Adnexal Service, Moorfields Eye Hospital, London, United Kingdom.
Ophthalmic Plast Reconstr Surg. 2021;37(6):576-582. doi: 10.1097/IOP.0000000000001957.
To report the characteristics and long-term outcomes of patients presenting with carcinoma ex-pleomorphic adenoma (CXPA) or malignant mixed tumor of the lacrimal gland.
A retrospective case-note review for patients with CXPA, seen at Moorfields Eye Hospital between 1985 and 2018, was performed for demographics, presentation, imaging, histopathology, management, and outcome.
Twenty-six patients (11 male; 42%) presented at a mean age of 46 years (median 46.4; range 24-81), with average symptom(s) duration of 67 months (median 24 months; range 6 weeks-53 years). The commonest symptoms were proptosis (13 patients; 50%), orbital ache or pain (11/26, 42%), having noted a lump in the upper eyelid (8/26, 31%), and diplopia (7/26, 27%). About 90% of patients had nonaxial globe displacement, over a half had a palpable mass and 3-quarters had reduced motility. Adenocarcinoma was the commonest malignant component of the tumor, occurring in 16 (62%) of the patients, followed by adenoid cystic carcinoma in 5 patients (19%); 10/26 (28%) tumors showed significant invasion on histological examination. A quarter of the patients had tumor-related death at an average of 2.8 years after diagnosis (median 2.5; range 1.5-4.4 years), and the overall probability of survival for the whole cohort was 63% at both 5 years and 10 years after treatment. The prognosis for CXPA was significantly worse with a history of prior incompletely excised pleomorphic adenoma (p < 0.001), a markedly invasive malignant component (p = 0.001), evidence of perineural invasion (p = 0.042), and an American Joint Committee on Cancer tumor size of T3 or greater (p = 0.024).
Intact excision of CXPAs that do not display histological evidence of extension beyond the tumor pseudocapsule have a good outcome, similar to that for intact excision of benign pleomorphic adenomas. In contrast, our patients with markedly invasive CXPA had a tendency to local recurrence or distant metastasis, and tumor-related death occurred in about two-thirds.
报告表现为癌型多形性腺瘤（CXPA）或泪腺恶性混合瘤的患者的特征和长期预后。
对 1985 年至 2018 年间在 Moorfields 眼科医院就诊的 CXPA 患者进行回顾性病例记录回顾，以获取人口统计学、表现、影像学、组织病理学、治疗和结局。
26 例患者（男性 11 例；42%）的平均年龄为 46 岁（中位数 46.4；范围 24-81），平均症状持续时间为 67 个月（中位数 24 个月；范围 6 周-53 年）。最常见的症状是眼球突出（13 例；50%）、眶痛或疼痛（11/26，42%）、上眼睑有肿块（8/26，31%）和复视（7/26，27%）。大约 90%的患者有非轴向眼球移位，超过一半的患者有可触及的肿块，四分之三的患者运动受限。腺癌是肿瘤最常见的恶性成分，发生在 16 例（62%）患者中，其次是腺样囊性癌 5 例（19%）；10/26（28%）肿瘤在组织学检查中显示明显侵袭。四分之一的患者在诊断后平均 2.8 年后（中位数 2.5；范围 1.5-4.4 年）因肿瘤相关死亡，整个队列的总体生存概率在治疗后 5 年和 10 年均为 63%。有既往不完全切除多形性腺瘤病史（p<0.001）、恶性成分明显侵袭（p=0.001）、神经周围侵犯证据（p=0.042）和美国癌症联合委员会肿瘤大小 T3 或更大（p=0.024）的患者，CXPA 的预后明显更差。
未显示肿瘤假包膜外扩展组织学证据的 CXPA 完整切除后预后良好，与良性多形性腺瘤完整切除后相似。相比之下，我们的 CXPA 患者侵袭性明显，有局部复发或远处转移的倾向，约三分之二的患者发生肿瘤相关死亡。
