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一名着色性干皮病患者的结膜平滑肌肉瘤:5年随访无复发

Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence.

作者信息

Akbaş Zeynep Şerikoğlu, Oto Bilge Batu, Gönen Busenur, Ülgen Övgü Aydın, Sarıcı Ahmet Murat

机构信息

Department of Ophthalmology, Cerrahpasa Medical Faculty, Istanbul University- Cerrahpaşa, Istanbul, Turkey.

Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University- Cerrahpaşa, Istanbul, Turkey.

出版信息

Arq Bras Oftalmol. 2022 Jul 18;87(1):0301. doi: 10.5935/0004-2749.2021-0301. eCollection 2022.

Abstract

Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

摘要

结膜平滑肌肉瘤是一种非常罕见的软组织恶性肿瘤。在此,我们描述了一例患有另一种罕见疾病——着色性干皮病的患者发生结膜平滑肌肉瘤的病例。这位27岁的单眼着色性干皮病患者主诉左眼有覆盖眼表的外生性肿物。检查发现一个覆盖左眼整个眼表的血管性、出血性肿物。因此,进行了肿物全切手术。病理诊断符合结膜平滑肌肉瘤。患者未接受额外的化疗、放疗或手术。在5年的随访期间未观察到复发或转移。原发性结膜平滑肌肉瘤和着色性干皮病均为非常罕见的疾病。对于着色性干皮病患者的结膜肿物,应谨慎处理,有必要进行组织病理学检查。对于结膜平滑肌肉瘤,早期诊断、病变局限未扩散以及完整切除可提供最佳预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/090f/11587499/b1ee5653dbec/abo-87-01-e2021-0301-g01.jpg

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