Kaliki Swathi, Singh Swati, Gowrishankar Swarnalata, Reddy Vijay Anand P
*Operation Eyesight Universal Institute for Eye Cancer, L. V. Prasad Eye Institute, Hyderabad, India; and †Apollo Hospitals, Jubilee Hills, Hyderabad, India.
Cornea. 2017 Jun;36(6):743-746. doi: 10.1097/ICO.0000000000001179.
To discuss the association between ocular surface squamous neoplasia (OSSN) and Papillon-Lefèvre syndrome (PLS) and present the long-term outcome in a patient with these diseases.
Case report.
A 14-year-old boy presented with a raised pigmented mass lesion at the limbus in the right eye, which was clinically suggestive of OSSN. He also had palmoplantar hyperkeratosis and periodontosis suggestive of PLS. Excision biopsy of the lesion confirmed the diagnosis of OSSN. He was free of tumor recurrence for 2 years and was lost to follow-up thereafter. Seven years later, the patient presented with diffuse tumor recurrence with orbital extension in the right eye, regional lymph node metastasis, and ipsilateral parotid gland infiltration. The patient underwent exenteration of the right orbital contents, right parotidectomy, and radical neck dissection followed by concomitant external beam radiotherapy and systemic chemotherapy. Eleven years from initial presentation, the patient was detected to have OSSN in the contralateral eye and was managed by wide excision biopsy and adjuvant cryotherapy. One year after detection of OSSN in the left eye, the patient was found to have systemic metastases to the lungs and brain. The patient died of the disease within 3 months of detection of systemic metastasis.
OSSN in PLS is associated with poor prognosis.
探讨眼表鳞状上皮肿瘤（OSSN）与帕皮永-勒费弗尔综合征（PLS）之间的关联，并介绍一名患有这些疾病患者的长期预后情况。
病例报告。
一名14岁男孩右眼角膜缘出现一个隆起的色素性肿块病变，临床提示为OSSN。他还伴有掌跖角化过度和牙周病，提示为PLS。病变切除活检确诊为OSSN。他在2年内无肿瘤复发，此后失访。7年后，患者右眼出现弥漫性肿瘤复发并累及眼眶、区域淋巴结转移及同侧腮腺浸润。患者接受了右眼眶内容物剜除术、右腮腺切除术和根治性颈清扫术，随后进行了同步外照射放疗和全身化疗。自初次就诊11年后，患者对侧眼被检测出患有OSSN，通过广泛切除活检和辅助冷冻治疗进行处理。在左眼检测出OSSN 1年后，患者被发现出现肺和脑的全身转移。患者在检测出全身转移后3个月内死于该疾病。
PLS中的OSSN与预后不良相关。
