Matyja Ewa, Maksymowicz Maria, Grajkowska Wiesława, Zieliński Grzegorz, Kunicki Jacek, Bonicki Wiesław, Witek Przemysław, Naganska Ewa
Prof. Ewa Matyja, Mossakowski Medical Research Centre, Polish Academy of Sciences, 5 Pawinskiego St., 02-106 Warsaw, Poland, e-mail:
Folia Neuropathol. 2015;53(3):203-18. doi: 10.5114/fn.2015.54421.
Ganglion cell tumours in the sellar region are uncommon. They are usually associated with pituitary adenomas, while isolated ganglion cell neoplasms are extremely rare. We report the clinicopathological studies of five cases diagnosed as ganglion cell tumours located in the intrasellar region: four mixed/collision tumours composed of gangliocytoma and pituitary adenoma, and one isolated ganglioglioma unrelated to adenoma. Clinically, two patients presented with acromegaly, while three others were initially diagnosed as non-functioning adenomas. In four cases, the histopathological examination of surgical specimens revealed intermixed lesions composed of pituitary adenoma and ganglion cell elements. The adenomas appeared to secrete growth hormone. Electron microscopy enabled identification of the sparsely granulated somatotroph cells. Neoplastic neuronal lesions were composed of mature ganglion cells, including binucleate or multinucleate cells. In all cases, boundaries between adenomatous and gangliocytic components were not clearly demarcated, and numerous gangliocytic cells were closely intermingled with adenomatous tissue. One case lacked endocrine symptoms, and no pituitary adenoma was identified in the surgically excised material; it was finally diagnosed as low-grade ganglioglioma. The etiopathogenesis of ganglion cell neoplasms in the sellar region is not clearly defined. Our study revealed that if ganglion cell neoplasms were combined with adenoma, both neoplastic components were closely related to each other, and numerous neuronal elements were strictly intermingled with adenoma cells. Such a tissue pattern indicates that these neoplastic changes, including their common respective etiopathogeneses, are closely related. The identification of both components in sellar regions may have some nosological implications.
鞍区神经节细胞瘤并不常见。它们通常与垂体腺瘤相关,而孤立性神经节细胞瘤极为罕见。我们报告了5例诊断为位于鞍内区域的神经节细胞瘤的临床病理研究:4例为神经节细胞瘤与垂体腺瘤组成的混合/碰撞性肿瘤,1例为与腺瘤无关的孤立性神经节胶质瘤。临床上,2例患者表现为肢端肥大症,另外3例最初诊断为无功能腺瘤。4例手术标本的组织病理学检查显示为垂体腺瘤和神经节细胞成分混合的病变。腺瘤似乎分泌生长激素。电子显微镜检查能够识别稀疏颗粒状的生长激素细胞。肿瘤性神经元病变由成熟的神经节细胞组成,包括双核或多核细胞。所有病例中,腺瘤性和神经节细胞性成分之间的界限均不清晰,大量神经节细胞与腺瘤组织紧密混合。1例患者无内分泌症状,手术切除的组织中未发现垂体腺瘤;最终诊断为低级别神经节胶质瘤。鞍区神经节细胞瘤的病因发病机制尚不明确。我们的研究表明,如果神经节细胞瘤与腺瘤合并存在,两种肿瘤成分相互密切关联,大量神经元成分与腺癌细胞紧密混合。这种组织模式表明这些肿瘤性改变,包括它们各自常见的病因发病机制,密切相关。在鞍区识别这两种成分可能具有一些分类学意义。