Buchfelder M, Fahlbusch R, Thierauf P, Müller O A
Department of Neurosurgery, University of Erlangen-Nürnberg, West Germany.
Neurosurgery. 1990 Dec;27(6):961-8. doi: 10.1097/00006123-199012000-00016.
Nelson's syndrome is generally regarded as an unusual sequela of primary bilateral adrenalectomy when performed for Cushing's disease. It is classically defined by cutaneous hyperpigmentation, considerably elevated adrenocorticotropic hormone (ACTH) levels, and an enlarged sella turcica. In this report, we present three cases initially treated by transsphenoidal sellar exploration for Cushing's disease. In two of these cases, remission of hypercortisolism did not occur after the initial pituitary exploration. A microadenomectomy was performed in one case and, in the other, no microadenoma was found. In both, Nelson's syndrome occurred after adrenalectomy. A second transsphenoidal operation and radiotherapy were required to control tumor growth. In another case, transsphenoidal adenomectomy of an ACTH-secreting tumor initially led to a remission of hypercortisolism for 4 years, but recurrent Cushing's disease necessitated adrenalectomy, and again Nelson's syndrome occurred. The documentation of a pre-existing ACTH-secreting basophilic pituitary microadenoma before adrenalectomy, as seen in two of our cases, has not been previously reported, and these observations of "non-classical" courses have major implications for the pathophysiology of Nelson's syndrome.
尼尔森综合征通常被认为是因库欣病而进行双侧肾上腺切除术的一种不寻常后遗症。其典型表现为皮肤色素沉着、促肾上腺皮质激素（ACTH）水平显著升高以及蝶鞍增大。在本报告中，我们呈现了3例最初因库欣病接受经蝶窦垂体探查术治疗的病例。其中2例在初次垂体探查后高皮质醇血症未缓解。1例进行了微腺瘤切除术，另1例未发现微腺瘤。这2例在肾上腺切除术后均发生了尼尔森综合征。需要再次进行经蝶窦手术及放疗以控制肿瘤生长。在另一例中，分泌ACTH肿瘤的经蝶窦腺瘤切除术最初使高皮质醇血症缓解了4年，但复发性库欣病需要进行肾上腺切除术，尼尔森综合征再次发生。如我们其中2例所见，肾上腺切除术前存在分泌ACTH的嗜碱性垂体微腺瘤的记录此前未见报道，这些“非典型”病程的观察结果对尼尔森综合征的病理生理学具有重要意义。
