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眼周促纤维增生性恶性黑色素瘤伴眼眶侵犯且病情无法控制:一例报告

Periocular Desmoplastic Malignant Melanoma and Orbital Invasion With Uncontrollable Disease: A Case Report.

作者信息

Ruiz-Arranz Celia, Bengoa-González Álvaro, Bencomo-Villegas Marcelo, Veras-Lista Mariña, Mencía-Gutiérrez Enrique, Laslău Bianca-Maria, Lago-Llinás María-Dolores

机构信息

Department of Ophthalmology, 12 de Octubre Hospital, Complutense University, Madrid, ESP.

Department of Pathology, 12 de Octubre Hospital, Complutense University, Madrid, ESP.

出版信息

Cureus. 2024 May 18;16(5):e60541. doi: 10.7759/cureus.60541. eCollection 2024 May.

Abstract

Primary orbital melanoma and metastatic cutaneous melanoma of the orbit are extremely rare. Desmoplastic melanoma (DM) is an infrequent variant of melanoma that can extend from a superficial location into deep tissues by neurotropic mechanisms. A 78-year-old male was referred to us with a periocular mixed malignant melanoma (spindle cell melanoma with desmoplastic reaction) in his left lower eyelid with uncontrollable disease (orbital and inferior orbital rim invasion) despite treatment. The surgical technique consisted of an extended orbital exenteration, maxillectomy, and ethmoidectomy, with a 2 cm macroscopic surgical margin. We performed a delayed socket reconstruction with a temporalis muscle flap using a transorbital approach. The patient remained disease-free for 1.5 years with a good quality of life since exenteration surgery. At this time, he presented a recurrence in the area of the malar scar with a new orbital invasion, and finally, he died due to mediastinal, pleural, and pulmonary metastasis. The treatment of a cutaneous melanoma arising in the periocular region is a challenging reconstructive problem and it may compromise the globe and visual function.

摘要

原发性眼眶黑色素瘤和眼眶转移性皮肤黑色素瘤极为罕见。促纤维增生性黑色素瘤(DM)是黑色素瘤的一种罕见变体,可通过嗜神经机制从浅表部位延伸至深部组织。一名78岁男性因左眼下睑出现眼周混合性恶性黑色素瘤(伴有促纤维增生性反应的梭形细胞黑色素瘤)前来就诊,尽管接受了治疗,但病情仍无法控制(眼眶及眶下缘侵犯)。手术技术包括扩大眼眶内容剜除术、上颌骨切除术和筛窦切除术,手术切缘肉眼可见为2厘米。我们采用经眶入路,用颞肌瓣进行了延迟性眼窝重建。自眼眶内容剜除术后,患者无病生存1.5年,生活质量良好。此时,他在颧部瘢痕区域出现复发并伴有新的眼眶侵犯,最终因纵隔、胸膜和肺部转移而死亡。眼周区域发生的皮肤黑色素瘤的治疗是一个具有挑战性的重建问题,可能会损害眼球和视觉功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c27e/11181248/7f9e4faed621/cureus-0016-00000060541-i01.jpg

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