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着色性干皮病患者的结膜黑色素瘤:一系列四例。

Conjunctival melanoma in patients with xeroderma pigmentosum: a series of four cases.

机构信息

Operation Eyesight Universal Institute for Eye Cancer (VHV, SK), L V Prasad Eye Institute, Hyderabad, 500034, India.

出版信息

Int Ophthalmol. 2020 May;40(5):1143-1146. doi: 10.1007/s10792-019-01279-2. Epub 2020 Jan 13.

Abstract

PURPOSE

To study the demographic features, treatment, histopathology, and outcomes in patients of xeroderma pigmentosum (XP) with conjunctival melanoma.

METHODS

Retrospective case series.

RESULTS

The median age at presentation was 18 years (range 9-30 years). There were three females and one male patient presenting with a median duration of symptoms of 3 months (range 1-60 months). The tumor was located in the bulbar conjunctiva in all 4 patients. All patients had corneal involvement by the tumor. The median tumor basal diameter was 7 mm (range 4-15 mm). Wide tumor excisional biopsy with alcohol keratoepithelectomy, cryotherapy to the free margins, and amniotic membrane grafting was done in three patients. One patient underwent orbital exenteration for extensive tumor. One patient also received adjuvant plaque brachytherapy for microscopic residual tumor. Over a median follow-up of 22 months (range 2-101 months), there were no recurrences, metastasis, or death.

CONCLUSION

Conjunctival melanoma in XP is rare and manifests at a younger age.

摘要

目的

研究伴有结膜黑色素瘤的着色性干皮病(XP)患者的人口统计学特征、治疗方法、组织病理学和结局。

方法

回顾性病例系列研究。

结果

中位发病年龄为 18 岁(范围 9-30 岁)。有 3 名女性和 1 名男性患者,中位症状持续时间为 3 个月(范围 1-60 个月)。4 名患者的肿瘤均位于球结膜。所有患者的肿瘤均累及角膜。肿瘤基底直径的中位数为 7 毫米(范围 4-15 毫米)。3 名患者行广泛肿瘤切除活检联合酒精性角膜上皮切除术、游离缘冷冻治疗和羊膜移植。1 名患者因广泛肿瘤行眼眶内容剜除术。1 名患者还接受了辅助的瘤床近距离放疗以治疗显微镜下残留的肿瘤。在中位随访 22 个月(范围 2-101 个月)期间,没有复发、转移或死亡。

结论

XP 伴发的结膜黑色素瘤罕见,发病年龄更早。

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