Li Xiaoman, Liu Yang, Miao Yuan, Wang Jinping, Wang Liang, Wang En-Hua
Key Laboratory of Medical Cell Biology, Ministry of Education.
Department of Pathology, College of Basic Medical Sciences and First Affiliated Hospital, China Medical University, Shenyang, China.
Medicine (Baltimore). 2019 Nov;98(44):e17772. doi: 10.1097/MD.0000000000017772.
Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and decreased libido. Unlike that of common pituitary adenoma, the association between pituicytoma and Cushing disease (CD) is extremely rare. There were only 6 reported cases till now. In the current study, we describe an unusual case of pituicytoma associated with severe CD with a recurrence-free follow-up period of 49 months.
A 32-year-old woman was referred to our hospital with moon face, central obesity, and purple stripes on the lower limbs.
The plasma cortisol level was 1122 nmol/L. The low-dose dexamethasone suppression test failed to suppress plasma cortisol. This test provided evidence of nonpituitary-dependent CD. However, magnetic resonance imaging demonstrated a sellar mass measuring approximately 7.6 × 5.7 mm. The patient was diagnosed with pituitary microadenoma. Histopathological analysis of the tissue sections based on the findings from the immunohistochemical staining diagnosed it as pituicytoma.
Transsphenoidal surgery was performed to remove the pituitary mass.
Within 2 months postoperatively, the patient's blood pressure and cortisol level decreased gradually and normalized on the 6th month when other symptoms of CD also disappeared. The patient is presently free from recurrence 49 months after the initial diagnosis.
Based on the postoperative remission, CD was caused by pituitary disorders. A reasonable assumption is that an extremely small coexisting adenoma was not detected by the surgeon and washed out during the dissection. Another possible explanation might be the mass effect caused by this intrasellar lesion.
垂体细胞瘤是鞍区和鞍上区极为罕见的肿瘤，临床表现包括头痛、视力障碍、垂体功能减退和性欲减退。与常见垂体腺瘤不同，垂体细胞瘤与库欣病（CD）的关联极为罕见。截至目前，仅有6例相关报道。在本研究中，我们描述了一例罕见的垂体细胞瘤合并严重CD的病例，随访49个月无复发。
一名32岁女性因满月脸、中心性肥胖和下肢紫纹转诊至我院。
血浆皮质醇水平为1122 nmol/L。小剂量地塞米松抑制试验未能抑制血浆皮质醇。该试验提供了非垂体依赖性CD的证据。然而，磁共振成像显示鞍区有一大小约为7.6×5.7 mm的肿块。患者被诊断为垂体微腺瘤。根据免疫组化染色结果对组织切片进行组织病理学分析，诊断为垂体细胞瘤。
行经蝶窦手术切除垂体肿块。
术后2个月内，患者血压和皮质醇水平逐渐下降，术后6个月恢复正常，同时CD的其他症状也消失。自初次诊断以来，患者目前已49个月无复发。
基于术后缓解情况，CD是由垂体疾病引起的。一个合理的假设是，外科医生未检测到极小的共存腺瘤，且在手术切除过程中被冲洗掉了。另一种可能的解释可能是该鞍内病变引起的占位效应。
