Adrenocortical carcinoma with rhabdoid features: a case report and literature review
10.3969/j.issn.1009-8291.2025.01.013
- VernacularTitle:具有横纹肌样特征的肾上腺皮质癌1例报告并文献复习
- Author:
Mingchuan CHU
1
,
2
;
Huimin SUN
3
;
Hao WANG
4
;
Jian SONG
5
;
Yongshun GUO
4
Author Information
1. School of Clinical Medicine, Shandong Second Medical University, Weifang 261000
2. Department of Urology, Weifang 261000, China
3. Precision Pathological Diagnosis Center, Weifang 261000, China
4. Department of Urology, Weifang 261000, China
5. Infection Management Office, Weifang People's Hospital, Weifang 261000, China
- Publication Type:Journal Article
- Keywords:
adrenocortical carcinoma;
rhabdoid features;
varicocele
- From:
Journal of Modern Urology
2025;30(1):64-68
- CountryChina
- Language:Chinese
-
Abstract:
[Objective] To investigate and summarize the clinicopathological features, diagnosis, treatment and prognosis of adrenocortical carcinoma with rhabdoid features. [Methods] The clinical diagnosis and treatment of a case of adrenocortical carcinoma with rhabdoid features admitled to Department of Urology, Weifang People's Hospital were reported.The clinical manifestations, pathological features, diagnosis and prognosis of the disease were analyzed in combination with relevant literature. [Results] A 34-year-old male patient was admitted due to scrotal distension and pain that had persisted for 6 months.Imaging examination showed a huge soft tissue tumor in the left adrenal region of the retroperitoneum with compression displacement of the left kidney, leading to obstruction of venous return in the left spermatic vein, which in turn caused varicose veins.The levels of serum renin, angiotensin, aldosterone, cortisol, and catecholamine were within normal ranges.Surgical resection of the tumor was performed, and postoperative pathological examination revealed that the tumor tissue was predominantly composed of rhabdoid cells, exhibiting positive immunohistochemical staining for INI 1, Syn, Calretinin and Vimentin.Genetic testing did not identify any deletion of SMARCB1 and SMARCA4 mutations.Therefore, the diagnosis was adrenocortical carcinoma with rhabdoid features.At the current 20-month follow-up, no recurrence or metastasis was observed.A review of the literature found that only 7 cases of this disease had been reported. [Conclusion] Adrenocortical carcinoma with rhabdoid features is a rare disease, and a definitive diagnosis is dependent upon pathological examination.Surgical resection remains the primary treatment.Long-term follow-up is essential, and further research is needed to evaluate the impact of adjuvant therapy.
