Central Mucoepidermoid Carcinoma: A Clinicopathologic and Immunohistochemical Study of 39 Chinese Patients
Central Mucoepidermoid Carcinoma: A Clinicopathologic and Immunohistochemical Study of 39 Chinese Patients
复制标题
中央粘液表皮样癌：39 例中国患者的临床病理学和免疫组织化学研究
DOI：
10.1097/pas.0b013e31822be0df
复制
发表时间：
2012-01-01
影响因子：
5.6
通讯作者：
Li, Tie-Jun
中科院分区：
文献类型：
--
作者：
Zhou, Chuan-Xiang;Chen, Xin-Ming;Li, Tie-Jun
中枢性黏液表皮样癌是一种罕见的颌骨内肿瘤。为明确中心性MEC的临床病理特点和发病机制，收集并分析了39例中枢性MEC的临床病理表现和随访资料。男16例，女23例(中位年龄43岁)。上颌骨16例，下颌骨23例。X线表现多为单房性或多房性透影伴骨质破坏，7例为散在钙化。14例病灶边缘模糊或弥漫，25例边缘较清晰。大多数病例(26/39)为低度恶性微血管内皮细胞，13例为中至高度恶性。有35名患者获得了随访数据，中位数为36个月。所有病例均为原发肿瘤，局部复发8例，其中低度肿瘤占75.0%。4例出现区域淋巴结转移，1例发生远处转移。11例有颌骨囊肿临床病史的患者中，8例最初表现为典型的牙源性囊肿，伴有局部MEC样增生。综上所述，中心性MEC最可能的发病机制是牙源性囊肿上皮衬里的肿瘤性转化，其诊断应基于临床、放射学和组织病理学结果。角蛋白的免疫组织化学图谱有助于鉴别诊断。根治性手术是治疗的首选，而放疗或化疗的作用仍有争议，需要仔细的长期随访。
Central mucoepidermoid carcinoma (MEC) is a rare neoplasm arising intraosseously in the jaws. To clarify the clinicopathologic profile and pathogenesis of central MEC, clinicopathologic findings and follow-up data of 39 cases were collected and analyzed. There were 16 male and 23 female patients (median age, 43 y). Sixteen cases affected the maxilla, and 23 occurred in the mandible. Radiographically, most cases (32 of 39) showed a unilocular or multilocular radiolucency with bone destruction, and 7 were found with scattered calcification. The margins of the lesions were ill defined or diffused in 14 cases and relatively well defined in 25 cases. Most cases (26 of 39) were classified as low-grade MECs, whereas 13 were moderate-to-high grade. Follow-up data were available for 35 patients with a median period of 36 months. All cases were found to be primary; local recurrence occurred in 8 cases, most (75.0%) of which were low-grade tumors. Four cases showed regional lymph node metastasis, and I developed distant metastasis. Of 11 cases with a clinical history of the jaw cyst, 8 initially showed a typical odontogenic cyst with local MEC-like proliferation. In summary, the most likely pathogenesis of central MEC is neoplastic transformation of the epithelial lining of an odontogenic cyst, diagnosis of which should be based on clinical, radiographic, and histopathologic findings. The immunohistochemical profile of keratins is helpful in differential diagnosis. Radical surgery is the treatment of choice, whereas the role of radiotherapy or chemotherapy is still controversial, and careful long-term follow-up is necessary.