Sakata Kiyohiko, Miyoshi Junko, Takeshige Nobuyuki, Komaki Satoru, Miyagi Naohisa, Nakashima Shinji, Morioka Motohiro, Sugita Yasuo
Department of Neurosurgery, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Fukuoka, Japan,
Pituitary. 2015 Oct;18(5):685-94. doi: 10.1007/s11102-015-0635-8.
Primary intracranial melanocytomas are rare neoplasms, especially in the sellar region. Intracranial melanocytoma is usually a dural-based tumor, fed by dural arterial branches in a manner similar to meningioma. Primary sellar melanocytoma may be misdiagnosed as hemorrhagic pituitary macroadenoma, spindle cell oncocytoma, and intrasellar meningioma. These tumors differ in some radiological respects, but are difficult to differentiate preoperatively.
Only five cases of primary sellar/suprasellar melanocytic tumors, excluding melanomas have been reported thus far. In this paper, we report an instructive new case of a 31-year-old woman presenting with a 2-year history of amenorrhea and an intrasellar mass with suprasellar extension, suggestive of hemorrhagic pituitary adenoma.
Transsphenoidal surgical excision was difficult due to extensive bleeding from the lesion, and at the time, the tumor could not be diagnosed histopathologically. Six years later, we operated again because of tumor regrowth. Angiography revealed a hypervascular tumor, which was fed from the dorsal sellar floor. We had difficulty resecting the tumor, but achieved total removal. Our case had typical radiographic characteristics of melanocytoma, revealed by both magnetic resonance imaging and angiography. However, it was difficult to reach a final diagnosis. Further histopathological examination, including immunohistochemical and ultrastructural studies, was helpful for diagnosis of melanocytoma.
Primary sellar melanocytic tumors are derived from melanocytes in the meningeal lining of the sellar floor or in the diaphragm sellae, based on both embryological assumptions and the clinical findings of our case. We discuss the problems of differential diagnosis and management of primary sellar melanocytic tumors.
原发性颅内黑色素细胞瘤是罕见肿瘤,尤其在鞍区。颅内黑色素细胞瘤通常是硬膜起源肿瘤,由硬膜动脉分支供血,方式类似于脑膜瘤。原发性鞍区黑色素细胞瘤可能被误诊为出血性垂体大腺瘤、梭形细胞嗜酸细胞瘤和鞍内脑膜瘤。这些肿瘤在某些放射学方面存在差异,但术前难以鉴别。
迄今为止,仅报道了5例原发性鞍区/鞍上黑色素细胞肿瘤(不包括黑色素瘤)。本文报告1例有指导意义的新病例,患者为31岁女性,有2年闭经病史,鞍内有肿块并向鞍上延伸,提示出血性垂体腺瘤。
经蝶窦手术切除因病变广泛出血而困难,当时肿瘤无法进行组织病理学诊断。6年后,因肿瘤复发再次手术。血管造影显示为富血管肿瘤,由鞍背底部供血。肿瘤切除困难,但实现了全切。我们的病例具有黑色素细胞瘤典型的影像学特征,磁共振成像和血管造影均有显示。然而,很难做出最终诊断。进一步的组织病理学检查,包括免疫组织化学和超微结构研究,有助于黑色素细胞瘤的诊断。
基于胚胎学假设和我们病例的临床发现,原发性鞍区黑色素细胞肿瘤起源于鞍底脑膜内衬或鞍膈的黑色素细胞。我们讨论了原发性鞍区黑色素细胞肿瘤的鉴别诊断和处理问题。