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[太田痣。1例合并细胞性蓝痣且怀疑有恶性变的病例报告及文献复习]

[Nevus of Ota. Presentation of a case associated with a cellular blue nevus with suspected malignant degeneration and review of the literature].

作者信息

Bisceglia M, Carosi I, Fania M, Di Ciommo A, Lomuto M

机构信息

Servizio di Anatomia patologica, IRCCS-Ospedale Casa Sollievo della Sofferenza, Foggia.

出版信息

Pathologica. 1997 Apr;89(2):168-74.

PMID:9411364
Abstract

BACKGROUND

Nevus of Ota is a melanotic pigmentary disorder ("dermal melanocytosis"), mostly congenital or acquired, involving the skin innervated by the first and the second branch of the trigeminal nerve, hence its descriptive label of "nevus fuscus coerulaeus ophthalmic and maxillaris". In more than half of patients this condition is associated with "ocular melanocytosis" ("melanosis oculi") involving the conjunctiva, the sclera, the uveal tract and possibly the optic nerve. In some cases a condition of "orbital melanocytosis" with involvement of orbital fat and periosteum by dendritic melanocytes is on record as well as in some other patients an analogous condition of "leptomeningeal melanocytosis" is present. At histology tissues from the above sites are seen infiltrated by dendritic melanocytes which can vary in number from so scarce up to so numerous that a diagnosis of a blue nevus of the common type is warranted. Sometimes the finding of a variously pigmented typical cellular blue nevus in the skin and alternatively that of heavily pigmented melanocytoma in the eye ("nevus magnocellularis") or in the meninges (so-called "melanotic meningioma") are respectively observed.

MATERIALS AND METHODS

A case of cellular blue nevus with histologically uncertain malignant potential in a nevus of Ota of 30 years duration in a white female patient aged 59 is described. The lesion which was surgically totally removed grossly appeared nodular shaped and 2 cm sized. Histologically it consisted of a fairly well-circumscribed proliferation of melanocytic spindle-shaped cells growing in a vaguely fascicular pattern. On the basis of random nuclear atypicalities and pleomorphism and additionally by virtue of the presence of a few scattered mitoses (one of which was atypical) but in absence of frank necrosis a diagnosis of unpredictable biologic behaviour seemed to be warranted. The patient was closely followed-up but no adjunctive therapy given. Four years after the excision and diagnosis no local recurrence or distant metastasis has been discovered. A computerized search of previously recorded cases of melanomas in nevus of Ota was made.

CONCLUSIONS

Forty-eight cases of malignant melanomas complicating this clinico-pathological setting are on record, mostly in the uveal tract, followed by locations in central nervous system, skin, and retro-orbital fatty tissue. Melanomas arising in nevus of Ota tend to be low grade lesions that do infiltrate locally but rarely metastasize. The importance of a closely dermatological and ophthalmic surveillance of patients with nevus of Ota is emphasized.

摘要

背景

太田痣是一种黑素沉着性色素沉着障碍(“真皮黑素细胞增多症”),大多为先天性或后天性,累及三叉神经第一和第二分支支配的皮肤,因此其描述性标签为“眼上颌蓝褐色痣”。超过半数的患者这种情况与“眼部黑素细胞增多症”(“眼黑素沉着症”)相关,累及结膜、巩膜、葡萄膜,可能还包括视神经。在某些病例中,有记录显示存在“眼眶黑素细胞增多症”,树突状黑素细胞累及眼眶脂肪和骨膜,在其他一些患者中还存在类似的“软脑膜黑素细胞增多症”。组织学上,上述部位的组织可见树突状黑素细胞浸润,其数量可从极少到极多不等,以至于有理由诊断为普通型蓝痣。有时在皮肤中可发现色素沉着各异的典型细胞性蓝痣,在眼部(“大细胞痣”)或脑膜(所谓“黑素性脑膜瘤”)中可发现色素沉着严重的黑素细胞瘤。

材料与方法

描述了一名59岁白人女性患者,其太田痣病程30年,患有组织学上恶性潜能不确定的细胞性蓝痣。手术完全切除的病变大体呈结节状,大小为2厘米。组织学上,它由黑素细胞梭形细胞的相当界限清楚的增生组成,呈模糊的束状生长模式。基于随机的核异型性和多形性,另外由于存在一些散在的有丝分裂(其中一个是非典型的),但无明显坏死,似乎有理由诊断为生物学行为不可预测。对该患者进行了密切随访,但未给予辅助治疗。切除及诊断四年后,未发现局部复发或远处转移。对先前记录的太田痣中黑色素瘤病例进行了计算机检索。

结论

有记录的48例恶性黑色素瘤并发于这种临床病理情况,大多发生在葡萄膜,其次是中枢神经系统、皮肤和眶后脂肪组织。太田痣中发生的黑色素瘤往往是低级别病变,倾向于局部浸润但很少转移。强调对太田痣患者进行密切的皮肤科和眼科监测的重要性。

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Melanocytoma of the optic disk: A review.视盘黑素细胞瘤:综述。
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An Orbital Malignant Melanoma Arising in Cellular Blue Nevus in a Patient with Nevus of Ota.一名患有太田痣的患者,其细胞性蓝痣发生眶部恶性黑色素瘤。
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Intermediate-grade meningeal melanocytoma associated with nevus of Ota: a case report and review of the literature.与太田痣相关的中级脑膜黑素细胞瘤:一例报告并文献复习
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Activating mutations of the GNAQ gene: a frequent event in primary melanocytic neoplasms of the central nervous system.GNAQ 基因的激活突变:中枢神经系统原发性黑色素细胞肿瘤的常见事件。
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