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[小儿涎腺肿瘤及肿瘤样病变]

[Pediatric salivary gland tumors and tumor-like lesions].

作者信息

Agaimy A, Iro H, Zenk J

机构信息

Institut für Pathologie, Universitätsklinikum Erlangen, Krankenhausstraße 8-10, 91054, Erlangen, Deutschland.

Klinik für Hals‑, Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie, Universitätsklinikum Erlangen, Erlangen, Deutschland.

出版信息

Pathologe. 2017 Jul;38(4):294-302. doi: 10.1007/s00292-017-0309-6.

Abstract

Salivary gland tumors and tumor-like lesions in the pediatric population are uncommon. They comprise a heterogeneous group of infectious/inflammatory and neoplastic conditions. Pediatric salivary neoplasms include benign tumors of mesenchymal or epithelial origin as well as malignancies of epithelial (carcinomas), mesenchymal (sarcoma) or hematolymphoid (lymphoma) derivation. Infectious/inflammatory conditions and hematolymphoid malignancies may represent either genuine parenchymal pathology or conditions involving intraglandular lymph nodes of the parotid glands (intraglandular lymphadenopathy and intraglandular nodal lymphomas). Pediatric sialadenitis may be of diverse etiologies including viral (mumps, CMV, HIV, etc.), bacterial, autoimmune (juvenile Sjögren syndrome) or idiopathic (chronic recurrent juvenile sialectatic sialadenitis). Angiomatous lesions (juvenile capillary hemangioma, lymphangioma and vascular malformation) and pleomorphic adenomas represent the most common pediatric benign mesenchymal and benign epithelial tumors, respectively. The vast majority of salivary gland carcinomas in children and adolescents represent low-grade mucoepidermoid carcinomas followed by acinic cell and adenoid cystic carcinomas (together >80% of carcinomas). Other malignant neoplasms include (rhabdomyo-) sarcomas, malignant lymphomas and very rarely sialoblastomas. This long differential diagnosis list of etiologically and biologically highly heterogeneous entities, their shared clinical presentation as "salivary gland enlargement" and the significant differences in their therapeutic strategies and prognosis underline the need for careful assessment to identify the correct diagnosis. Diagnosis is mainly based on a set of typical clinical and imaging features, serological/microbiological findings and, in selected cases, histomorphological characteristics in biopsy specimens.

摘要

儿童唾液腺肿瘤及肿瘤样病变并不常见。它们包括一组异质性的感染性/炎症性和肿瘤性疾病。儿童唾液腺肿瘤包括间叶或上皮来源的良性肿瘤以及上皮性(癌)、间叶性(肉瘤)或血液淋巴性(淋巴瘤)来源的恶性肿瘤。感染性/炎症性疾病和血液淋巴性恶性肿瘤可能代表真正的实质病变,也可能是累及腮腺腺内淋巴结的疾病(腺内淋巴结病和腺内淋巴结淋巴瘤)。儿童涎腺炎可能有多种病因,包括病毒(腮腺炎、巨细胞病毒、人类免疫缺陷病毒等)、细菌、自身免疫(青少年干燥综合征)或特发性(慢性复发性青少年涎腺扩张性涎腺炎)。血管瘤性病变(青少年毛细血管瘤、淋巴管瘤和血管畸形)和多形性腺瘤分别是最常见的儿童良性间叶性和良性上皮性肿瘤。儿童和青少年的唾液腺癌绝大多数为低级别黏液表皮样癌,其次是腺泡细胞癌和腺样囊性癌(两者合计占癌的80%以上)。其他恶性肿瘤包括(横纹肌)肉瘤、恶性淋巴瘤,涎母细胞瘤则极为罕见。这份病因和生物学高度异质性实体的鉴别诊断清单很长,它们共同的临床表现为“唾液腺肿大”,且治疗策略和预后存在显著差异,这突出了仔细评估以明确正确诊断的必要性。诊断主要基于一系列典型的临床和影像学特征、血清学/微生物学检查结果,以及在特定病例中活检标本的组织形态学特征。

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