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舌腺样囊性癌:一例突出罕见疾病外科治疗的病例研究

Adenoid cystic carcinoma of the tongue: A case study highlighting surgical management of an exceptionally rare entity.

作者信息

Khademi Bijan, Soltani Sajjad, Yousufzai Shayan, Yousefi Alireza, Golshannia Mohammad Ehsan, Mardani Zhale

机构信息

Head and neck Surgery Department, Khalili Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Head and neck Surgery Department, Khalili Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Int J Surg Case Rep. 2025 Feb;127:110915. doi: 10.1016/j.ijscr.2025.110915. Epub 2025 Jan 20.

Abstract

BACKGROUND

Adenoid cystic carcinoma (ACC) is a rare malignant tumor that accounts for <1 % of head and neck malignancies, with an estimated involvement of the tongue at approximately 3 %.

CASE PRESENTATION

This report presents a case of a 42-year-old male with no significant history of tobacco or alcohol use, who developed a painless, progressively enlarging mass on the ventral surface of his tongue over the course of eight months. Initial surgical resection confirmed the diagnosis of ACC, which necessitated a right hemiglossectomy followed by radiotherapy to address potential residual disease.

DISCUSSION

ACC of the tongue presents unique clinical challenges due to its rarity and aggressive nature. The lack of traditional risk factors indicates that ACC can arise in low-risk populations, underscoring the necessity for heightened awareness among healthcare professionals to facilitate early detection. Surgical resection remains the standard treatment; however, achieving negative margins is challenging due to the tumor's infiltrative characteristics, which can lead to local recurrence. Histologically, ACC is classified as a biphasic neoplasm often associated with perineural invasion, and high-grade transformation increases the risk of metastasis. Postoperative radiotherapy is recommended to reduce the risk of recurrence, particularly in cases exhibiting unfavorable histological features. A personalized treatment strategy that takes into account tumor characteristics and patient-specific factors is essential for effective management.

CONCLUSION

This case underscores the importance of vigilance in identifying symptoms of ACC of the tongue, even in low-risk individuals. It advocates for a multidisciplinary approach that includes surgical intervention and adjuvant radiotherapy to achieve optimal outcomes.

摘要

背景

腺样囊性癌(ACC)是一种罕见的恶性肿瘤,占头颈部恶性肿瘤的比例不到1%,估计舌部受累率约为3%。

病例报告

本报告介绍了一名42岁男性患者,无明显吸烟或饮酒史,在八个月的时间里,其舌腹面出现了一个无痛性、逐渐增大的肿块。初次手术切除确诊为ACC,这需要进行右半舌切除术,随后进行放疗以处理潜在的残留病灶。

讨论

舌部ACC因其罕见性和侵袭性而带来独特的临床挑战。缺乏传统风险因素表明ACC可发生在低风险人群中,这凸显了医疗专业人员提高认识以促进早期发现的必要性。手术切除仍然是标准治疗方法;然而,由于肿瘤的浸润性特征,实现切缘阴性具有挑战性,这可能导致局部复发。从组织学上看,ACC被归类为一种常伴有神经周浸润的双相肿瘤,高级别转化会增加转移风险。建议术后放疗以降低复发风险,特别是在具有不良组织学特征的病例中。考虑肿瘤特征和患者特定因素的个性化治疗策略对于有效管理至关重要。

结论

本病例强调了即使在低风险个体中,警惕舌部ACC症状的重要性。它提倡采用包括手术干预和辅助放疗在内的多学科方法以实现最佳治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5666/11803258/695e1e23c0d5/gr1.jpg

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