Orbital Oncology & Ophthalmic Plastic Surgery, Department of Plastic Surgery.
Department of Pathology.
Ophthalmic Plast Reconstr Surg. 2020 Sep/Oct;36(5):497-502. doi: 10.1097/IOP.0000000000001625.
To review the clinical presentation, treatment, and prognosis of patients with malignant mixed tumor (carcinoma ex pleomorphic adenoma) of the lacrimal gland.
Clinical records and radiographic images were reviewed for patients with malignant mixed tumor of the lacrimal gland treated at the center during 2008-2019.
The study included 9 patients (6 men, 3 women) aged 17-66 years (median age, 56 years). Six had primary malignant mixed tumor with no history of orbital lesions, and 3 had previously been diagnosed with pleomorphic adenoma. Tumor, Node, Metastasis classification per the eighth edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual were T1aN0M0 in 2 patients, T2aN0M0 in 3 patients, T4bN0M0 in 2 patients, and T4cN0M0 in 2 patients. Two patients underwent orbital exenteration, 6 patients underwent eye-sparing surgery, and 1 patient had an unresectable tumor because of cavernous sinus extension. All patients received radiotherapy (intensity-modulated radiotherapy in 3 and proton therapy in 6). All patients received chemotherapy, 8 concurrently with radiotherapy and 1 after radiotherapy. The median follow-up time was 70 months. At last contact, 6 patients were alive without evidence of disease; 2 had died of disease, 1 of distant metastasis, and the other of cavernous sinus invasion.
The findings suggest that de novo malignant mixed tumor of the lacrimal gland is more common than disease that results from transformation after incomplete resection of lacrimal gland pleomorphic adenoma. Most cases can be treated with eye-sparing surgery and radiation unless skull base extension is present.
回顾泪腺恶性混合瘤（癌源于多形性腺瘤）患者的临床表现、治疗方法和预后。
对 2008 年至 2019 年在中心治疗的泪腺恶性混合瘤患者的临床记录和影像学图像进行了回顾。
本研究纳入 9 例（6 男 3 女）患者，年龄 17-66 岁（中位年龄 56 岁）。6 例患者为原发性恶性混合瘤，无眼眶病变史，3 例患者曾被诊断为多形性腺瘤。根据第八版美国癌症联合委员会（AJCC）癌症分期手册，肿瘤、淋巴结、远处转移（TNM）分类为 2 例 T1aN0M0，3 例 T2aN0M0，2 例 T4bN0M0，2 例 T4cN0M0。2 例患者行眶内容剜除术，6 例患者行保眼手术，1 例患者因海绵窦延伸而无法切除肿瘤。所有患者均接受放疗（3 例采用调强放疗，6 例采用质子治疗）。所有患者均接受化疗，8 例与放疗同时进行，1 例在放疗后进行。中位随访时间为 70 个月。末次随访时，6 例患者无病生存；2 例患者死于疾病，1 例死于远处转移，另 1 例死于海绵窦侵犯。
研究结果表明，原发于泪腺的恶性混合瘤比多形性腺瘤切除不完全后恶变更为常见。大多数病例可以通过保眼手术和放疗治疗，除非存在颅底侵犯。
