Todeschi J, Chibbaro S, Clavier J-B, Lhermitte B, Goichot B, Proust F
Service de neurochirurgie, hôpital Hautepierre, hôpitaux universitaires de Strasbourg, 1, avenue Molière, 67098 Strasbourg, France.
Service de radiothérapie, centre de lutte contre le cancer Paul-Strauss, 67065 Strasbourg, France.
Neurochirurgie. 2016 Dec;62(6):339-343. doi: 10.1016/j.neuchi.2016.08.002. Epub 2016 Oct 26.
Sellar and suprasellar primary melanocytic tumors are exceptional occurrences. Besides the difficulty of differential diagnosis between a primary and secondary lesion, treatment of these pathologies is still unclear and controversial.
We describe the case of a 36-year-old woman with no relevant previous medical history who presented with 1 month history of diabetes insipidus, blurred vision and generalized weakness; a brain MRI disclosed an atypical pituitary stalk lesion; initially the tumor was biopsied through an endonasal endoscopic approach that revealed a melanocytic tumor; the patient was afterwards managed by a second stage extended endonasal endoscopic approach achieving a subtotal tumor removal. The overall survival was of 14 months due to the multidisciplinary management including surgery, radio and chemotherapy.
If a biopsy is essential to deal with these invasive lesions, treatment including surgical resection should be part of a multidisciplinary approach.
鞍区和鞍上原发性黑素细胞瘤极为罕见。除了原发性和继发性病变鉴别诊断困难外，这些病变的治疗仍不明确且存在争议。
我们描述了一名36岁女性患者的病例，她既往无相关病史，出现尿崩症、视力模糊和全身无力1个月；脑部磁共振成像（MRI）显示垂体柄有非典型病变；最初通过鼻内镜入路对肿瘤进行活检，结果显示为黑素细胞瘤；随后患者接受了二期扩大鼻内镜入路手术，实现了肿瘤大部分切除。由于采用了包括手术、放疗和化疗在内的多学科管理，患者总生存期为14个月。
如果活检对于处理这些侵袭性病变至关重要，那么包括手术切除在内的治疗应成为多学科方法的一部分。
