Mucinous adenocarcinoma of minor salivary glands: a high-grade malignancy prone to lymph node metastasis
Mucinous adenocarcinoma of minor salivary glands: a high-grade malignancy prone to lymph node metastasis
复制标题
小唾液腺粘液性腺癌：易发生淋巴结转移的高级别恶性肿瘤
作者：
F. Ide;K. Mishima;A. Tanaka;I. Saito;K. Kusama
唾液腺粘液腺癌（MAC）是一种很少见且知之甚少的肿瘤。我们描述了另外两例表现为老年女性唇部病变的病例。预后很差，一名患者死亡，一名患者出现复发/转移性疾病。将这些与文献中的 19 个病例结合起来，发现口内 MAC 主要发生在老年人（平均 65 岁）的上颚（43%），男性略多（59%）。肿瘤进展缓慢（>2年），大多数（60%）在晚期才被诊断出来。局部复发常见（33%），颈淋巴结转移和远处转移常见（分别为 63% 和 29%）。大约一半的患者 (47%) 在 6 年内死于肿瘤。从组织学和免疫组织化学角度来看，MAC 缺乏腺泡、肌上皮和神经内分泌表型。细胞角蛋白 7 和 20 以及雌激素和孕激素受体的某种组合可能有助于区分原发性和转移性粘液癌。小唾液腺 MAC 属于高级别类别，具有显着的局部复发、淋巴结转移和致命结果的风险。
Salivary gland mucinous adenocarcinoma (MAC) is a rarely encountered and poorly understood tumor. We describe two additional cases presenting as a lip lesion of older women. The prognosis was dismal with one death and one patient with recurrent/metastatic disease. Combining these with 19 cases in the literature unveiled that intraoral MAC occurred primarily in the palate (43%) of elderly individuals (average 65 years) with slight male preponderance (59%). Tumor evolved slowly (>2 years) and most (60%) were diagnosed at an advanced stage. Local recurrences were common (33%) and cervical lymph node and distant metastases were frequent (63% and 29%, respectively). About half the patients (47%) died of tumor within 6 years. Both histologically and immunohistochemically, MAC lacked acinar, myoepithelial, and neuroendocrine phenotypes. Cytokeratins 7 and 20 and estrogen and progesterone receptors, in some combination, may assist to distinguish primary versus metastatic mucinous carcinoma. Minor salivary gland MAC belongs to a high-grade category with a significant risk of local recurrence, lymph node metastasis, and fatal outcome.