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Introduction <p>Sarcomatoid Adrenocortical Carcinoma (SAC) is an extremely rare, highly aggressive, and poorly differentiated subtype of Adrenocortical Carcinoma (ACC). To date, only 38 cases have been reported worldwide. No standardized clinical diagnosis and treatment protocols have been established, and the prognosis of affected patients is extremely poor. This study reports a case of SAC in a 60-year-old female patient presenting with an abdominal mass, aiming to analyze the clinical characteristics, diagnosis, and treatment features of this rare tumor.</p> Case presentation <p>A 60-year-old female was admitted to our hospital due to an upper abdominal mass. Imaging examinations revealed a cystic-solid space-occupying lesion in the left retroperitoneum, and the tumor was highly suspected to be of adrenal cortical origin preoperatively. Given the rapid disease progression and the patient’s strong demand for urgent surgery, preoperative endocrine function examinations including cortisol, aldosterone, sex hormones and catecholamines were not performed. Laparotomy with complete tumor resection was Sarcomatoid Adrenocortical Carcinomasubsequently performed, and the final pathological diagnosis was SAC. The tumor was composed of 60% carcinomatous components and 40% high-grade sarcomatous components, classified as Fuhrman nuclear grade IV, with extensive parenchymal necrosis and vascular tumor thrombi observed in the lesion. One month after surgery, the patient developed extensive peritoneal metastasis and ascites, and eventually died on the 42nd day postoperatively.</p> Conclusion <p>SAC has non-specific manifestations and high diagnostic difficulty, with histopathology plus immunohistochemistry as the diagnostic gold standard. Highly invasive and rapidly progressive, it is mainly treated with surgical resection by following ACC protocols, yet their pathological features differ significantly. Given the extremely limited number of reported cases, current evidence is insufficient to either support or refute the efficacy of mitotane for SAC, and more multicenter large-sample studies are urgently needed.</p>

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Sarcomatoid adrenocortical carcinoma with aggressive disease course: a rare case report and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年6月23日

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  • Xin-Xin Xu,
  • Guang-Fu Zhou,
  • Cheng-Zhou Du,
  • Xiao-Xin Gao,
  • Xiao-Wei Guo,
  • Ya-Long Li,
  • Meng-Yuan Lu,
  • Tian-Xiao He,
  • Peng Chen,
  • Hong-Tao Li

摘要

Introduction

Sarcomatoid Adrenocortical Carcinoma (SAC) is an extremely rare, highly aggressive, and poorly differentiated subtype of Adrenocortical Carcinoma (ACC). To date, only 38 cases have been reported worldwide. No standardized clinical diagnosis and treatment protocols have been established, and the prognosis of affected patients is extremely poor. This study reports a case of SAC in a 60-year-old female patient presenting with an abdominal mass, aiming to analyze the clinical characteristics, diagnosis, and treatment features of this rare tumor.

Case presentation

A 60-year-old female was admitted to our hospital due to an upper abdominal mass. Imaging examinations revealed a cystic-solid space-occupying lesion in the left retroperitoneum, and the tumor was highly suspected to be of adrenal cortical origin preoperatively. Given the rapid disease progression and the patient’s strong demand for urgent surgery, preoperative endocrine function examinations including cortisol, aldosterone, sex hormones and catecholamines were not performed. Laparotomy with complete tumor resection was Sarcomatoid Adrenocortical Carcinomasubsequently performed, and the final pathological diagnosis was SAC. The tumor was composed of 60% carcinomatous components and 40% high-grade sarcomatous components, classified as Fuhrman nuclear grade IV, with extensive parenchymal necrosis and vascular tumor thrombi observed in the lesion. One month after surgery, the patient developed extensive peritoneal metastasis and ascites, and eventually died on the 42nd day postoperatively.

Conclusion

SAC has non-specific manifestations and high diagnostic difficulty, with histopathology plus immunohistochemistry as the diagnostic gold standard. Highly invasive and rapidly progressive, it is mainly treated with surgical resection by following ACC protocols, yet their pathological features differ significantly. Given the extremely limited number of reported cases, current evidence is insufficient to either support or refute the efficacy of mitotane for SAC, and more multicenter large-sample studies are urgently needed.