Saleh Daniyah, Al Ghamdi Doaa
Department of Anatomic Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.
Department of Anatomic Pathology and Laboratory Medicine, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.
Case Rep Pathol. 2020 Jun 30;2020:5837659. doi: 10.1155/2020/5837659. eCollection 2020.
Salivary gland tumors are uncommon in children. They consist of variable histopathological subtypes of benign and malignant tumors. EMC is a discrete entity among the WHO classification of salivary gland tumors since 1991. EMC is considered a low-grade malignant salivary gland tumor arising from intercalated ducts. Typically, it affects an adult female individual. Surgical resection with a negative margin is the mainstay treatment option. EMC has a potential for metastasis with a high rate of recurrence. Based on the available English literature, two cases of EMC diagnosed in a pediatric age group have been reported. Therefore, we describe the third EMC that developed in the left parotid gland of a young child. The diagnosis of EMC was established through histopathological examination of the total parotidectomy specimen and neck lymph node dissection, together with ancillary studies. Later, the patient suffered from cervical lymph node enlargement due to metastasis in which FNAB was taken. Metastasis from the known EMC was suspected with cytomorphological features in smears and cell block. Immunohistochemistry markers for the biphasic components were supportive of EMC. Due to advanced disease, the patient necessitated a concomitant treatment of radiochemotherapy. Besides, there was radiological evidence of bilateral multiple lung metastatic nodules. However, a biopsy was not sent for pathological confirmation.
涎腺肿瘤在儿童中并不常见。它们由良性和恶性肿瘤的多种组织病理学亚型组成。自1991年以来，上皮-肌上皮癌（EMC）在世界卫生组织涎腺肿瘤分类中是一个独立的实体。EMC被认为是一种起源于闰管的低级别恶性涎腺肿瘤。通常，它影响成年女性个体。切缘阴性的手术切除是主要的治疗选择。EMC有转移的可能性且复发率高。根据现有的英文文献，已报道了两例在儿童年龄组中诊断出的EMC病例。因此，我们描述了第三例发生在一名幼儿左腮腺的EMC。通过对腮腺全切除术标本和颈部淋巴结清扫标本进行组织病理学检查以及辅助研究，确立了EMC的诊断。后来，患者因转移出现颈部淋巴结肿大，为此进行了细针穿刺抽吸活检（FNAB）。根据涂片和细胞块中的细胞形态学特征，怀疑已知的EMC发生了转移。双相成分的免疫组织化学标志物支持EMC的诊断。由于疾病进展，患者需要同步进行放化疗。此外，有影像学证据显示双侧肺部有多个转移结节。然而，未送检活检进行病理确诊。
