肾上腺神经源性瘤（AS）和肾上腺周围神经源性瘤（PAS）是极为罕见的来源于肾上腺髓质和肾上腺周围周围神经的神经源性肿瘤。其潜在的遗传事件仍然难以捉摸。我们在我们医疗机构的数据库中搜索了AS/PAS病例，并审查了其组织学和临床结果。我们进行了全面的分子学检查。在同一时期（1992年至2022年）提交的1248例肾上腺病变的组织病理学中，我们发现报告了4例AS/PAS病例（0.32%）。其中两位患者为男性，两位患者为女性，年龄跨度为59-80岁。中位瘤大小为70mm（范围50-100mm），从放射学角度来看，最初怀疑这些病变是源自肾上腺或肾脏的恶性病变。所有病例的激素分析结果均正常。组织学上，3例被注释为细胞型AS或PAS，1例被注释为微囊性AS。使用专注于下一代测序的分子特征化未发现SMARCB1或NF2突变，这些变化先前与其他解剖部位的神经源性瘤相关。所有患者在术后期间均无并发症，并且随访中未显示出任何复发或转移性疾病的迹象。AS/PAS是罕见的肿瘤，大多数情况下为良性，其分子病因与已知的神经源性瘤相关基因突变无关。由于这些肿瘤可能会被误诊为恶性病变，对于放射科医师、内分泌学家、外科医生和病理学家来说，了解这种实体是必不可少的。©2023年作者（们）。

Adrenal schwannoma (AS) and periadrenal schwannoma (PAS) are exceedingly rare Schwann cell tumors that develop from the adrenal medulla and periadrenal peripheral nerves respectively. The underlying genetic events are elusive.We searched our institutional database for AS/PAS cases and reviewed the histology and clinical outcome. Comprehensive molecular work-up was performed.We found reports of 4 AS/PAS cases diagnosed between 1992 and 2022 among the 1248 adrenal lesions submitted for histopathology during the same time period (0.32%). Two patients were male, two were female, and the age span was 59-80 years. Median size was 70 mm (range 50-100 mm), and from a radiology perspective, the lesions were initially suspected of malignant lesions originating from either adrenals or kidneys. Hormonal analyses were normal in all cases. Histologically, three cases were annotated as cellular AS or PAS, and one case was annotated as microcystic AS. Molecular characterization using focused next-generation sequencing did not identify SMARCB1 or NF2 mutations, alterations previously associated to schwannoma at other anatomical sites. The postoperative period was without complications for all patients, and follow-up did not show any signs of relapse or metastatic disease.AS/PAS are rare neoplasms that are most often benign, and the molecular etiology is most likely not related to mutations in established schwannoma-related genes. Since these tumors may be misinterpreted as malignant, knowledge of this entity is essential for radiologists, endocrinologists, surgeons and pathologists.© 2023. The Author(s).