepub.com/home/ijs](journals.sagepub.com/home/ijs) 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. Keywords adrenal gland, leiomyosarcoma, angiosarcoma, immunohistochemistry, rare tumors Background Primary adrenal leiomyosarcoma and primary adrenal epithelioid angiosarcoma are exceedingly uncommon malignant mesenchymal neoplasms of the adrenal gland, each constituting a rare but aggressive subset of nonepithelial adrenal tumors.1–3 In contrast to adrenocortical carcinoma and pheochromocytoma, both adrenal leiomyosarcoma and angiosarcoma are hormonally inactive and are most often discovered incidentally during imaging performed for unrelated complaints or in the workup of nonspeciﬁc abdominal or ﬂank pain.2–4 Owing to their rarity, nonfunctionality, and overlapping radiologic features with more prevalent adrenal lesions such as adenomas, timely diagnosis remains challenging.4,5 From a histopathological standpoint, primary adrenal leiomyosarcomas, similar to their counterparts in any organ system, are characterized by intersecting fascicles of spindle cells exhibiting smooth muscle differentiation, with varying degrees of atypia and mitotic activity.2,3 Immunohistochemical (IHC) analysis is essential for deﬁnitive diagnosis, with positive staining for markers such as desmin, caldesmon, and smooth muscle actin, and negative expression of epithelial, neuroendocrine, or adrenocortical markers.4,5 In contrast, again similar to angiosarcomas arising in any organ system, primary adrenal angiosarcoma demonstrates epithelioid cytomorphology and may exhibit variable immunoreactivity for endothelial markers including CD31, ERG, and FLI1, often accompanied by focal or aberrant expression of pankeratin, further complicating 1Department of Pathology, Kocaeli University School of Medicine, Kocaeli, Turkey 2Department of Surgical and Molecular

FAQs

Why are primary adrenal leiomyosarcoma and epithelioid angiosarcoma difficult to diagnose?

They are exceptionally rare, typically nonfunctional, and often resemble other adrenal tumors or metastatic disease on imaging, creating diagnostic uncertainty.

What role does immunohistochemistry play in confirming the diagnosis?

Immunohistochemical staining is essential to accurately classify these tumors, using smooth muscle markers for leiomyosarcoma and endothelial marker patterns (e.g., CD31/ERG) with additional markers for epithelioid angiosarcoma.

How were the patients managed and what were the reported outcomes?

Complete surgical resection was achieved for all reported patients, and the cases illustrate diagnostic complexity and variability in prognosis, including one case progressing to hepatic metastasis.

Sep 29, 2026

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