Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics

Fatema Jabarkhel; Henri Puttonen; Lina Hansson; Andreas Muth; Oskar Ragnarsson

doi:10.1210/jendso/bvaa055

Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics

J Endocr Soc. 2020 May 15;4(6):bvaa055. doi: 10.1210/jendso/bvaa055. eCollection 2020 Jun 1.

Authors

Fatema Jabarkhel¹, Henri Puttonen², Lina Hansson³, Andreas Muth⁴, Oskar Ragnarsson¹

Affiliations

¹ Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg and The Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden.
² Department of Pathology, Sahlgrenska University Hospital, Gothenburg, Sweden.
³ Department of Oncology, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁴ Department of Surgery, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Sahlgrenska University Hospital, Gothenburg, Sweden.

Abstract

Primary adrenal leiomyosarcoma (PAL) is a rare, high-grade proliferating mesenchymal tumor with a considerable risk of metastasis, deriving from the smooth muscle wall of a central adrenal vein, or its tributaries. Roughly 40 patients with PAL have been reported in the literature. Herein, we present 3 patients with incidentally discovered PAL, along with an overview of the current knowledge on the clinical, radiological, and histopathological characteristics of PAL.

Keywords: adrenal carcinoma; adrenal incidentaloma; leiomyosarcoma; vena cava occlusion.

Publication types

Case Reports