Unit of Ophthalmology, Department of Medicine, Surgery and Neuroscience, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Ocular Oncology Service, Helsinki University Central Hospital, Helsinki, Finland.
Eur J Ophthalmol. 2022 Jul;32(4):NP88-NP93. doi: 10.1177/11206721211001266. Epub 2021 Mar 11.
Orbital recurrence of malignant choroidal melanoma is quite uncommon, occurring in about 3% of patients undergoing enucleation for large tumors. Orbital recurrences after more than 10 years from enucleation are even rarer. In literature, only few reports described orbital recurrence that occurred between 10 and 40 years after primary diagnosis. Herein we report a very late choroidal melanoma recurrence, 40 years post enucleation, of a 52 year-old female who had undergone left enucleation at the age of 12. She presented to our clinic for consultation in 2017, complaining of difficulty to contain the prosthesis, as well as, presence of small pigmented palpable nodules inferiorly in her anophthalmic socket. The patient was not aware of the medical condition which had lead to the enucleation. We requested her medical reports and detailed history through her family. We realized after reviewing her charts 40 years back, that her enucleation was due to malignant choroidal melanoma (CMM). Full screening was performed. After magnetic resonance imaging (MRI), that showed the presence of nodular masses in the anophthalmic socket, an excisional biopsy was performed. Histopathology confirmed the diagnosis of CMM (epithelioid and spindle cell type), supporting the hypothesis that residual melanoma cells may remain clinically dormant for long periods, even for decades. A literature review was performed in order to review similar cases and to understand and discuss multiple factors, which may explain this extremely delayed recurrence. To the best of our knowledge, this is the third case to be reported in the literature.
眼窝内恶性脉络膜黑色素瘤的复发非常罕见，约占行眼球摘除术治疗大型肿瘤患者的 3%。眼球摘除 10 年以上后发生眼窝复发的情况更为罕见。在文献中，只有少数报道描述了原发性诊断后 10 至 40 年间发生的眼窝复发。在此，我们报告了一例非常迟发性脉络膜黑色素瘤复发病例，患者为 52 岁女性，于 12 岁时行左眼眼球摘除术，40 年后（即 2017 年）就诊于我院。她主诉难以控制义眼，且在义眼窝下方可触及小结节。患者对导致眼球摘除的疾病并不知情。我们通过其家属查阅了她的病历和详细病史。通过查阅她 40 年前的病历，我们发现她的眼球摘除是由于恶性脉络膜黑色素瘤（CMM）。我们对其进行了全面筛查。磁共振成像（MRI）显示义眼窝内存在结节状肿块后，我们对其进行了切除活检。组织病理学检查证实了 CMM（上皮样和梭形细胞型）的诊断，这支持了这样一种假说，即残留的黑色素瘤细胞可能在很长一段时间内（甚至数十年）保持临床休眠状态。我们还进行了文献复习，以回顾类似病例，并了解和讨论多个可能解释这种极其延迟复发的因素。据我们所知，这是文献中报道的第三例病例。
