Haap M, Gallwitz B, Meyermann R, Mittelbronn M
Department of Endocrinology, Metabolism, Nephrology and Clinical Chemistry, University of Tübingen, Germany.
Exp Clin Endocrinol Diabetes. 2009 Jun;117(6):289-93. doi: 10.1055/s-0028-1085997. Epub 2008 Oct 1.
Herein, we present the case of a 63-year old female patient with initial symptoms of myopathy, hypokaliemia, glucosuria and psychotic symptoms. Laboratory analysis demonstrated elevated plasma levels of ACTH and cortisol. Additionally, urine cortisol excretion was increased approximately 60-fold. MRI imaging revealed a possible pituitary microadenoma. To confirm the diagnosis a bilateral inferior petrosal sinus sampling was performed presenting higher ACTH levels on the right side. However, after surgery cortisol levels did not return to normal range. Histological examination of the tumor revealed a microadenoma. Six days postoperatively, the patient developed several pneumonic infiltrations and fever therefore antibiotic and antifungal therapy was started immediately. In addition aspergillus antigen was elevated. During this septic condition, cortisol levels further increased. The patient died despite optimal intensive care under septical conditions 8 days after surgery. Microbiological analysis identified Aspergillus fumigatus in broncho-alveolar lavage and several organ systems including the heart and brain. Neuropathological autopsy revealed nodular proliferations of corticotropic cells in the pituitary gland that are assumed to be morphological entities between diffuse hyperplasias and adenomas, termed as tumorlets. In single reports, multiple pituitary lesions in patients with Cushing's disease have been demonstrated, but to our knowledge none of these cases presented the combination of an ACTH-producing microadenoma and corticotroph cell hyperplasia in the same patient. Therefore, even after resection of a pituitary microadenoma one should be aware of the possibility of continuously elevated ACTH level being due to multifocal nodular corticotroph hyperplasia which is invisible by neuroradiological examination.
在此，我们报告一例63岁女性患者，其最初症状为肌病、低钾血症、糖尿和精神症状。实验室分析显示血浆促肾上腺皮质激素（ACTH）和皮质醇水平升高。此外，尿皮质醇排泄量增加了约60倍。磁共振成像（MRI）显示可能存在垂体微腺瘤。为明确诊断，进行了双侧岩下窦采血，结果显示右侧ACTH水平较高。然而，手术后皮质醇水平并未恢复到正常范围。肿瘤组织学检查显示为微腺瘤。术后6天，患者出现多处肺部浸润和发热，因此立即开始使用抗生素和抗真菌治疗。此外，曲霉菌抗原升高。在这种脓毒症状态下，皮质醇水平进一步升高。尽管在脓毒症状态下给予了最佳的重症监护，但患者在术后8天仍死亡。微生物学分析在支气管肺泡灌洗以及包括心脏和大脑在内的多个器官系统中发现了烟曲霉。神经病理学尸检显示垂体中促肾上腺皮质激素细胞呈结节状增生，这些增生被认为是弥漫性增生和腺瘤之间的形态学实体，称为微腺瘤样结节。在个别报告中，已证实库欣病患者存在多个垂体病变，但据我们所知，这些病例中没有一例在同一患者中同时出现产生ACTH的微腺瘤和促肾上腺皮质激素细胞增生。因此，即使切除垂体微腺瘤后，也应意识到ACTH水平持续升高可能是由于多灶性结节性促肾上腺皮质激素细胞增生所致，而这种增生在神经放射学检查中是看不见的。
