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揭示小涎腺乳腺样分泌癌的罕见性——一例报告

Unmasking the rarity of mammary analogue secretory carcinoma of the minor salivary gland - A case report.

作者信息

Sakhariya Samkit V, Chincholkar Anuja, Tidke Sanika, Setiya Sneha, Mane Noopur, Markand Megha

机构信息

Dept. of Oral and Maxillofacial Surgery, Dr. D. Y. Patil Dental College and Hospital, Dr. D.Y. Patil Vidyapeeth, Pune, Maharashtra, India.

Dept. of Oral and Maxillofacial Surgery, Dr. D. Y. Patil Dental College and Hospital, Dr. D.Y. Patil Vidyapeeth, Pune, Maharashtra, India.

出版信息

Int J Surg Case Rep. 2024 Nov;124:110374. doi: 10.1016/j.ijscr.2024.110374. Epub 2024 Sep 30.

Abstract

INTRODUCTION

Mammary analogue secretory carcinoma (MASC) of the salivary gland was first described by Skálová et al. in 2010. It is often associated with a translocation, t(12;15)(p13;q25), which results in the fusion gene ETV6-NTRK3. Major salivary glands, primarily the parotid gland, are involved in 70 % of cases of MASC, while small salivary glands are involved in less than 25 % of cases. This report aims to consolidate in unveiling, diagnosing, and managing the rarity of MASC in the minor salivary gland and its existing knowledge and encourage new research on this increasingly important salivary gland malignancy.

PRESENTATION OF THE CASE

A 27-year-old female reported with a complaint of swelling on the right cheek region of face since 10 weeks. On bimanual palpation, a soft lobulated mass was appreciated beneath the healthy mucosal layer. The radiographic image (orthopantomogram) showed no obvious calcified mass. An excisional biopsy was planned and performed under local anesthesia. Microscopic and immunohistochemistry confirmed the tumor to be a MASC of minor salivary gland.

DISCUSSION

Due to their infrequency and multiplicity of histopathology, MASC presents difficulty in diagnosis. A key to determining diagnostic criteria for MASC is to study cellular morphology, cytoplasmic filament expression, and ultrastructural features of the tumor and apply this information to defining MASC.

CONCLUSION

MASC is an important molecularly defined entity of the salivary gland with low-grade malignant potential. Correct diagnosis is essential for appropriate treatment and will help to provide better information about this potentially low-grade malignant salivary gland neoplasm.

摘要

引言

涎腺乳腺样分泌癌(MASC)于2010年由斯卡洛娃等人首次描述。它常与t(12;15)(p13;q25)易位相关,该易位导致融合基因ETV6-NTRK3。70%的MASC病例累及大涎腺,主要是腮腺,而小涎腺受累病例不到25%。本报告旨在汇总揭示、诊断和处理小涎腺中罕见的MASC及其现有知识,并鼓励对这种日益重要的涎腺恶性肿瘤开展新的研究。

病例介绍

一名27岁女性自10周前起主诉右侧面颊部肿胀。双手触诊时,在健康黏膜层下方可触及一个柔软的分叶状肿块。影像学图像(曲面断层片)未显示明显的钙化肿块。计划并在局部麻醉下进行切除活检。显微镜检查和免疫组织化学证实该肿瘤为小涎腺的MASC。

讨论

由于MASC罕见且组织病理学表现多样,其诊断存在困难。确定MASC诊断标准的关键是研究肿瘤的细胞形态、细胞质细丝表达和超微结构特征,并将这些信息应用于MASC的定义。

结论

MASC是一种重要的涎腺分子定义实体,具有低级别恶性潜能。正确诊断对于恰当治疗至关重要,将有助于提供有关这种潜在低级别恶性涎腺肿瘤的更好信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/357b/11471663/e087ef309768/gr1.jpg

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