De Groot V, Verhelst E, Hogendoorn P C W, de Keizer R J W
Department of Ophthalmology, University Hospital Antwerp, Antwerp, Belgium.
Antwerp University, Faculty of Medicine, Antwerp, Belgium.
Ocul Oncol Pathol. 2019 Aug;5(5):333-339. doi: 10.1159/000494497. Epub 2019 Jan 24.
To describe the differential diagnosis and management of a rare conjunctival malignancy.
A 79-year-old man presented with a conjunctival mass at the limbus. Excisional biopsy revealed a malignant mesenchymal tumor with myogenic differentiation. Six months later, three suspect lesions developed at the border of the previous excision. Pathological diagnosis pointed to a leiomyosarcoma. Adjuvant radiotherapy with strontium-90 brachytherapy was applied. After 3 years, there was neither recurrence nor distant metastases. A literature review revealed 11 cases of conjunctival leiomyosarcoma.
All 12 cases seemed to originate at the limbal conjunctiva. Scleral invasion was found only in one patient with multiple previous resections. Corneal invasion was reported in two patients. Four patients had a globe-sparing resection. In two of them, margins were not tumor free and additional brachytherapy gave a tumor-free follow-up of 1 and 3 years. Four cases underwent an exenteration.
Primary conjunctival leiomyosarcoma is a rare tumor with a favorable prognosis due to early detection and consequently limited size. Diagnosis involves histopathological investigation including immunohistochemistry. If possible, complete resection has the best prognosis. Adjunctive radiotherapy can be effective when the margins are not free and should be considered.
描述一种罕见的结膜恶性肿瘤的鉴别诊断和治疗方法。
一名79岁男性患者，角膜缘处出现结膜肿物。切除活检显示为具有肌源性分化的恶性间叶性肿瘤。6个月后，在先前切除边缘处出现3个可疑病变。病理诊断为平滑肌肉瘤。采用锶-90近距离放射治疗进行辅助放疗。3年后，既无复发也无远处转移。文献回顾发现11例结膜平滑肌肉瘤病例。
所有12例似乎均起源于角膜缘结膜。仅1例曾多次手术切除的患者出现巩膜侵犯。2例患者有角膜侵犯。4例患者进行了眼球保留切除术。其中2例切缘有肿瘤残留，追加近距离放射治疗后分别获得1年和3年无瘤随访。4例患者进行了眼球摘除术。
原发性结膜平滑肌肉瘤是一种罕见肿瘤，由于早期发现且肿瘤大小受限，预后良好。诊断需进行包括免疫组化在内的组织病理学检查。若可能，完整切除预后最佳。当切缘有肿瘤残留时，辅助放疗可能有效，应予以考虑。
