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具有显著微囊变和黏液样改变、酷似胸腺黏液表皮样癌的非典型胸腺瘤(WHO B3型):5例临床病理及免疫组化研究

Atypical Thymoma (WHO Type B3) with Prominent Microcystic and Mucoid Changes Mimicking Thymic Mucoepidermoid Carcinoma: A Clinicopathological and Immunohistochemical Study of Five Cases.

作者信息

Weissferdt Annikka, Moran Cesar A

机构信息

Department of Anatomic Pathology, The University of Texas MD Anderson Cancer Center, Houston, USA.

出版信息

Int J Surg Pathol. 2025 Apr;33(2):394-398. doi: 10.1177/10668969241266935. Epub 2024 Aug 16.

Abstract

Five atypical thymomas (WHO type B3) with prominent microcystic and mucoid changes are presented. The patients were four men and one woman between the ages of 57 and 72 years. The patients presented with non-specific symptoms of cough, chest pain, and dyspnea. None of the patients had a history of myasthenia gravis. Diagnostic imaging revealed the presence of anterior mediastinal masses and surgical resection was accomplished in all patients. Macroscopically, the tumors ranged in size from 3.5 to 5.0 cm in greatest diameter; four of these were well circumscribed but unencapsuled, tan colored tumors without evidence of necrosis, hemorrhage, or gross cystic changes. One tumor had more infiltrative borders and was involving the mediastinal pleura. Microscopically, the low power view was characterized by prominent microcysts that were filled with a mucoid granular material. Higher magnification demonstrated a homogenous epithelial proliferation with mild cytologic atypia but lacking mitotic activity. Focal areas of squamoid differentiation were identified but perivascular spaces were absent. Histochemical staining confirmed mucinous material in the microcysts but no intracytoplasmic mucin. Immunohistochemical stains showed positive staining of the tumor cells with keratin AE1/AE3, keratin 5/6, p63, and p40. No terminal deoxynucleotidyl transferase+/CD3 + immature lymphocytes were identified. Clinical follow-up demonstrated that four patients have remained alive without recurrence while one patient was lost to follow-up. This report highlights histological features in atypical thymoma that may be confused with other tumors, especially thymic mucoepidermoid carcinoma. Separation of these tumors may be important for patient management and prognosis.

摘要

本文报告了5例具有显著微囊样和黏液样改变的非典型胸腺瘤(WHO B3型)。患者包括4名男性和1名女性,年龄在57至72岁之间。患者表现为咳嗽、胸痛和呼吸困难等非特异性症状。所有患者均无重症肌无力病史。诊断性影像学检查显示前纵隔肿块,所有患者均接受了手术切除。大体上,肿瘤最大直径为3.5至5.0 cm;其中4例边界清晰但无包膜,呈黄褐色,无坏死、出血或明显囊性变。1例肿瘤边界更具浸润性,累及纵隔胸膜。显微镜下,低倍视野可见显著的微囊,内充满黏液样颗粒物质。高倍镜下可见均匀的上皮细胞增生,细胞轻度异型性,但无有丝分裂活性。可见局灶性鳞状分化区域,但无血管周围间隙。组织化学染色证实微囊内有黏液物质,但无胞质内黏液。免疫组织化学染色显示肿瘤细胞对角蛋白AE1/AE3、角蛋白5/6、p63和p40呈阳性染色。未发现末端脱氧核苷酸转移酶+/CD3 +未成熟淋巴细胞。临床随访显示,4例患者存活且无复发,1例失访。本报告强调了非典型胸腺瘤可能与其他肿瘤,尤其是胸腺黏液表皮样癌混淆的组织学特征。区分这些肿瘤对于患者管理和预后可能很重要。

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