Lymphocytic hypophysitis in the pediatric population

BACKGROUND: Lymphocytic hypophysitis (LYH) is a rare inflammatory disease of the pituitary gland that usually affects women in their ante- or immediate postpartum period; males are affected less frequently than females. An autoimmune pathogenesis is suggested. Symptoms comprise anterior and/or posterior pituitary insufficiency of varying degrees. So far, specific characteristics of this rare disease in childhood are not well described. CASE HISTORY: We report the case of a 12-year-and-11-month-old boy with histologically confirmed LYH clinically presenting with diabetes insipidus. A high-dose steroid therapy was administered, in which the therapeutic effect was uncertain. His 6-year follow-up is presented. DISCUSSION: The literature is reviewed for children presenting with LYH; their characteristics, pituitary involvement, and clinical follow-up are listed and discussed. CONCLUSION: Even though magnetic resonance imaging can be highly suspicious for LYH, only surgical exploration can confirm the diagnosis. The efficacy of medical treatment is still controversial; a close follow-up is necessary to control and correct the endocrinological function, if required.     

Autoantibodies against pituitary proteins in patients with adrenocorticotropin-deficiency

BACKGROUND: An autoimmune cause of adrenocorticotropin (ACTH)-deficiency is presented, as it is known to be a characteristic feature of lymphocytic hypophysitis, a disease of the pituitary gland considered to be autoimmune. MATERIALS AND METHODS: The aim of this study was twofold: (1) to evaluate the occurrence of pituitary autoantibodies and (2) to correlate it to clinical and immunological features in a large group of patients with ACTH-deficiency of possible autoimmune aetiology. Sixty-five patients with ACTH-deficiency and 57 healthy subjects participated in the study. Pituitary autoantibodies were measured by an immunoblotting assay with human pituitary cytosol as antigen. RESULTS: Autoantibodies to a novel 36-kDa pituitary autoantigen were seen in sera from 18.5% (12/65) patients and only 3.5% (2/57) of control subjects (P = 0.0214). When taking only those subjects with strong immunoreactivity into account, the significance was lost; P = 0.3642. Immunoreactivity to a 49-kDa pituitary autoantigen was observed in 21.5% (14/65) of ACTH-deficient patients compared with 8.8% (5/57) of control subjects (P = 0.0910). This 49-kDa pituitary autoantigen has recently been identified as neurone-specific enolase and a candidate marker for neuroendocrine autoimmunity. Clinical parameters in patients with positive versus those with negative pituitary immunoreactivity did not differ. However, autoantibodies to thyroglobulin were positively correlated to immunoreactivity against the 36-kDa pituitary autoantigen (P = 0.014). CONCLUSIONS: Our findings of pituitary autoantibodies in patients' sera support the theory that an autoimmune destruction of corticotrophs may be the underlying cause of hormonal deficit in some patients with ACTH-deficiency.     

淋巴细胞性垂体炎患者治疗前后激素水平变化1例报告及文献复习

目的：探讨淋巴细胞性垂体炎（LYH）的临床表现和影像学特点及治疗原则,阐述明确诊断及激素治疗的重要性。方法：收集1例经手术病理诊断为LYH患者的临床资料,结合国内外相关文献,分析其临床表现、影像学特点及治疗方法。结果：患者,女性,28岁,因间断性头痛2个月就诊。查体未见明显阳性体征,头MRI提示垂体占位性病变,行甲状腺彩超、垂体-甲状腺轴激素、垂体-肾上腺轴激素、垂体-性腺轴激素和戈那瑞林实验等相关检查。结合患者的临床表现、影像学检查和实验室检查结果初步考虑垂体腺瘤的可能性大,行鞍区占位性病变切除术,术后病理提示LYH。患者术后头痛症状明显好转,但仍有腺垂体功能低下,给予激素替代治疗。3个月后,患者性激素水平正常,恢复正常月经,仍有间断头痛,甲状腺功能减低。结论：LYH与垂体腺瘤很难鉴别。病理活检是LYH的诊断金标准。LYH患者手术治疗后仍可能存在腺垂体功能低下,需长期激素替代治疗。     

Rathke囊肿继发性垂体炎病例分析并文献复习

目的 分析Rathke囊肿继发性垂体炎的临床特征,提高临床医师对继发性垂体炎的诊治能力。方法 对2010年1月至2020年5月在榆林市第一医院内分泌科住院的Rathke囊肿破裂所致继发性垂体炎患者临床特点及预后进行回顾性分析,并进行文献复习。结果 ①6例Rathke囊肿继发性垂体炎,男性2例,女性4例,发病年龄(46.33±14.42)岁,病程(2.40±1.98)月;②4例首发症状为头痛,3例表现为烦渴、多尿;5例患者出现低促性腺激素性性腺功能减退症,3例轻度高泌乳素血症,3例肾上腺皮质功能减退,2例中枢性甲状腺功能减退症,1例生长激素缺乏,3例尿崩症。③垂体影像学示病灶最长直径4~23 mm,多表现为稍短T1或等T1、长T2信号,环形不均匀强化,垂体柄增粗和异常强化,视交叉受压或未受压,伴或不伴垂体后叶高信号消失;病理组织见炎性细胞浸润,伴或不伴纤维化;免疫组化见上皮样细胞。结论 Rathke囊肿继发性垂体炎一旦确诊,建议及时手术治疗,以减少全垂体功能减退。     

Granulomatous hypophysitis: presentation and MRI appearance

Granulomatous hypophysitis (GrHy) is a relatively rare inflammatory disease compared with lymphocytic hypophysitis. Only a few cases with magnetic resonance imaging (MRI) findings have been reported to date. We describe the MRI findings for two patients with GrHy with unusual histories and clinical outcomes.     

Lymphocytic hypophysitis with cystic MRI appearance

Summary Lymphocytic hypophysitis (LH) is an infrequent primary inflammatory disorder, which is usually diagnosed after surgery for lesions suspected to be pituitary adenomas. Some radiological features have been described that may allow a preoperative diagnosis, such as a symmetric enlargement of the gland with diffuse contrast enhancement extending to the basal hypothalamus in a tongue-like fashion. We describe the case of a patient with LH presenting with the MR imaging of a cystic lesion with ring contrast enhancement. It seems that this appearance in imaging studies is not unusual, and should be considered among the features suggesting this disease in an appropriate clinical context.     

Inflammatory Hypophysitis – The Spectrum of Disease

Summary  Background. Inflammatory conditions of the pituitary are rare and consequently there are a number of single case reports of this condition but few reports of series. The condition is often divided into lymphocytic and granulomatous hypophysitis and it has been suggested that these two conditions represent the ends of a spectrum of disease.  Method. We present our experience with 14 cases of this condition, correlating the presenting symptoms with the neuroradiology, surgical findings and subsequent histology.  Findings. The subjects (11 female 3 male) ranged in age from 13 to 64 years. Final histopathological diagnoses included 5 cases of lymphocytic hypophysitis, 4 cases of Rathke's pouch cyst with granulomatous response, 2 cases of granulomatous hypophysitis and 1 case with an inflammatory process that did not fit the current classification. Two subjects did not undergo surgery. Headache was a presenting feature in 11 of 14 cases and fever in 3 of 14 cases. Length of symptoms prior to presentation varied from acute onset to 9 years. One case of lymphocytic hypophysitis was associated with pregnancy. Evidence of hypopituitarism was present in 9 of 10 subjects assessed preoperatively. Preoperative radiology showed three patterns of disease: A cystic appearance was common with low signal content on MRI T1 weighting with an enhancing ring and a thickened enhancing stalk (5 patients). 4 patients showed a solid enhancing mass. A third group (2 patients) showed cysts with high signal content on T1 weighting – both of these were Rathke's cysts on histology.  Interpretation. Overall there were no striking features in the clinical presentation to distinguish pituitary inflammation from pituitary adenoma. The prognosis was generally good.     

Diagnosis and Management of Immune Checkpoint Inhibitor–related Toxicities in Ovarian Cancer: A Series of Case Vignettes

Use of immune checkpoint inhibitors targeting cytotoxic T-lymphocyte-associated antigen 4 and programmed cell death protein 1 have led to improved survival outcomes for advanced solid-tumor malignancies. This report helps the reader gain a better understanding of adverse events in patients with ovarian cancer on checkpoint inhibitor therapy. We describe 3 hypothetical case vignettes of patients with gynecologic cancer on checkpoint inhibitor immunotherapy and discuss common immune-related adverse events. The typical presentation and onset of immune-related events are different from those associated with conventional chemotherapy. This report highlights the importance of early recognition and management of these events.     

糖皮质激素联合硫唑嘌呤治疗复发性淋巴细胞性垂体炎三例报道

报道3例复发性淋巴细胞性垂体炎(LYH)患者均为女性,其中病例2为老年女性.病例1主要累及垂体前叶,并侵犯海绵窦和视交叉,病例2则主要累及下丘脑和漏斗部,病例3则是垂体前叶和垂体柄均受累.病例1手术后4个月第一次复发,给予大剂量甲基泼尼龙治疗(HDMPT)有效,但停药后14个月再次复发;病例2中等剂量强的松治疗停药16个月后复发,而病例3则在HDMPT治疗,糖皮质激素减量的过程中复发.3例患者复发后均以糖皮质激素联合硫唑嘌呤治疗16周,疗效良好,停药后随访3例患者,MRI显示LYH均无复发,而且病例1和3垂体功能正常.

Abstract：

All three cases of recurrent lymphocytic hypophysitis were female, one of them being 70-yearold postmenopausal woman.Adenohypophysis, cavernous sinus, and optic chiasm were involved in case 1,hypothalamus and neurohypophysis were invaded in case 2, and adenohypophysis and hypophyseal stalk were involved in case 3.Relapse occured 4 months after operation in case 1, then high dosage methylprednisolone pulse therapy (HDMPT) brought about a remission lasting for 14 months before second relapse set in.Relapse occurred in case 2 at 16 months after prednisone treatment was discontinued, and case 3 recurred during the period of dose reduction.All three patients were then treated with glucocorticoid plus azathioprine for 16 weeks, and good response was seen in 3 cases.During follow-up, the symptoms were relieved and significant reduction of lesion was revealed by MRI in all thee patients, and the pituitary function was resumed in case1and 3.     

Prolactinoma coexistent with granulomatous hypophysitis

Summary A 54-year-old man with a long-lasting left-sided headache and biochemical evidence of hyperprolactinemia was found to have a large chromophobe pituitary adenoma, which concurred with a granulomatous hypophysitis. The mechanism of the inflammatory reaction is discussed and the immunohistochemical and ultrastructural findings of this hitherto undescribed combination of lesions are presented.