| 61. |
- Raverot, Gerald, et al.
(författare)
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European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas
- 2018
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Ingår i: European Journal of Endocrinology. - 1479-683X. ; 178:1, s. 1-24
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Pituitary tumours are common and easily treated by surgery or medical treatment in most cases. However, a small subset of pituitary tumours does not respond to standard medical treatment and presents with multiple local recurrences (aggressive pituitary tumours) and in rare occasion with metastases (pituitary carcinoma). The present European Society of Endocrinology (ESE) guideline aims to provide clinical guidance on diagnosis, treatment and follow-up in aggressive pituitary tumours and carcinomas.METHODS: We decided upfront, while acknowledging that literature on aggressive pituitary tumours and carcinomas is scarce, to systematically review the literature according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. The review focused primarily on first- and second-line treatment in aggressive pituitary tumours and carcinomas. We included 14 single-arm cohort studies (total number of patients = 116) most on temozolomide treatment (n = 11 studies, total number of patients = 106). A positive treatment effect was seen in 47% (95% CI: 36-58%) of temozolomide treated. Data from the recently performed ESE survey on aggressive pituitary tumours and carcinomas (165 patients) were also used as backbone for the guideline. SELECTED RECOMMENDATION: (i) Patients with aggressive pituitary tumours should be managed by a multidisciplinary expert team. (ii) Histopathological analyses including pituitary hormones and proliferative markers are needed for correct tumour classification. (iii) Temozolomide monotherapy is the first-line chemotherapy for aggressive pituitary tumours and pituitary carcinomas after failure of standard therapies; treatment evaluation after 3 cycles allows identification of responder and non-responder patients. (iv) In patients responding to first-line temozolomide, we suggest continuing treatment for at least 6 months in total. Furthermore, the guideline offers recommendations for patients who recurred after temozolomide treatment, for those who did not respond to temozolomide and for patients with systemic metastasis.
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| 62. |
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| 63. |
- Rosén, Thord, et al.
(författare)
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Traumatisk hjärnskada kan ge hypofyssvikt
- 2012
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Ingår i: Läkartidningen. - : Läkartidningen Förlag. - 0023-7205 .- 1652-7518. ; 109:12, s. 629-632
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Tidskriftsartikel (refereegranskat)abstract
- Traumatisk hjärnskada (traumatic brain injury, TBI) är en av de vanligaste orsakerna till död och nedsatt funktion hos människor i arbetsför ålder.Patienter som överlever svåra skallskador utgör en växande patientkategori inom dagens sjukvård.På senare år har det aktualiserats att allvarligt skalltrauma kan ge skador i hypofys–hypotalamusregionen.Det är angeläget att dessa patienter utreds för eventuell hypofyssvikt så att hormonell substitutionsterapi kan inledas om endokrin insufficiens fastställs. Detta kan förhoppningsvis underlätta efterföljande rehabilitering och därmed förbättra prognosen.Vi presenterar här ett förslag till riktlinjer för utvärdering av hypofysfunktionen hos TBI-patienter. Samtidigt vill vi informera en bredare läsekrets om TBI-begreppet.
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| 64. |
- Rosén, Thord, et al.
(författare)
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Traumatisk hjärnskada kan ge hypofyssvikt : Förslag till riktlinjer för utvärdering av hypofysfunktionen
- 2012
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Ingår i: Läkartidningen. - : Läkartidningen förlag AB. - 0023-7205 .- 1652-7518. ; 109:12, s. 629-632
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Traumatisk hjärnskada (traumatic brain injury, TBI) är en av de vanligaste orsakerna till död och nedsatt funktion hos människor i arbetsför ålder. Patienter som överlever svåra skallskador utgör en växande patientkategori inom dagens sjukvård. På senare år har det aktualiserats att allvarligt skalltrauma kan ge skador i hypofys–hypotalamusregionen. Det är angeläget att dessa patienter utreds för eventuell hypofyssvikt så att hormonell substitutionsterapi kan inledas om endokrin insufficiens fastställs. Detta kan förhoppningsvis underlätta efterföljande rehabilitering och därmed förbättra prognosen. Vi presenterar här ett förslag till riktlinjer för utvärdering av hypofysfunktionen hos TBI-patienter. Samtidigt vill vi informera en bredare läsekrets om TBI-begreppet.
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| 65. |
- Sathiavageeswaran, Mahesh, et al.
(författare)
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Effects of GH on cognitive function in elderly patients with adult-onset GH deficiency: a placebo-controlled 12-month study
- 2007
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Ingår i: European Journal of Endocrinology. - : Oxford University Press (OUP). - 1479-683X .- 0804-4643. ; 156:4, s. 439-447
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Young adults with childhood-onset GH deficiency (GHD) have reduced memory and attention, which can be improved by treatment with GH. Little information is available on cognitive function in elderly GHD patients. Design: Single center, double-blind, randomized, placebo-controlled study of 52-week duration. Methods: Elderly GH therapy naive GHD patients (n = 34; age range 60-77 years) were enrolled and randomized to receive placebo or GH therapy which was titrated to achieve a target IGF-I level of + 1 to + 2 S.D. of the normal mean for age. Cognitive function was assessed at baseline and after 24 and 52 weeks, using a computerized psychometric test package (Neurobehavioral Examination System-2). Results: The mean GH dose was 0.16 +/- 0.06 mg/day; mean IGF-I increased from 135 +/- 59 ng/ml at baseline to 213 + 77 ng/ml during active treatment. The GH-treated group had better mean serial digit learning scores compared with placebo group (P < 0.05). Assessment of effect sizes showed that improvements in memory occurred with GH after 24 weeks. The overall adverse event rates were similar in the GH and the placebo group. Conclusion: This study indicates that GH replacement may be accompanied by improvement in certain measures of cognitive function in elderly patients with GHD.
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| 66. |
- Schill, Fredrika, et al.
(författare)
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Pituitary Metastases : A Nationwide Study on Current Characteristics With Special Reference to Breast Cancer
- 2019
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Ingår i: Journal of Clinical Endocrinology and Metabolism. - : Oxford University Press. - 0021-972X .- 1945-7197. ; 104:8, s. 3379-3388
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To investigate the contemporary presentation of pituitary metastases. Patients: Thirty-eight patients diagnosed with pituitary metastases from 1996 to 2018 in Sweden.Methods: Pituitary metastases were confirmed by histopathology (n = 27) or considered highly likely according to radiological findings, including rapid tumor progression (n = 11). Medical records were reviewed and cellar images reexamined centrally.Results: Breast and lung cancers were the most common primary tumors, in 45% and 21% of patients, respectively. Sixty-seven percent of breast cancers overexpressed human epidermal growth factor receptor 2 (HER2); 53% of pituitary metastases from breast cancers appeared >= 10 years after diagnosis of the primary tumor. At presentation, 71% appeared to have ACTH deficiency, 65% had TSH deficiency, and 26% had diabetes insipidus. Fatigue, nausea/vomiting, loss of appetite, weight loss, myalgia, and/or arthralgia were reported in 47% of patients with morning cortisol <100 nmol/L vs 23% with cortisol >= 200 nmol/L. Sixteen patients had visual field defects, and eight had diplopia. Intrasellar and suprasellar tumor growth was the most frequent finding. Initially, a pituitary adenoma was considered the etiology in 18% of patients. Radiotherapy, pituitary surgery, and chemotherapy were used in 68%, 68%, and 11% of patients, respectively. One and 2 years after diagnosis of pituitary metastases, 50% and 26% of patients were alive.Conclusion: Pituitary metastases may be mistaken for pituitary adenomas and can appear late, especially in breast cancer. Breast cancers overexpressing HER2 seem prone to metastasize to the pituitary. Hypocortisolism may be misdiagnosed as cancer-related malaise. An increased awareness of pituitary metastases and undiagnosed pituitary failure can improve management in these patients.
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| 67. |
- Sharrack, Basil, et al.
(författare)
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Autologous haematopoietic stem cell transplantation and other cellular therapy in multiple sclerosis and immune-mediated neurological diseases : updated guidelines and recommendations from the EBMT Autoimmune Diseases Working Party (ADWP) and the Joint Accreditation Committee of EBMT and ISCT (JACIE)
- 2020
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Ingår i: Bone Marrow Transplantation. - : Springer Nature. - 0268-3369 .- 1476-5365. ; 55:2, s. 283-306
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Tidskriftsartikel (refereegranskat)abstract
- These updated EBMT guidelines review the clinical evidence, registry activity and mechanisms of action of haematopoietic stem cell transplantation (HSCT) in multiple sclerosis (MS) and other immune-mediated neurological diseases and provide recommendations for patient selection, transplant technique, follow-up and future development. The major focus is on autologous HSCT (aHSCT), used in MS for over two decades and currently the fastest growing indication for this treatment in Europe, with increasing evidence to support its use in highly active relapsing remitting MS failing to respond to disease modifying therapies. aHSCT may have a potential role in the treatment of the progressive forms of MS with a significant inflammatory component and other immune-mediated neurological diseases, including chronic inflammatory demyelinating polyneuropathy, neuromyelitis optica, myasthenia gravis and stiff person syndrome. Allogeneic HSCT should only be considered where potential risks are justified. Compared with other immunomodulatory treatments, HSCT is associated with greater short-term risks and requires close interspeciality collaboration between transplant physicians and neurologists with a special interest in these neurological conditions before, during and after treatment in accredited HSCT centres. Other experimental cell therapies are developmental for these diseases and patients should only be treated on clinical trials.
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| 68. |
- Simon, Julia, et al.
(författare)
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Prevalence and clinical correlations of SF3B1 variants in lactotroph tumours
- 2023
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Ingår i: European Journal of Endocrinology. - 1479-683X. ; 189:3, s. 372-378
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: A somatic mutational hotspot in the SF3B1 gene was reported in lactotroph tumours. The aim of our study was to examine the prevalence of driver SF3B1 variants in a multicentre independent cohort of patients with lactotroph tumours and correlate with clinical data. DESIGN AND METHODS: This was a retrospective, multicentre study involving 282 patients with lactotroph tumours (including 6 metastatic lactotroph tumours) from 8 European centres. We screened SF3B1 exon 14 hotspot for somatic variants using Sanger sequencing and correlated with clinicopathological data. RESULTS: We detected SF3B1 variants in seven patients with lactotroph tumours: c.1874G > A (p.Arg625His) (n = 4, 3 of which metastatic) and a previously undescribed in pituitary tumours variant c.1873C > T (p.Arg625Cys) (n = 3 aggressive pituitary tumours). In two metastatic lactotroph tumours with tissue available, the variant was detected in both primary tumour and metastasis. The overall prevalence of likely pathogenic SF3B1 variants in lactotroph tumours was 2.5%, but when we considered only metastatic cases, it reached the 50%. SF3B1 variants correlated with significantly larger tumour size; higher Ki67 proliferation index; multiple treatments, including radiotherapy and chemotherapy; increased disease-specific death; and shorter postoperative survival. CONCLUSIONS: SF3B1 variants are uncommon in lactotroph tumours but may be frequent in metastatic lactotroph tumours. When present, they associate with aggressive tumour behaviour and worse clinical outcome.
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| 69. |
- Touraine, Philippe, et al.
(författare)
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Pituitary function and the response to GH therapy in patients with Langerhans cell histiocytosis : analysis of the KIMS database
- 2022
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Ingår i: Yosetsu Gakkai Shi/Journal of the Japan Welding Society. - 0021-4787. ; 187:3, s. 373-381
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To analyze the effectiveness and safety of growth hormone (GH) replacement treatment in adult patients with Langerhans cell histiocytosis (LCH) and GH deficiency (GHD) enrolled in KIMS (Pfizer International Metabolic Database). Patients and methods: Patients with LCH and GHD were studied at baseline and some of them after 1 year of GH treatment. The effectiveness of GH is presented as change after 1 year of treatment (mean, 95% CI). The LCH population was compared to two other groups of patients enrolled in KIMS, granulomatous and lymphocytic hypophysitis. Results: At baseline, 81 adults with LCH (27 with childhood onset, 56% females), mean age at GHD onset of 29 (15) years were studied. Diabetes insipidus was diagnosed in 86% of patients. Analysis of 1 year of GH treatment was possible in 37 patients. One-year cross-sectional values for the GH dose were 0.39 (S.D. ± 0.21) mg and -0.5 (-1.2 to 0.2) for insulin-like growth factor-1 S.D. Total cholesterol decreased 0.9 (-1.5 to -0.3 (mmol/L); P < 0.05); AGHDA-QoLscore (n = 20) was improved by 2.8 points (-5.6 to 0.0; P < 0.05), while mean BMI increased 0.6 ± 3 kg/m2 (95% CI: -0.2 to 1.4). All these effects did not differ from the two other groups after adjusting for age, gender, and baseline values. In 20 of 77 patients included in the safety analysis, 36 serious adverse events were reported during 435 patient-years (82.8/1000); no new safety signals were reported. Conclusion: After 1 year of GH treatment in patients with LCH, metabolic variables and quality of life improved, with no new safety signals.
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| 70. |
- Trouillas, Jacqueline, et al.
(författare)
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Aggressive pituitary tumours and carcinomas : two sides of the same coin?
- 2018
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Ingår i: European Journal of Endocrinology. - 1479-683X. ; 178:6
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Forskningsöversikt (refereegranskat)abstract
- The European Society of Endocrinology (ESE) survey reported on the largest cohort of 125 aggressive pituitary tumours (APT) and 40 pituitary carcinomas (PC). Whilst the survey focused on treatment effectiveness, all pathological data were not explored in detail. Here, we comment on some interesting pathological findings, notably the difference between APT and PC.
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