Klinika Endokrynologii, Chorób Metabolicznych i Chorób Wewnętrznych, Pomorski Uniwersytet Medyczny, ul. Unii Lubelskiej 1, 71-252 Szczecin, Poland.
Endokrynol Pol. 2018;69(2):212-228. doi: 10.5603/EP.2018.0023.
The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions. The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe-sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke's cleft cyst, which became possible with the use of ¹¹C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke's cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess. The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig-ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.
鞍区及鞍上囊性病变的鉴别在临床实践中是一个重大问题,因为它们在临床、影像学甚至组织病理学表现上都有相似之处。做出正确的诊断对于采取适当的治疗决策至关重要。位于鞍区及鞍上病变最常见的临床表现是头痛。它常与垂体柄受压引起的垂体前叶功能减退或高泌乳素血症的症状同时存在。尿崩症、肥胖、精神障碍和昼夜节律紊乱可能与穿透鞍上间隙的病变有关。排除可能存在的垂体微腺瘤和 Rathke 裂囊肿非常重要,¹¹C-蛋氨酸正电子发射断层扫描/计算机断层扫描(C-MET PET/CT)的应用使这一点成为可能。文献报道表明,垂体微腺瘤可能与 Rathke 裂囊肿共存于 10%的患者中。鞍区及鞍上囊性病变还应与囊性垂体腺瘤或脓肿相鉴别。有症状的鞍区及鞍上囊性病变的首选治疗方法是神经外科手术,它通常可以缓解头痛和改善视力障碍,而较少恢复正常的垂体功能。在鞍上病变中,神经外科手术可能会引发或加重下丘脑已有损伤的症状。由于这些病变的高复发率、潜在的恶性转化以及通过化生可能发展为腺瘤,接受神经外科手术治疗的位于鞍区及鞍上囊性病变的患者需要监测几年。BRAF/MEK 通路的靶向治疗的出现为颅咽管瘤患者带来了新的治疗机会。