Milman Tatyana, Shields Carol L, Brooks John S J, Lally Sara E, Shields Jerry A, Tuluc Madalina, Eagle Ralph C
Department of Ophthalmic Pathology, Thomas Jefferson University, Philadelphia, PA, USA.
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.
Ocul Oncol Pathol. 2018 Jun;4(4):230-235. doi: 10.1159/000485427. Epub 2018 Jan 12.
Primary cutaneous angiosarcoma is a rare vasoformative malignant neoplasm that can present a diagnostic and therapeutic challenge. We describe a 76-year-old Caucasian man with right upper eyelid swelling and nodularity, initially suspected clinically to represent either ocular adnexal lymphoma or basal cell carcinoma. Incisional biopsy and wide resection of the mass with frozen section control of margins were interpreted as compatible with hobnail (Dabska-retiform) hemangioendothelioma. Foci of atypia were noted in the tumor, raising speculation of evolution into a more aggressive neoplasm, such as conventional angiosarcoma. The patient subsequently underwent two additional wide resections with frozen section control of margins in an attempt to obtain complete excision of residual tumor, which demonstrated histopathologic features favoring angiosarcoma. The histologic material from the original and subsequent resections was sent in consultation to several soft tissue pathology experts and the final diagnosis of low-grade cutaneous angiosarcoma was established. Despite repeated surgical interventions, there was continued persistence of the tumor in the deep orbital tissues. Various management options, including adjuvant radiotherapy/chemotherapy with and without orbital exenteration, were discussed. The patient decided against further surgical intervention and is currently undergoing adjuvant radiotherapy/chemotherapy. This case illustrates the diagnostic and management difficulties of ocular adnexal angiosarcoma.
原发性皮肤血管肉瘤是一种罕见的血管形成性恶性肿瘤，在诊断和治疗方面具有挑战性。我们描述了一名76岁的白种男性，其右上眼睑肿胀并伴有结节，临床初步怀疑为眼附属器淋巴瘤或基底细胞癌。对肿物进行切开活检并广泛切除，术中冰冻切片检查切缘，结果提示符合鞋钉样（达布斯卡-网状）血管内皮瘤。肿瘤中可见非典型性病灶，这引发了其可能演变为更具侵袭性肿瘤（如传统血管肉瘤）的推测。随后，患者又接受了两次广泛切除手术，术中均进行冰冻切片检查切缘，试图彻底切除残留肿瘤，但组织病理学特征均支持血管肉瘤的诊断。将最初及后续切除的组织标本送请多位软组织病理学专家会诊，最终确诊为低级别皮肤血管肉瘤。尽管多次进行手术干预，肿瘤仍持续存在于眼眶深部组织中。讨论了包括辅助放疗/化疗以及是否行眼眶内容剜除术在内的多种治疗方案。患者决定不再接受进一步手术干预，目前正在接受辅助放疗/化疗。该病例说明了眼附属器血管肉瘤在诊断和治疗上的困难。
