儿童唾液腺上皮性肿瘤:单机构经验,重点关注组织学谱和临床结局。

Salivary gland epithelial neoplasms in pediatric population: a single-institute experience with a focus on the histologic spectrum and clinical outcome.

作者信息

Xu Bin, Aneja Amandeep, Ghossein Ronald, Katabi Nora

机构信息

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, 10065.

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, 10065.

出版信息

Hum Pathol. 2017 Sep;67:37-44. doi: 10.1016/j.humpath.2017.07.007. Epub 2017 Jul 21.

Abstract

Salivary gland epithelial neoplasms are rare in children and adolescents, with only a handful of large series having been published. A retrospective study was conducted for 57 cases in patients 20 years or younger. The tumors were located in the parotid (n=36), submandibular gland (n=7), and minor salivary glands (n=14). Nineteen (33%) tumors were pleomorphic adenoma, whereas the remaining (67%) were malignant. The histologic types of carcinomas were mucoepidermoid carcinoma (MEC, n=19, 33%), acinic cell carcinoma (n=7, 12%), adenoid cystic carcinoma (n=6, 11%), secretory carcinoma (mammary analogue) (SC, n=4, 7%), and myoepithelial carcinoma (n=2, 4%). Ninety-three percent (13/14) of the minor and 58% (25/43) of the major salivary gland tumors were malignant. A 7-year-old girl (2%) with a high-grade MEC died from her disease because of uncontrollable locoregional recurrence. Seven patients (16%) developed recurrence including 2 distant metastases from adenoid cystic carcinoma and 6 locoregional recurrences (2 pleomorphic adenomas, 1 SC, 1 myoepithelial carcinoma, 1 adenoid cystic carcinoma, and 1 MEC). The following parameters were associated with decreased disease-free survival in malignant tumors: elevated mitotic index of >4/10 high-power fields (log-rank test, P<.001), and advanced American Joint Committee on Cancer pT (P=.029) and pN stage (P<.001). In conclusion, myoepithelial carcinoma and SC can occur in the pediatric population and should be considered in the differential diagnosis. Salivary gland malignancies in children appear to have better clinical outcome, associated with a 10-year recurrence-free survival rate of 74% and a 10-year disease-specific survival of 94%.

摘要

涎腺上皮性肿瘤在儿童和青少年中较为罕见,仅有少数大型系列研究发表。对20岁及以下的57例患者进行了一项回顾性研究。肿瘤位于腮腺(n = 36)、下颌下腺(n = 7)和小涎腺(n = 14)。19例(33%)肿瘤为多形性腺瘤,其余(67%)为恶性肿瘤。癌的组织学类型为黏液表皮样癌(MEC,n = 19,33%)、腺泡细胞癌(n = 7,12%)、腺样囊性癌(n = 6,11%)、分泌性癌(乳腺型)(SC,n = 4,7%)和肌上皮癌(n = 2,4%)。小涎腺肿瘤的93%(13/14)和大涎腺肿瘤的58%(25/43)为恶性。一名7岁女孩(2%)患有高级别MEC,因局部区域复发无法控制而死于该病。7例患者(16%)出现复发,包括2例腺样囊性癌远处转移和6例局部区域复发(2例多形性腺瘤、1例SC、1例肌上皮癌、1例腺样囊性癌和1例MEC)。以下参数与恶性肿瘤无病生存期降低相关:有丝分裂指数>4/10个高倍视野升高(对数秩检验,P<.001),以及美国癌症联合委员会晚期pT(P =.029)和pN分期(P<.001)。总之,肌上皮癌和SC可发生于儿童人群,在鉴别诊断中应予以考虑。儿童涎腺恶性肿瘤似乎具有较好的临床结局,10年无复发生存率为74%,10年疾病特异性生存率为94%。

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