Shet Tanuja, Arora Brijesh, Laskar Siddhartha, Basak Rajan, Kane Shubhada, Kurkure Purna
Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India.
Pediatr Dev Pathol. 2009 Mar-Apr;12(2):152-5. doi: 10.2350/07-12-0395.1. Epub 2008 May 5.
We describe the first case of an lymphoepithelioma-like carcinoma (LEC) of the mandible with a view to discuss probable histogenesis from salivary gland inclusions in the mandible, effective therapy, the unusual pattern of nodal metastases, and association with the Epstein-Barr virus. An 11-year-old boy presented with a lytic lesion in the left mandible and bilateral neck node enlargement. Histologically, the biopsied metastatic neck nodes showed sinusoids distended with histiocytes and single large atypical cells that mimicked Hodgkin lymphoma. The mandibular tumor on biopsy showed morphology similar to an undifferentiated carcinoma or LEC of nasopharynx with nuclear Epstein-Barr virus-associated in situ hybridization signals. The patient received ifosfamide, cisplatin, and etoposide chemotherapy followed by a hemimandibulectomy, which did not reveal any residual tumor. Postoperative radiotherapy was completed, and 3 years later the patient is alive and free of disease.
我们描述了首例下颌骨淋巴上皮瘤样癌（LEC）病例，旨在探讨其可能起源于下颌骨唾液腺包涵体的组织发生、有效治疗方法、不寻常的淋巴结转移模式以及与爱泼斯坦-巴尔病毒的关联。一名11岁男孩出现左下颌骨溶骨性病变及双侧颈部淋巴结肿大。组织学检查显示，活检的颈部转移淋巴结中，窦状隙内充满组织细胞和单个大的非典型细胞，类似霍奇金淋巴瘤。下颌骨肿瘤活检显示形态类似于未分化癌或鼻咽癌的LEC，且有核爱泼斯坦-巴尔病毒相关原位杂交信号。患者接受了异环磷酰胺、顺铂和依托泊苷化疗，随后进行了半侧下颌骨切除术，未发现任何残留肿瘤。术后完成了放疗，3年后患者存活且无疾病。
