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硬腭唾液腺乳腺样分泌癌伴对侧颈淋巴结转移:一例报告

Mammary analogue secretory carcinoma of a salivary gland of the hard palate with contralateral cervical lymph node metastases: A case report.

作者信息

Straub Anton, Linz Christian, Strobel Sabrina, Hartmann Stefan, Hohm Julian, Fuchs Andreas, Müller-Richter Urs, Kübler Alexander, Brands Roman

机构信息

Department of Maxillofacial and Plastic Surgery of The University of Wuerzburg, D-97070 Wuerzburg, Germany.

Institute of Pathology of The University of Wuerzburg, D-97080 Wuerzburg, Germany.

出版信息

Mol Clin Oncol. 2021 Nov;15(5):226. doi: 10.3892/mco.2021.2389. Epub 2021 Sep 6.

Abstract

Mammary analogue secretory carcinoma (MASC) is a rare malignant tumour of the salivary glands, with only few cases reported in the literature to date. Initial preoperative staging is crucial for all patients with an oral malignancy to visualize the tumour, detect lymph node or distant metastases and plan therapeutic interventions. In the case presented herein, radiological imaging revealed a tumour of the right hard palate with suspected positive contralateral lymph nodes. Therefore, local tumour resection comprising hemimaxillectomy and bilateral neck dissection was performed. The diagnosis of MASC was finally based on characteristic histopathological and immunohistochemical findings, such as S100 protein and mammaglobin positivity. The diagnosis of MASC may be challenging, as such findings lack specificity. To confirm the diagnosis, molecular genetic examinations may be performed to detect a highly specific ETV6-NTRK3 fusion gene. Depending on the results of these examinations, surgery, alone or combined with adjuvant radiation or chemoradiation, is the recommended approach. In summary, MASC should be treated similarly to other low-grade salivary gland tumours, such as acinic cell carcinoma, as they exhibit biological and histopathological similarities.

摘要

乳腺样分泌性癌(MASC)是一种罕见的唾液腺恶性肿瘤,迄今为止文献中报道的病例很少。对于所有口腔恶性肿瘤患者,术前初始分期对于观察肿瘤、检测淋巴结或远处转移以及规划治疗干预至关重要。在本文所述病例中,影像学检查发现右侧硬腭有肿瘤,对侧淋巴结疑似阳性。因此,进行了包括半侧上颌骨切除术和双侧颈部清扫术的局部肿瘤切除。MASC的诊断最终基于特征性的组织病理学和免疫组化结果,如S100蛋白和乳腺珠蛋白阳性。MASC的诊断可能具有挑战性,因为这些结果缺乏特异性。为了确诊,可进行分子遗传学检查以检测高度特异性的ETV6-NTRK3融合基因。根据这些检查结果,推荐的方法是单独手术或联合辅助放疗或放化疗。总之,MASC的治疗应与其他低级别唾液腺肿瘤(如腺泡细胞癌)类似,因为它们具有生物学和组织病理学相似性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5643/8506652/00ae35b5849b/mco-15-05-02389-g00.jpg

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