Azoury Saïd C, Crompton Joseph G, Straughan David M, Klemen Nicholas D, Reardon Emily S, Beresnev Tatiana H, Hughes Marybeth S
Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA; Department of Surgery, The Johns Hopkins Hospital, Johns Hopkins University, School of Medicine, USA.
Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Int J Surg Case Rep. 2015;10:232-5. doi: 10.1016/j.ijscr.2015.03.053. Epub 2015 Apr 1.
Primary nasopharyngeal melanoma is an exceedingly rare pathology with unclear etiology and oftentimes obscure clinical presentation. Despite improved diagnostic capabilities, these lesions are often diagnosed at an advanced stage and associated prognosis is poor, partly due to high rates of recurrences and metastasis.
A 74-year-old woman was diagnosed with metastatic melanoma to the liver, of unknown primary. Just prior to the time of diagnosis, she experienced several episodes of severe epistaxis which she managed conservatively. Her symptoms eventually subsided without further medical evaluation. The patient was initially treated with interleukin-2 (IL-2) for her advanced disease, but her cancer progressed. She was then enrolled in a protocol for percutaneous hepatic perfusion (PHP) with melphalan and had complete radiographic resolution of disease, yet her nosebleeds recurred and persisted despite conservative measures. Six years after her initial diagnosis, a nasopharyngoscopy demonstrated a pigmented lesion in the posterior nasopharynx. Surgical resection was performed (pathology consistent with mucosal melanoma) followed by radiation therapy. She has since had complete resolution of bleeding and shows no evidence of cancer.
To our knowledge, this is the first report of a diagnosis of primary nasopharyngeal melanoma 6-years following complete remission of metastatic disease. Surgery remains the primary treatment for disease and symptom control in this setting.
Timely diagnosis of nasopharyngeal melanomas remains challenging. Thorough clinical evaluations should be performed in such patients, and attention should be paid to recurrent and persistent symptoms, such as epistaxis and hearing loss. This may allow for earlier detection of primary disease.
原发性鼻咽黑色素瘤是一种极其罕见的病理类型，病因不明，临床表现常常隐匿。尽管诊断能力有所提高，但这些病变往往在晚期才被诊断出来，且相关预后较差，部分原因是复发和转移率较高。
一名74岁女性被诊断为肝脏转移性黑色素瘤，原发部位不明。在诊断前，她经历了几次严重鼻出血，采取了保守治疗。她的症状最终自行缓解，未作进一步医学评估。该患者最初因晚期疾病接受白细胞介素-2（IL-2）治疗，但癌症仍进展。随后她参加了一项使用美法仑进行经皮肝灌注（PHP）的方案，影像学上疾病完全消退，但尽管采取了保守措施，鼻出血仍复发且持续存在。初次诊断六年后，鼻咽镜检查显示鼻咽后部有一个色素沉着病变。进行了手术切除（病理结果与黏膜黑色素瘤一致），随后进行了放射治疗。此后她的出血症状完全缓解，且无癌症迹象。
据我们所知，这是第一例转移性疾病完全缓解6年后诊断为原发性鼻咽黑色素瘤的报告。在这种情况下，手术仍然是疾病治疗和症状控制的主要方法。
鼻咽黑色素瘤的及时诊断仍然具有挑战性。对此类患者应进行全面的临床评估，并关注鼻出血和听力丧失等复发和持续症状。这可能有助于更早发现原发性疾病。
