Coping strategies have a strong impact on quality of life, depression, and embitterment in patients with Cushing's disease.

PURPOSE: Quality of life (QoL) and psychosocial well-being are substantially impaired in patients with Cushing's disease (CD), not only at the acute illness stage but also after therapy; however, the reason for these impairments remains unclear. METHODS: In this cross-sectional, patient-reported outcome study, we conducted a postal survey on psychosocial impairment and coping strategies in patients after surgical treatment of CD in three large tertiary referral centers. In total, 176 patients with CD completed a compilation of self-assessment inventories pertaining to depression (Hospital Anxiety and Depression Scale, HADS), QoL (Short Form SF-36, Tuebingen CD; Tuebingen CD-25), coping style (Freiburg questionnaire on coping with illness, FKV-LIS), and embitterment (Bern Embitterment Inventory), on average 6.8 ± 6.66 years after surgery. Regression analyses were performed to identify predictors of psychosocial impairment. RESULTS: At the time of the study, 21.8 % of patients suffered from anxiety, 18.7 % experienced an above-average feeling of embitterment, and 13.1 % suffered from depression. Maladaptive coping styles (FKV-LIS subscales depressive coping and minimizing importance) emerged as robust and strong predictors of psychosocial impairment in all inventories; while age, sex, and hydrocortisone intake failed to explain the variance in these measures. CONCLUSION: Similar to several studies in non-pituitary patient cohorts (e.g., patients with multiple sclerosis or lower back pain), our results indicate that psychosocial impairment in CD is significantly influenced by how the patient deals with the illness. Therefore, psychological training of positive coping styles could be a helpful complementary therapy in the overall treatment strategy of CD.

Validation of the Tuebingen CD-25 Inventory as a Measure of Postoperative Health-Related Quality of Life in Patients Treated for Cushing's Disease.

BACKGROUND: To evaluate the construct and criterion validity of the Tuebingen Cushing's disease quality of life inventory (Tuebingen CD-25) for application in patients treated for Cushing's disease (CD). METHODS: A total of 176 patients with adrenocorticotropin hormone-dependent CD (144 of them female, overall mean age 46.1 ± 13.7 years) treated at 3 large tertiary referral centers in Germany were studied. Construct validity was assessed by hypothesis testing (self-perceived symptom reduction assessment) and contrasted groups (patients with vs. without hypercorticolism). For this purpose, already existing data from 55 CD patients was used, representing the hypercortisolemic group. Criterion validity (concurrent validity) was assessed in relation to the Cushing's quality of life questionnaire (CushingQoL), the Short Form 36 health survey (SF-36), and the body mass index (BMI). RESULTS: Patients with self-perceived remarkable symptom reduction had significant lower Tuebingen CD-25 scores (i.e. better health-related quality of life) than patients with self-perceived insufficient symptom reduction (p < 0.05). Similarly, the mean scores of the Tuebingen CD-25 scales were lower in patients without hypercortisolism (total score 27.0 ± 17.2) compared to those with hypercortisolism (total score 45.3 ± 22.1; each p < 0.05), providing evidence for construct validity. Criterion validity was confirmed by the correlations between the Tuebingen CD-25 total score and the CushingQoL (Spearman's coefficient -0.733), as well as all scales of the SF-36 (Spearman's coefficient between -0.447 and -0.700). CONCLUSION: The analyses presented in this large-sample study provide robust evidence for the construct and criterion validity of the Tuebingen CD-25.

Similar psychopathological profiles in female and male Cushing's disease patients after treatment but differences in the pathogenesis of symptoms.

BACKGROUND: Female Cushing's disease (CD) patients with active disease present more frequently with depression compared to their male co-sufferers. This study investigated whether the gender difference prevails after remission and whether gender-specific factors contributing to mental health exist. METHODS: 72 biochemically cured CD patients (11 male, mean age 45.9 ± 13.7 years) who underwent transsphenoidal tumour removal filled out the Symptom Checklist-90-Revised inventory on average 42.1 ± 32.9 months after surgery. Multiple regression analyses included the following independent factors: (i) age, (ii) presence of comorbidities, (iii) presence of hypocortisolism, (iv) presence of hypopituitarism, (v) disease duration until diagnosis, (vi) time elapsed since surgery, and (vii) postoperative radiotherapy to predict postoperative psychopathology. RESULTS: Regarding the Global Severity Index, 23.0% of the female and 27.3% of the male CD patients presented with abnormal scores. In all nine dimensions, psychopathological abnormalities were present in both female and male patients with the same frequency and intensity (each p > 0.05). Prolonged time to diagnosis was a strong predictive factor for worse psychopathological status only in male patients. Among female patients, only the presence of comorbidities and to some extent pituitary deficiencies were related to psychopathological status. CONCLUSIONS: During the remission phase of CD, female and male patients present with similar psychopathological profiles. In males, long-term biochemical effects of previous hypercortisolism seem to be salient for psychopathology. In contrast, in females, the presence of comorbidities/stressors they have to cope with is the predictive factor for psychopathology. The results underline gender differences in CD and the need to separate them on various issues.

Transcriptome analysis of MENX-associated rat pituitary adenomas identifies novel molecular mechanisms involved in the pathogenesis of human pituitary gonadotroph adenomas.

Gonadotroph adenomas comprise 15-40% of all pituitary tumors, are usually non-functioning and are often large and invasive at presentation. Surgery is the first-choice treatment, but complete resection is not always achieved, leading to high recurrence rates. As gonadotroph adenomas poorly respond to conventional pharmacological therapies, novel treatment strategies are needed. Their identification has been hampered by our incomplete understanding of the molecular pathogenesis of these tumors. Recently, we demonstrated that MENX-affected rats develop gonadotroph adenomas closely resembling their human counterparts. To discover new genes/pathways involved in gonadotroph cells tumorigenesis, we performed transcriptome profiling of rat tumors versus normal pituitary. Adenomas showed overrepresentation of genes involved in cell cycle, development, cell differentiation/proliferation, and lipid metabolism. Bioinformatic analysis identified downstream targets of the transcription factor SF-1 as being up-regulated in rat (and human) adenomas. Meta-analyses demonstrated remarkable similarities between gonadotroph adenomas in rats and humans, and highlighted common dysregulated genes, several of which were not previously implicated in pituitary tumorigenesis. Two such genes, CYP11A1 and NUSAP1, were analyzed in 39 human gonadotroph adenomas by qRT-PCR and found to be up-regulated in 77 and 95% of cases, respectively. Immunohistochemistry detected high P450scc (encoded by CYP11A1) and NuSAP expression in 18 human gonadotroph tumors. In vitro studies demonstrated for the first time that Cyp11a1 is a target of SF-1 in gonadotroph cells and promotes proliferation/survival of rat pituitary adenoma primary cells and cell lines. Our studies reveal clues about the molecular mechanisms driving rat and human gonadotroph adenomas development, and may help identify previously unexplored biomarkers for clinical use.

Tuebingen CD-25 is a sensitive tool to investigate health-related quality of life in Cushing's disease patients in the course of the disease.

BACKGROUND: The primary object was to investigate whether the Tuebingen CD-25 captures changes in health-related quality of life (HRQoL) sensitively in Cushing's disease (CD) and to identify factors that favour postoperative HRQoL. METHODS: 17 CD patients were scheduled for transsphenoidal tumour removal and filled out the inventory before and after surgery. The mean time elapsed after surgery was 14.4 ± 11.3 months. All patients were in remission at the second timepoint of investigation. RESULTS: HRQoL as assessed with the Tuebingen CD-25 improved significantly after successful surgical tumour removal. A large effect size (Cohen's d = 0.84) in the total score indicates good sensitivity to change. 13 patients (76.5%) showed impaired HRQoL preoperatively compared with a general population sample. Postoperatively, 35.3% of the patients still suffered from an impaired HRQoL. Stepwise linear regression analysis revealed that less comorbidities (≤2) and greater morning cortisol decrease were promotive factors for better postoperative HRQoL (p < 0.05). The postoperative improvement in HRQoL could be best predicted by the presence of preoperative HRQoL impairment and age of the patients, i.e. patients who were younger were more likely to improve. Moreover, patients without postoperative pituitary deficiencies improved significantly more in the cognition scale. A tendency towards more improvement in overall HRQoL was observed in non-hypocortisolaemic patients. CONCLUSIONS: The Tuebingen CD-25 has proved to be a capable and sensitive instrument to investigate HRQoL in the course of disease. The number of postoperative comorbidities had the greatest impact on postoperative well-being.

Health-related quality of life and psychiatric symptoms improve effectively within a short time in patients surgically treated for pituitary tumors--a longitudinal study of 106 patients.

BACKGROUND: Reduced health-related quality of life (HRQoL) is a common complaint in patients suffering from pituitary tumors. Although successful tumor treatment has been reported to lead to an improvement in perceived HRQoL, the temporal gradient at which these improvements occur has not been fully addressed. METHODS: Using three validated health-related questionnaires (SF-36, SCL-90-R, QLS-H), we assessed HRQoL in 106 adult patients harboring pituitary tumors (mean age 48.0 ± 16.0 years) before as well as 3 and 12 months after initiation of treatment. The AcroQoL questionnaire was additionally applied in acromegalic patients. RESULTS: There was a significant improvement in all but one scale (role-physical) of the SF-36 questionnaire and all but two scales (interpersonal sensitivity, paranoid ideation) of the SCL-90-R, the QLS-H score and the AcroQoL subscales within 3 months after surgical treatment. The trend to amelioration continued at the 12 month re-assessment, but did not reach statistical significance. Linear regression analyses revealed that younger age and male gender favor a more distinct improvement of HRQoL after treatment. CONCLUSIONS: HRQoL is considerably reduced before treatment for pituitary disease. Improvement is an early postoperative phenomenon and occurs within 3 months after treatment. Men and younger patients are more likely to improve within this time span.

The development of the Tuebingen Cushing's disease quality of life inventory (Tuebingen CD-25). Part I: construction and psychometric properties.

OBJECTIVE: To develop a disease-specific questionnaire for Cushing's disease (CD), the Tuebingen Cushing's disease quality of life inventory (Tuebingen CD-25). METHODS: Sources for item generation consisted of technical literature, interviews with patients and the rating of neurosurgeons, endocrinologists and a neuropsychologist. A preliminary inventory with 64 items was handed out to 63 CD patients. Twenty-eight patients filled out the questionnaire preoperative, the remaining 35 patients evaluated their health-related quality of life (HRQoL) retrospectively. Item reduction and scale generation followed the principles of classical test theory. Validation was performed with the WHOQoL-BREF. RESULTS: The final version of the Tuebingen CD-25 contained 25 items, showed high reliability (Cronbach's alpha = 0.93) and validity (r = -0.65) and includes the subdomains Depression, Sexual Activity, Environment, Eating Behaviour, Bodily Restrictions and Cognition. The retrospective rating of the Tuebingen CD-25 showed similar results compared to the pretreatment group. We found a non-linear correlation between the Tuebingen CD-25 scores and patients' age, younger (21-30 years) and middle-aged (51-60 years) patients having inferior HRQoL than patients between 31 and 50 years and older than 61 years. Preoperative 24 h urinary free cortisol (UFC) levels correlated significantly with the subscale Cognition and only marginally failed significance level for the subscale Eating Behaviour, while preoperative cortisol and ACTH levels did not correlate with any scale. CONCLUSION: The Tuebingen CD-25 is a valid and reliable instrument to evaluate HRQoL in CD. Based on impairment of HRQoL for the different subdimensions, specific support can be offered to the patients.

Contemporary microsurgical concept for the treatment of Cushing's disease: endocrine outcome in 83 consecutive patients.

BACKGROUND: Evidence suggests that occult adenoma remnants are responsible for persistent Cushing's disease (CD) following transsphenoidal surgery (TSS). To optimize the outcome, we have adapted our microsurgical concept. The influence of our surgical strategy on remission rate and pituitary function is presented. DESIGN AND PATIENTS: 83 patients undergoing TSS for newly diagnosed CD. An enlarged resection was performed in 36 patients. A modified exploration technique with radial incisions was performed in 19 patients in whom an adenoma was not readily detectable. RESULTS: The overall remission rate of primary surgery was 84·3% (70/83). A remission rate of 87·5% (63/72) was achieved in microadenomas. Six patients with microadenomas were re-operated for persistence, and hypercortisolism was corrected in five of them. With re-operation included, the overall remission rate for microadenomas was 94·4%. No procedure-related complications occurred in primary surgery. Of the patients in remission, 72·5% had early postoperative random cortisol levels below 2 µg/dl, 17·4% had cortisol levels between 2 and 5 µg/dl, and 10·1% had cortisol levels >5 µg/dl. 15·2% of the patients with microadenomas developed postoperative partial hypopituitarism and 3% diabetes insipidus. No increased rate of hypopituitarism was found with enlarged adenomectomy compared to selective adenomectomy. Only a slightly higher rate of partial hypopituitarism (23·1%) was found if extensive exploration was required. CONCLUSION: With our microsurgical concept, a high initial cure rate is achievable with minimal surgical morbidity. Enlarged adenomectomy has no adverse effect on the rate of postoperative hypopituitarism. Early repeat surgery is a successful option if CD persists.

The development of the Tuebingen Cushing's disease quality of life inventory (Tuebingen CD-25). Part II: normative data from 1784 healthy people.

OBJECTIVE: In part I of the study, a health-related quality of life (HRQoL) inventory for Cushing's disease (CD), the Tuebingen Cushing-25 quality of life inventory (Tuebingen CD-25) was developed. In this second part, we aimed to assess normative data from healthy controls (HC) with which the individual patients' scores can be compared. DESIGN: Cross-sectional study. PARTICIPANTS: Sixty-three patients with CD (52 women, 11 men) and 1784 HC (1210 women, 574 men) filled out the Tuebingen CD-25. MEASUREMENTS: HC received the inventory as a nondisease-specific quality of life questionnaire omitting the introductory sentence 'Because of my Cushing's disease' which was included in the CD group to be able to compare HRQoL in persons with and without CD. Slight and severe impairments were categorized according to the following principles: percent ranges >84.0% were classified as slight and percent ranges >95.0% as severe impairment. RESULTS: In 28.6% of our patients with CD, we found slight and in 41.3% severe impairment in the Total Score of the Tuebingen CD-25 compared with HC. Less than one-third of our patient sample presented with unimpaired HRQoL. In the patient population, impairment was found in all scales of the Tuebingen CD-25 to a similar extent (P = 0.444), pointing to the fact that all HRQoL domains are similarly impaired. We observed that female patients perceived a worse HRQoL than men in the domains depressive symptoms and social environment (P < 0.05). CONCLUSION: The Tuebingen CD-25 is a feasible instrument to assess HRQoL in CD in a clinical and investigative setting and provides normative data for all age groups and genders.

Postoperative intracranial seeding of craniopharyngiomas--report of three cases and review of the literature.

PURPOSE: Seeding of craniopharyngioma has been rarely reported. We present three cases that ectopically recurred with seeding along the surgical route and CSF spaces. METHODS: The first patient was a 13-year-old boy who had initially undergone radical excision of a suprasellar and retrosellar craniopharyngioma by a right pterional approach. Postoperative MRI showed no evidence of residual tumor. Two years later, MRI revealed a local recurrence and in addition a separated cystic tumor on the right side adjacent to the middle cerebral artery consistent with seeding along the surgical route. Both tumors were removed by re-operation. On histopathological examination, both, the local recurrent tumor and the distant deposit turned out to be adamantinomatous craniopharyngiomas. The second patient was a 27-year-old woman who was operated on for the first time via a right pterional and 1 year later for a recurrent craniopharyngioma via a transsphenoidal approach. Two years later, MRI revealed a right fronto-lateral intracranial metastasis at the site of the former craniotomy, which was removed by re-craniotomy. This deposit in the operative pathway was found to be an adamantinomatous craniopharyngioma, as was the initial tumor. The third patient was a 42-year-old man who was operated on 10 years ago for the first time via a right fronto-temporal approach. The recent control MRT revealed a right parietal intracranial tumor with peripheral contrast enhancement, which was located distant to the former craniotomy. The tumor was removed and histopathological examination revealed an adamantinomatous craniopharyngioma in accordance with the initial tumor. Postoperatively, the three patients were neurologically intact. CONCLUSION: Although craniopharyngiomas exhibit a benign histopathological pattern, cerebrospinal fluid seeding along the surgical route or along the CSF pathways has been observed. Ectopic recurrence of craniopharyngioma suggests that meticulous protection of the whole surgical field and careful handling of the tumor during the operation are required. It should be emphasized that long-term follow-up is mandatory, even in patients undergoing a total removal.

Aftercare in patients with Cushing's disease and acromegaly: is there room for improvement?

PURPOSE: Evaluation of aftercare, medical therapy and remission rate for Cushing's disease (CD) and acromegaly (AC). METHODS: Fifty-eight CD and 83 AC patients operated on over 10 years were carefully evaluated. The patients received a disease-related questionnaire and were invited for a follow-up at the outpatient clinic of the Department of Neurosurgery. Thirty-three CD and 52 AC patients returned the questionnaire; 25 CD and 37 AC patients participated personally. CD patients underwent a dexamethasone suppression test, and IGF-1 levels were assessed in AC patients. Data on postoperative therapy were assessed. RESULTS: 84.8% of those with CD and 75.0% of those with AC had been followed by endocrinologists; 9.1% of CD and 1.9% of AC patients had been under no aftercare; 96% of CD patients were in remission. A recurrence occurred in four patients, two were newly detected by our study. IGF-1 was postoperatively normalized in 25 of 37 AC patients (67.6%). Twenty patients remained in remission, five relapsed. One patient received medical therapy upon recurrence and presented normal IGF-1; four patients with relapse had not been treated yet. Ten of 12 patients with elevated early postoperative IGF-1 received medical therapy that was commenced within 6 months in nine cases. The mean duration of medical therapy was 36 months (range 2-92). IGF-1 was still elevated at the last follow-up in eight of these ten patients, all of them receiving monotherapy. In four patients, the dose had been increased once. CONCLUSIONS: The postoperative transfer back to the endocrinologist after operative treatment is achieved well in both groups. In uncured AC, medical therapy is initiated early, but options of therapy offer room for improvement. Therapy of recurrence is delayed in both pathologies.

ACTH- and prolactin-producing pituitary gland microadenoma with biphasic features of atypia and intermediate filament expression.

Herein, we report the case of a 28-year old woman clinically presenting with unclear weight gain over the last years. The patient displayed facial and neck edema in combination with unobtrusive striae distensae. Endocrinological examinations led to the diagnosis of Cushing's disease. Neuroradiological examination revealed an intrasellar tumor mass of 7 mm in diameter. Subsequently, transsphenoidal tumor resection was performed. Histological and immunohistochemical investigations revealed a pituitary gland adenoma showing a biphasic tumor growth pattern with two morphologically different tumor areas producing ACTH and prolactin respectively. Co-expression of ACTH and prolactin is exceedingly rare in pituitary adenoma. To our surprise, both tumor areas exhibited features of atypia consisting in elevated MIB-1 proliferation index in the ACTH-producing portion as well as p53 expression selectively in the prolactin-producing tumor parts. To our knowledge, this is the first case of an ACTH- and prolactin-producing pituitary gland adenoma exhibiting biphasic features of atypia.

From first symptoms to final diagnosis of Cushing's disease: experiences of 176 patients.

OBJECTIVE: To obtain structured information on the diagnostic delay in patients with Cushing's disease (CD) from the patients perspective to provide leverage points for earlier diagnosis. DESIGN: The study includes 176 patients with ACTH-dependent CD who had received pituitary surgery completed a self-developed questionnaire on their symptomatology before the illness was diagnosed, the course and length of the diagnostic process, and the role of the involved health care professionals. METHODS: Data were analyzed statistically. Answers in free text options were categorized and counted. RESULTS: The overall diagnostic process took 3.8±4.8 years (median 2 years), during which 4.6±3.8 (1-30) physicians were consulted, most frequently the family physician (FP; 83.0%). The presented symptoms were various and often vague, e.g. 'poor general condition' (at FPs), or very common in the field of the visited specialist (i.e. 'skin changes' at dermatologists). Women recognized the first CD symptoms themselves significantly more frequently than men, whereas physicians recognized CD symptoms significantly more frequently in males. CONCLUSION: A clear difficulty of diagnosing CD seems that patients describe isolated symptoms to the FP or the respective specialists according to their fields of specialization. As FPs are contacted most frequently, they should be trained to recognize the broad spectrum of CD symptoms, especially in female patients with weight gain, and initiate endocrinological referral.

"Imagine your neighbor mows the lawn": a pilot study of psychological sequelae due to awake craniotomy: clinical article.

OBJECT: Although it has been reported that awake neurosurgical procedures are well tolerated, the long-term occurrence of general psychological sequelae has not yet been investigated. This study assessed the frequency and effects of psychological symptoms after an awake craniotomy on health-related quality of life (HRQOL). METHODS: Sixteen patients undergoing an awake surgery were surveyed with a self-developed questionnaire, the Posttraumatic Stress Disorder Inventory For Awake Surgery Patients, which adopts the core components of the Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) posttraumatic stress disorder (PTSD) criteria. The mean time between surgery and data collection was 97.3 ± 93.2 weeks. Health-related quality of life was assessed with the 36-Item Short Form Health Survey. RESULTS: Forty-four percent of the patients stated that they had experienced either repetitive distressing recollections or dreams related to the awake surgery, 18.8% stated persistent avoidance of stimuli associated with the awake surgery, and symptoms of increased arousal occurred in 62.5%. Two patients presented with postoperative psychological sequelae resembling PTSD symptoms. Younger age at surgery and female sex were risk factors for symptoms of increased arousal. The experience of intense anxiety during awake surgery appears to favor the development of postsurgical PTSD symptoms, while recurrent distressing recollections particularly affect HRQOL negatively. CONCLUSIONS: In many cases awake craniotomy is necessary to preserve language and motor function. However, in some cases awake craniotomy can lead to postoperative psychological sequelae resembling PTSD symptoms. Therefore, possible long-term effects of an awake surgery should be considered and discussed with the patient when planning this type of surgery.

Treatment Options in Cushing's Disease.

Endogenous Cushing's syndrome is a grave disease that requires a multidisciplinary and individualized treatment approach for each patient. Approximately 80% of all patients harbour a corticotroph pituitary adenoma (Cushing's disease) with excessive secretion of adrenocorticotropin-hormone (ACTH) and, consecutively, cortisol. The goals of treatment include normalization of hormone excess, long-term disease control and the reversal of comorbidities caused by the underlying pathology. The treatment of choice is neurosurgical tumour removal of the pituitary adenoma. Second-line treatments include medical therapy, bilateral adrenalectomy and radiation therapy. Drug treatment modalities target at the hypothalamic/pituitary level, the adrenal gland and at the glucocorticoid receptor level and are commonly used in patients in whom surgery has failed. Bilateral adrenalectomy is the second-line treatment for persistent hypercortisolism that offers immediate control of hypercortisolism. However, this treatment option requires a careful individualized evaluation, since it has the disadvantage of permanent hypoadrenalism which requires lifelong glucocorticoid and mineralocorticoid replacement therapy and bears the risk of developing Nelson's syndrome. Although there are some very promising medical therapy options it clearly remains a second-line treatment option. However, there are numerous circumstances where medical management of CD is indicated. Medical therapy is frequently used in cases with severe hypercortisolism before surgery in order to control the metabolic effects and help reduce the anestesiological risk. Additionally, it can help to bridge the time gap until radiotherapy takes effect. The aim of this review is to analyze and present current treatment options in Cushing's disease.

Pituitary adenomas and craniopharyngiomas are CDX2 negative neoplasms.

OBJECTIVES: Previous studies have shown an inverse correlation between the expression of CDX2 (also known as CDX3) and tumour grade, stage and lymph node dissemination in colorectal adenomas and adenocarcinomas. Although less frequent, expression of CDX2 has also been reported in various other epithelial tissues and carcinomas. While many neoplasms have been studied, to date, no data is available on CDX2 expression in craniopharyngiomas. Furthermore, only very few data are available on CDX2 expression in normal pituitary gland tissue and/or pituitary adenomas. MATERIAL AND METHODS: We investigated CDX2 expression in 28 normal pituitary glands, 75 pituitary adenomas of varying hormonal activity (including 7 invasive adenomas and 7 atypical adenomas) and 23 craniopharyngiomas (17 adamantinous and 6 papillary) in tissue microarrays. RESULTS: None of the pituitary adenomas, craniopharyngiomas and normal pituitary glands showed expression of CDX2. CONCLUSIONS: There is no evidence for that CDX2 might play a role in tumourigenesis, invasive growth or tumour recurrence of pituitary adenomas or in tumourigenesis of craniopharyngiomas. But, presence of CDX2 expression might be useful in distinguishing intrasellar metastases from primary tumours of the sellar region.

Synovial cyst of the thoracic spine causing myelopathy.

STUDY DESIGN: Pre- and postoperative follow-up of a patient with progressive myelopathy caused by a thoracic synovial cyst developing within 6 months. OBJECTIVE: To present the natural history of a developing thoracic synovial cyst and to highlight this unusual case. SUMMARY OF BACKGROUND DATA: Thoracic synovial cysts are a rare finding and can produce myelopathy from spinal cord compression. The patient presented with progressing spasticity and sensory loss of both legs. Hereditary motor-sensory neuropathy was suspected and excluded by a molecular genetic examination. The cause of deterioration was finally found in an MRI of the thoracic spine revealing the rapid growth of a synovial cyst that had been detected in a previously performed MRI 6 months earlier. METHODS: Clinical and MRI details are presented documenting the natural history and development of a thoracic synovial cyst causing myelopathy. RESULTS: The thoracic synovial cyst was successfully decompressed. Stabilization and fusion were not performed. The myelopathy resolved after surgery and the patient stays well in the 6-months follow-up. CONCLUSION: Although thoracic synovial cysts are a rare finding, they have to be considered as a source for progressive myelopathy. If an MRI of the spine does not reveal any pathologies, it should be repeated even after a short period of time if the patient's condition deteriorates, continuously to detect newly developed findings.

Rathke's cleft cyst rupture as potential initial event of a secondary perifocal lymphocytic hypophysitis: proposal of an unusual pathogenetic event and review of the literature.

Herein, we report on a lymphocytic hypophysitis related to a ruptured Rathke's cleft cyst which is not associated with pregnancy. A 45-year-old woman initially presented with headache and temporary double vision followed by amenorrhea. Preoperative imaging showed an intra- and suprasellar cystic mass. Complete resection of the tumor mass was performed via a transnasal, transseptal approach. Pathological examination displayed lymphocytic infiltrates within fibrotic tissue and residual pituitary cells accompanied by epithelial tissue of a Rathke's cleft cyst. The strongest inflammatory reaction was observed at the site of disrupture of the cyst integrity, suggesting that high protein levels from ruptured Rathke's cleft cyst might have triggered a lymphocytic hypophysitis. Our review of the literature provides further insights regarding the clinical behavior and different histological types of the lesions as well as the inflammatory changes that can occur in Rathke's cleft cysts.