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Primary sellar melanocytoma: pathological, clinical and treatment review.
Journal of Endocrinological Investigation ( IF 5.4 ) Pub Date : 2019-12-03 , DOI: 10.1007/s40618-019-01158-8
L Albano 1 , M Losa 1 , L R Barzaghi 1 , G Spatola 2 , P Panni 1 , M R Terreni 3 , P Mortini 1
Affiliation  

BACKGROUND Sellar melanocytomas represent a small subgroup of primary melanocytic tumors. They arise from melanocytes located in the meningeal lining of the sellar floor or in the diaphragma sellae and this location is very uncommon. Usually, sellar melanocytomas are benign and slow-growing tumors with a high likelihood of recurrence. PURPOSE To our knowledge, due to the rarity of this condition, there are no guidelines regarding their diagnosis and treatment in the medical literature to date. We have developed a narrative review, analyzing the available studies regarding primary sellar melanocytomas reported in the medical literature. We have found ten papers on this topic and all of them are case reports. In all patients, tumor diagnosis was performed after the occurrence of neurological symptoms, in particular progressive visual loss or endocrinological disorders. The diagnosis is difficult, and it requires several preoperative and postoperative investigations, but histological examination is crucial. CONCLUSIONS Transsphenoidal surgery is the first-choice treatment. In case of tumor's recurrence or regrowth, the role of radiation therapy and chemotherapy is not entirely clear.

中文翻译:

原发性鞍黑素细胞瘤:病理,临床和治疗审查。

背景蝶鞍黑素细胞瘤代表原发性黑素细胞瘤的一小部分。它们起源于位于鞍底的脑膜内膜或蝶膜中的黑素细胞,这种位置非常罕见。通常,鞍状黑素细胞瘤是良性且生长缓慢的肿瘤,极有可能复发。目的据我们所知,由于这种疾病的罕见性,迄今为止在医学文献中还没有关于其诊断和治疗的指南。我们已经进行了叙述性评论,分析了医学文献中报道的有关原发性蝶鞍黑素细胞瘤的可用研究。我们已经找到了10篇关于该主题的论文,所有都是案例报告。在所有患者中,肿瘤诊断都是在出现神经系统症状后进行的,特别是进行性视力减退或内分泌失调。诊断很困难,需要进行术前和术后的几次检查,但组织学检查至关重要。结论经蝶窦手术是首选治疗方法。在肿瘤复发或再生长的情况下,放射疗法和化学疗法的作用尚不完全清楚。
更新日期:2020-04-21
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