| 31. |
- Erfurth, Eva Marie, et al.
(author)
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Is there an increase in second brain tumours after surgery and irradiation for a pituitary tumour?
- 2001
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In: Clinical Endocrinology. - : Wiley. - 1365-2265 .- 0300-0664. ; 55:5, s. 613-616
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Journal article (peer-reviewed)abstract
- Objective To assess the incidence of second brain tumours in patients operated and irradiated for pituitary tumours. Design and patients The study base consisted of a consecutive series of 325 patients operated and irradiated for pituitary tumours, excluding patients with acromegaly and Cushing's disease. Comparison was made with the general population from the same catchment area as the patients. The follow-up period started in 1958 and on an individual basis patients were followed from the onset of postoperative irradiation until December 1995, or until date of death, emigration or a second brain tumour diagnosis, whichever occurred first. Results Three brain tumours (two astrocytomas and one meningioma) were observed, compared with 1.13 expected (standardized incidence ratios (SIR) 2.7; 95% confidence interval (CI) 0.6-7.8). Conclusion The present study gives no firm support for an increased incidence of a second brain tumour in patients operated and irradiated for pituitary tumours. A crude meta-analysis of the present and previously published cohort studies of patients with irradiated pituitary tumours gives an SIR of 6.1 (95% Cl 3.16-10.69). Thus, the results of the meta-analysis are in favour of an increased risk for second brain tumours. A genetic trait that predisposes to both pituitary tumours and brain tumours is an alternative causal factor. There is no definite proof that cranial irradiation per se is the causal factor. This question cannot be fully answered until sufficient cohort studies of nonirradiated pituitary tumour patients have been carried out.
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| 32. |
- Erfurth, Eva Marie, et al.
(author)
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Mortality and morbidity in adult craniopharyngioma.
- 2013
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In: Pituitary. - : Springer Science and Business Media LLC. - 1573-7403 .- 1386-341X. ; 16, s. 46-55
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Journal article (peer-reviewed)abstract
- A craniopharyngioma (CP) is an embryonic malformation of the sellar and parasellar region. The annual incidence is 0.5-2.0 cases/million/year and approximately 60 % of CP are seen in adulthood. Craniopharyngiomas have the highest mortality of all pituitary tumors. Typical initial manifestations at diagnosis in adults are visual disturbances, hypopituitarism and symptoms of elevated intracranial pressure. The long-term morbidity is substantial with hypopituitarism, increased cardiovascular risk, hypothalamic damage, visual and neurological deficits, reduced bone health, and reduction in quality of life and cognitive function. Therapy of choice is surgery, followed by cranial radiotherapy in about half of the patients. The standardised overall mortality rate varies 2.88-9.28 in cohort studies. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. Women with CP have an even higher risk.
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| 33. |
- Erfurth, Eva Marie
(author)
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Obesity and metabolic disturbances in adamantinomatous craniopharyngioma patients
- 2017
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In: Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma. - Cham : Springer International Publishing. - 9783319518909 - 9783319518886 ; , s. 85-99
-
Book chapter (peer-reviewed)abstract
- An adamantinous craniopharyngioma (CP) is rare benign pituitary tumour often growing invasively and thereby affecting the hypothalamus. The recurrence rate in CP is high and therapy of choice is surgery, followed by cranial radiotherapy (CRT) in about half of the patients. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. The majority of patients have an extended hypopituitarism that needs balanced hormone supplementation. About half of the patient population suffer from hypothalamic damage due to the tumour or operation. These patients suffer from hypothalamic obesity together with increased vascular morbidity and compromised bone health, thirst disorders as well as somnolence and sleep apnea. Cardiovascular risk factors need up front therapy. There is a need for centralising this rare aggressive and difficult tumour to only a few centres and that it will be managed by a multidisciplinary team with experienced neurosurgeons, endocrinologists, neurooncologists, neuroradiologists, and neuroopthamologists for a better prognosis.
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| 34. |
- Erfurth, Eva Marie, et al.
(author)
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Pituitary disease mortality: is it fiction?
- 2013
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In: Pituitary. - : Springer Science and Business Media LLC. - 1573-7403 .- 1386-341X. ; 16:3, s. 402-412
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Journal article (peer-reviewed)abstract
- During the last 20 years a tremendous improvement in the care of patients with pituitary tumors and of hypopituitarism has been achieved. If we resolve most of the possible causes of the increased cardiovascular disease and stroke mortality a normal survival is expected in these patients. Recently, a large population based study showed a decline in the risk of non-fatal stroke and of non-fatal cardiac events in GH deficient patients. This improvement was achieved by complete hormone replacement, including long term GH replacement, together with prescription of cardio protective drugs. If we follow the latest achievements in pituitary imaging, surgery techniques, hormone substitutions, cardio protective medications, we would expect a normal longevity in these patients. This review will focus on; (1) pituitary insufficiencies and hormone substitutions, (2) modes of cranial radiotherapy, and (3) new techniques in the surgery of a pituitary adenoma.
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| 35. |
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| 36. |
- Erfurth, Eva Marie
(author)
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Uncertainties in endocrine substitution therapy for central endocrine insufficiencies: growth hormone deficiency.
- 2014
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In: Handbook of Clinical Neurology. - 0072-9752. ; 124, s. 407-416
-
Journal article (peer-reviewed)abstract
- The growth hormone deficiency (GHD) syndrome is associated with several metabolic abnormalities and it has been postulated that the increased cardiovascular disease (CVD) morbidity and mortality in GHD patients may be related to the missing metabolic effects of GH. Many CVD risk factors show improvements after GH therapy. Reduced bone mineral density (BMD) has been recorded both in patients with isolated GHD and in those with multiple pituitary deficiencies, indicating that GHD per se is responsible for the low BMD in both types of patients. These matters are, however, more complicated, as hypopituitary patients with GHD may have different phenotypes due to differences in underlying diagnoses. These phenotypes may not be clear-cut in individual patients. Moreover, patients may transit between different phenotypes over time due to extension of the pathology in the pituitary and/or the consequences of the treatment (surgery and/or radiotherapy). Three different phenotypes of hypopituitary patients will be discussed, with a focus on CVD risk and bone health: (1) patients with isolated GHD, e.g. due to prophylactic cranial radiotherapy for lymphoblastic leukaemia in childhood; (2) patients with GHD and multiple hormone deficiencies due to pituitary macroadenomas treated by surgery; (3) patients with GHD caused by craniopharyngiomas with multiple hormone deficiencies and hypothalamic involvement, where hypothalamic damage frequently dominates the positive metabolic effects of GH therapy. These phenotypes illustrate the differential impact of various pituitary pathologies on the phenotype of patients with GHD.
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| 37. |
- Erfurth, Eva Marie
(author)
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Update in Mortality in GH treated patients.
- 2013
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In: Journal of Clinical Endocrinology and Metabolism. - : The Endocrine Society. - 1945-7197 .- 0021-972X. ; 98:11, s. 4219-4226
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Journal article (peer-reviewed)abstract
- During GH therapy for 2.3-9.6 years a male adult onset (AO) GH deficient (GHD) patient with a diagnosis of a non-functioning adenoma, have no increased all-cause mortality. However, women with AO GHD are still at a slightly higher risk. This general improvement in mortality is due to; more contemporary regiment of cardiovascular drugs, a refinement of surgical procedures, besides GH therapy improved hormone replacement regimens with lowered glucocorticoid replacement, and updated approaches of sex steroids for women, and less use of cranial radiotherapy. The underlying disease is the most important predictor for mortality: e.g. a craniopharyngioma, malignant causes of hypopituitarism, previous Cushing's disease, presence of diabetes insipidus/aggressive tumors. The main cause of increased mortality was cerebrovascular diseases and of infectious/repiratory diseases in ACTH deficient patients. Further, there was a significant impact of young age at disease onset and of death from secondary brain tumors, with a higher risk after cranial radiotherapy.Four cohorts of GH treated childhood onset (CO) GHD patients have been published. Two of them included only patients with idiopathic isolated GH deficiency (IGHD), neurosecretory dysfunction, idiopathic short stature (ISS) or born short for gestational age (SGA). Increased mortality in circulatory disorders, ill defined diseases and bone cancer was recorded in one, but not in the other smaller study, where suicide and accidents caused the majority of deaths. A third CO GHD cohort included patients with a background of malignant tumors, craniopharyngioma, pituitary adenomas, pituitary aplasia/hypoplasia, and trauma. An increase of all cause mortality was recorded in both males and females. The fourth cohort included IGHD and ISS (60%), but also diagnosis e.g. chronic renal failure, and Turner's syndrome. In these latter studies an underlying serious condition was the most important factor for death, with CNS tumors (recurrent or new tumor) being the leading cause of mortality.
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| 38. |
- Fjalldal, Sigridur, et al.
(author)
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Brain white matter lesions are associated with reduced hypothalamic volume and cranial radiotherapy in childhood-onset craniopharyngioma
- 2021
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In: Clinical Endocrinology. - : Wiley. - 0300-0664 .- 1365-2265. ; 94:1, s. 48-57
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Journal article (peer-reviewed)abstract
- Context: White matter lesions (WML) are caused by obstruction of small cerebral vessels associated with stroke risk. Craniopharyngioma (CP) patients suffer from increased cerebrovascular mortality. Objective: To investigate the effect of reduced HT volume and cranial radiotherapy (CRT) on WML in childhood-onset CP patients. Design: A cross-sectional study of 41 patients (24 women) surgically treated childhood-onset CP in comparison to controls. Setting: The South Medical Region of Sweden (2.5 million inhabitants). Methods: With magnetic resonance imaging (MRI), we analysed qualitative measurement of WML based on the visual rating scale of Fazekas and quantitative automated segmentation of WML lesion. Also, measurement HT volume and of cardiovascular risk factors were analysed. Results: Patients had a significant increase in WML volume (mL) (P =.001) compared to controls. Treatment with cranial radiotherapy (CRT) vs no CRT was associated with increased WML volume (P =.02) as well as higher Fazekas score (P =.001). WML volume increased with years after CRT (r = 0.39; P =.02), even after adjustment for fat mass and age. A reduced HT volume was associated with increased WML volume (r = −0.61, P <.001) and explained 26% of the variation (r2 = 0.26). Altogether, 47% of the WML volume was explained by age at investigation, HT volume and CRT. Patients with more WML also had higher cardiovascular risk. Conclusions: CRT may be associated directly with increased WML volume or indirectly with reduced HT volume associated with higher cardiovascular risk. Risk factors should be carefully monitored in these patients.
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| 39. |
- Fjalldal, Sigridur, et al.
(author)
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Hypothalamic Involvement Predicts Cognitive Performance and Psychosocial Health in Long-term Survivors of Childhood Craniopharyngioma
- 2013
-
In: Journal of Clinical Endocrinology and Metabolism. - : Endocrine Society. - 0021-972X .- 1945-7197. ; 98:8, s. 3253-3262
-
Journal article (peer-reviewed)abstract
- Context: Hypothalamic damage caused by craniopharyngioma (CP) is associated with poor functional outcome. less thanbrgreater than less thanbrgreater thanObjective: To assess cognitive function and quality of life in childhood-onset CP on hormonal replacement, including GH treatment. less thanbrgreater than less thanbrgreater thanDesign: A cross-sectional study with a median follow-up time of 20 years (1-40). less thanbrgreater than less thanbrgreater thanSetting: Patients were recruited from the South Medical Region of Sweden. less thanbrgreater than less thanbrgreater thanParticipants: The study included 42 patients (20 women) surgically treated for a childhood-onset CP between 1958 and 2000. Patients were aged andgt;= 17 years. Equally many controls, matched for age, sex, residence, and smoking habits, were included. Tumor growth into the third ventricle was found in 25 patients. less thanbrgreater than less thanbrgreater thanMain Outcome Measures: All subjects were examined with a battery of cognitive tests and the following questionnaires: Symptom Checklist-90, the Interview Schedule for Social Interaction, and the Social Network concept. less thanbrgreater than less thanbrgreater thanResults: The CP patients had lower cognitive performance, reaching statistical significance in 12 of 20 test variables, including executive function and memory. Comparison of patients with tumor growth into the third ventricle to controls revealed a significant lower mean total score (P = .006). A significant negative correlation was recorded between mean z-score of cognitive performance and years since operation (r = -0.407; P = .014). No statistically significant group differences were observed across any of the 9 Symptom Checklist-90 subscales. less thanbrgreater than less thanbrgreater thanConclusions: Adults with childhood-onset CP, on hormone replacement, including GH treatment, have memory defects, disturbed attention, and impaired processing speed. Patients with hypothalamic involvement are more affected. Patients rated their quality of life as good as their matched controls.
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| 40. |
- Follin, Cecilia, et al.
(author)
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Associations between Metabolic Risk Factors and the Hypothalamic Volume in Childhood Leukemia Survivors Treated with Cranial Radiotherapy.
- 2016
-
In: PLoS ONE. - : Public Library of Science (PLoS). - 1932-6203. ; 11:1
-
Journal article (peer-reviewed)abstract
- Metabolic complications are prevalent in individuals treated with cranial radiotherapy (CRT) for childhood acute lymphoblastic leukemia (ALL). The hypothalamus is a master regulator of endocrine and metabolic control. The aim of this study was to investigate whether the hypothalamic volume would be associated to metabolic parameters in ALL survivors. Thirty-eight (21 women) survivors participated in this study 34 years after diagnosis and with a median age of 38 (27-46) years. All were treated with a median CRT dose of 24 Gy and 11 years (3-13) of complete hormone supplementation. Comparisons were made to 31 matched controls. We performed analyses of fat mass, fat free mass, plasma (p)-glucose, p-insulin, Homa-Index (a measure of insulin resistance), serum (s)-leptin, s-ghrelin and of the hypothalamic volume in scans obtained by magnetic resonance imaging (MRI) at 3 Tesla. Serum leptin/kg fat mass (r = -0.4, P = 0.04) and fat mass (r = -0.4, P = 0.01) were negatively correlated with the HT volume among ALL survivors, but not among controls. We also detected significantly higher BMI, waist, fat mass, p-insulin, Homa-Index, leptin/kg fat mass and s-ghrelin and significantly lower fat free mass specifically among female ALL survivors (all P<0.01). Interestingly, s-ghrelin levels increased with time since diagnosis and with low age at diagnosis for childhood ALL. Our results showed that leptin/kg fat mass and fat mass were associated with a reduced HT volume 34 years after ALL diagnosis and that women treated with CRT after ALL are at high risk of metabolic abnormalities. Taken together our data suggest that the hypothalamus is involved in the metabolic consequences after CRT in ALL survivors.
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