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Frontiers in Endocrinology 2023Hedgehog signaling plays pivotal roles in embryonic development, adult homeostasis and tumorigenesis. However, its engagement in the pituitary gland has been long... (Review)
Review
Hedgehog signaling plays pivotal roles in embryonic development, adult homeostasis and tumorigenesis. However, its engagement in the pituitary gland has been long underestimated although Hedgehog signaling and pituitary embryogenic development are closely linked. Thus, deregulation of this signaling pathway during pituitary development results in malformation of the gland. Research of the last years further implicates a regulatory role of Hedgehog signaling in the function of the adult pituitary, because its activity is also interlinked with homeostasis, hormone production, and most likely also formation of neoplasms of the gland. The fact that this pathway can be efficiently targeted by validated therapeutic strategies makes it a promising candidate for treating pituitary diseases. We here summarize the current knowledge about the importance of Hedgehog signaling during pituitary development and review recent data that highlight the impact of Hedgehog signaling in the healthy and the diseased adult pituitary gland.
Topics: Adult; Humans; Hedgehog Proteins; Pituitary Gland; Signal Transduction; Pituitary Neoplasms; Pituitary Diseases
PubMed: 37476499
DOI: 10.3389/fendo.2023.1219018 -
Hormones (Athens, Greece) Sep 2021Derangement of pituitary hormone axes can induce changes in bone remodeling and metabolism with possible alterations in bone microarchitectural structure and increased... (Review)
Review
Derangement of pituitary hormone axes can induce changes in bone remodeling and metabolism with possible alterations in bone microarchitectural structure and increased susceptibility to fractures. Vertebral fractures (VFs), which are a hallmark of skeletal fragility, have been described in a very large number of patients with pituitary diseases. These fractures are clinically relevant, since they predispose to further fractures and may negatively impact on patients' quality of life. However, the management of skeletal fragility and VFs in the specific setting of pituitary diseases is a challenge, since the awareness for this disease is still low, prediction of VFs is uncertain, the diagnosis of VFs cannot be solely based on a clinical approach and also needs a radiological and morphometric approach, the risk of fractures may not be decreased via treatment of pituitary hormone disorders, and the effectiveness of bone-active drugs in this setting is not always evidence-based. This review is an update on skeletal fragility in patients with pituitary diseases, with a focus on clinical and therapeutic aspects concerning the management of VFs.
Topics: Bone Density; Humans; Pituitary Diseases; Quality of Life; Spinal Fractures
PubMed: 33606197
DOI: 10.1007/s42000-021-00275-5 -
European Journal of Endocrinology Oct 2019The transition age is the period between childhood to adulthood; it refers to a broad set of physical, cognitive and sociocultural modifications, arbitrarily defined as... (Review)
Review
INTRODUCTION
The transition age is the period between childhood to adulthood; it refers to a broad set of physical, cognitive and sociocultural modifications, arbitrarily defined as starting in late puberty and ending with full adult maturation. Pituitary disorders in adolescence represent a challenge that requires careful management during the transition to adult care.
METHODS
Given the complexity of care of pituitary disorders in the transition age, we have reviewed the relevant medical literature focusing on aetiology, clinical manifestations, treatment strategies of GH deficiency (GHD), hypogonadotrophic hypogonadism (HH) in male and female adolescents, central hypothyroidism (CH), central adrenal insufficiency (CAI) and cranial diabetes insipidus (CDI) at this time. The objective of the present review is to provide an up-to-date evaluation of the transition period to evaluate the specific needs of adolescents with chronic pituitary disease in order to optimise their management.
RESULTS
We provide an overview of current clinical management of GHD, HH, CH, CAI and CDI in the transition age.
CONCLUSIONS
Specific changes occur in pituitary function during the transition period. A holistic approach including discussion of patients' concerns and emotional support should constitute a key component of managing pituitary disorders in adolescence. Special transition clinics where paediatric and adult endocrinologists work together, should be increasingly created and strengthened to bridge care, to promote continuity and adherence to treatment and to limit potential negative development, metabolic, skeletal and cardiovascular sequelae of discontinuity of care among adolescents with pituitary disorders.
Topics: Adolescent; Age Factors; Child; Human Growth Hormone; Humans; Patient Transfer; Pituitary Diseases; Sexual Maturation; Young Adult
PubMed: 31370006
DOI: 10.1530/EJE-19-0298 -
Vnitrni Lekarstvi 2022Enlargement of the pituitary gland is heterogenous in the etiology. Common causes of pituitary enlargement are physiological hypertrophy during pregnancy, primary and...
Enlargement of the pituitary gland is heterogenous in the etiology. Common causes of pituitary enlargement are physiological hypertrophy during pregnancy, primary and secondary tumors, autoimmune hypophysitis including side effects of anticancer therapy with check-point inhibitors. Terms like hypertrophy, hyperplasia, sellar expansion and hypophysitis are commonly used to describe enlargement of the pituitary gland on MR scan regardless its etiology. The most common pathology causing pituitary gland enlargement is pituitary adenoma. Magnetic resonance imaging can differentiate pituitary tumors from diffuse enlargement due to hypophysitis in most but not all cases. Changes on imaging during time or response to pharmacotherapy might help determine the final diagnosis in uncertain cases. We present a case report of a young woman with sellar expansion due to prolonged untreated peripheral hypothyroidism mimicking pituitary adenoma. Interdisciplinary cooperation of endocrinologist, radiologist and neurosurgeon is crucial in determining the diagnosis.
Topics: Adenoma; Diagnosis, Differential; Female; Humans; Hypertrophy; Hypophysitis; Hypothyroidism; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Pituitary Neoplasms; Pregnancy
PubMed: 35459348
DOI: No ID Found -
Veterinary Journal (London, England :... 2023Equine pituitary pars intermedia dysfunction (PPID) is common in aged horses. The majority of horses respond well to treatment, but treatment is lifelong, meaning... (Review)
Review
Equine pituitary pars intermedia dysfunction (PPID) is common in aged horses. The majority of horses respond well to treatment, but treatment is lifelong, meaning accurate diagnosis of PPID is important. Similar to any condition, there is no perfect laboratory test to diagnose PPID and accuracy is affected by the characteristics of the population in which the test is being evaluated. This review details the importance of consideration of clinical factors and diagnostic test accuracy. Basal adrenocorticotrophic hormone (ACTH) concentration is used most frequently in practice and has very good diagnostic accuracy when used in combination with clinical judgement and the correct application of diagnostic thresholds. The thyrotropin-releasing hormone stimulation test can be used in horses with equivocal test results following basal ACTH testing, or to evaluate subtle cases due to its improved accuracy.
Topics: Horses; Animals; Thyrotropin-Releasing Hormone; Pituitary Gland, Intermediate; Horse Diseases; Pituitary Diseases; Adrenocorticotropic Hormone
PubMed: 37805159
DOI: 10.1016/j.tvjl.2023.106036 -
World Journal of Gastroenterology Nov 2020Pituitary stalk interruption syndrome (PSIS) is a rare congenital abnormality characterized by thinning or disappearance of the pituitary stalk, hypoplasia of the... (Review)
Review
Pituitary stalk interruption syndrome (PSIS) is a rare congenital abnormality characterized by thinning or disappearance of the pituitary stalk, hypoplasia of the anterior pituitary and an ectopic posterior pituitary. Although the etiology of PSIS is still unclear, gene changes and perinatal adverse events such as breech delivery may play important roles in the pathogenesis of PSIS. PSIS can cause multiple hormone deficiencies, such as growth hormone, which then cause a series of changes in the human body. On the one hand, hormone changes affect growth and development, and on the other hand, they could affect human metabolism and subsequently the liver resulting in nonalcoholic fatty liver disease (NAFLD). Under the synergistic effect of multiple mechanisms, the progression of NAFLD caused by PSIS is faster than that due to other causes. Therefore, in addition to early identification of PSIS, timely hormone replacement therapy and monitoring of relevant hormone levels, clinicians should routinely assess the liver function while managing PSIS.
Topics: Female; Growth Hormone; Human Growth Hormone; Humans; Hypopituitarism; Liver; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Pregnancy
PubMed: 33311939
DOI: 10.3748/wjg.v26.i44.6909 -
Frontiers in Endocrinology 2023Current guidelines recommend temozolomide as the first-line chemotherapy for aggressive pituitary neuroendocrine tumours. However, no clinical trials have been conducted...
Current guidelines recommend temozolomide as the first-line chemotherapy for aggressive pituitary neuroendocrine tumours. However, no clinical trials have been conducted to date and clinical experience is quite limited. We retrospectively analyzed 28 patients (9 women and 19 men), aged 46.6 + 16.9, with aggressive pituitary tumours (4 pituitary carcinomas and 24 aggressive adenomas) treated with temozolomide in 10 Spanish pituitary reference centres. Four patients had Cushing's disease, 9 prolactinomas and 15 clinically non-functioning pituitary tumours (seven silent corticotroph, three silent somatotroph, one silent lactotroph, one silent gondotroph and three null-cell tumours). Median size at diagnosis was 10.5 cm3 (IQR 4.7-22.5), with cavernous sinus invasion in 88% and no metastases. Pre-temozolomide treatment, these data were 5.2 cm3 (IQR 1.9-12.3), 89.3% and 14.3% (2 intracranial and 2 spinal metastases). All patients had undergone surgery (1-5 surgeries), 25 (89.3%) had received radiotherapy (7 of them reirradiated) and 13(46.4%) had received cabergoline. One patient interrupted temozolomide prematurely. The remaining 27 patients received a median of 13 cycles (range 3-66) of 5 days every 28 days, with a mean initial dose of 265 ± 73 mg when administered alone and of 133 ± 15 mg when co-administered with radiotherapy. Eight patients (29.6%) had a significant reduction (>30%) in tumour volume and 14 (51.9%) attained tumour stabilization. After a median follow-up of 29 months (IQR 10-55), 8 out of these 22 showed disease progression. A longer progression-free survival was found in the five patients who received concomitant radiotherapy. Seven patients (25%) died (all of them because of tumour progression or complications of treatments) at 77 months (IQR 42-136) after diagnosis and 29 months (IQR 16-55) after the first dose of temozolomide. Adverse effects occurred in 18 patients (14 mild and 4 moderate or severe). In conclusion, temozolomide is an effective medical treatment for aggressive pitNET and pituitary carcinomas but is sometimes followed by tumour progression. Co-administration with radiotherapy may increase progression-free survival.
Topics: Male; Humans; Female; Pituitary Neoplasms; Temozolomide; Neuroendocrine Tumors; Spain; Retrospective Studies; Pituitary Diseases
PubMed: 37720528
DOI: 10.3389/fendo.2023.1204206 -
Endocrine-related Cancer May 2022Once temozolomide has failed, there is no recommended treatment option for pituitary carcinomas and aggressive pituitary tumors. Immune-checkpoint inhibitors (ICIs)...
Once temozolomide has failed, there is no recommended treatment option for pituitary carcinomas and aggressive pituitary tumors. Immune-checkpoint inhibitors (ICIs) represent the most recent therapeutic avenue, having raised hope with the publication of the first successful case in 2018. Here, we present an overview of immunotherapy in pituitary carcinomas and aggressive pituitary tumors, starting with the rationale for using ICIs and the implications of tumor-infiltrating lymphocytes in anterior pituitary tumors, followed by a systematic review of all published cases, analyzing both treatment response and potential predictors of response and finishing with research and clinical perspectives. Seven corticotroph and four lactotroph tumors have been so far treated with ICIs. Corticotroph tumors showed radiological partial response in 57% of cases, followed by stable disease in 29% of cases, which was accompanied by biochemical partial or complete response in 83% of cases. Half of lactotroph tumors showed radiological complete or partial response, accompanied by biochemical complete response in 33% of the cases. In the case of a dissociate response, continuation of immunotherapy combined with local treatment represents a good option. At this time, a high tumor mutational burden appears to be the most promising predictive marker of response. MMR deficiency does not guarantee a response. Negative PD-L1 staining should not preclude ICIs administration. Therefore, ICIs are a promising option after temozolomide failure. This review highlights key clinical aspects that can already be implemented into practice and also discusses tumor biology concepts and perspectives expected to improve immunotherapy outcomes.
Topics: Carcinoma; Humans; Immune Checkpoint Inhibitors; Immunotherapy; Pituitary Neoplasms; Temozolomide
PubMed: 35521777
DOI: 10.1530/ERC-22-0037 -
Pituitary Dec 2018Pituitary tumors may have a considerable impact on patients' functional status, including paid employment, yet research in this area is sparse.
INTRODUCTION
Pituitary tumors may have a considerable impact on patients' functional status, including paid employment, yet research in this area is sparse.
OBJECTIVE
To describe work disability and its determinants in patients treated for a pituitary tumor.
METHODS
Cross-sectional study including patients treated for a pituitary tumor in the working age (18-65 years), who completed five validated questionnaires assessing work disability [Short Form-Health and Labour Questionnaire, Work Role Functioning Questionnaire 2.0 (WRFQ)], health-related quality of life (HRQoL) and utility (Short Form-36, EuroQoL) and disease burden (Leiden Bother and Needs Questionnaire-Pituitary). Additional data were extracted from the medical records (age, gender, tumor type, treatment, date of diagnosis) and self-reports (marital status, education, endocrine status). Associations of disease-specific and sociodemographic characteristics, HRQoL, and disease burden with (not) having a paid job were examined through multivariate logistic regression.
RESULTS
We included 241 patients (61% female, median age 53 years, median time since diagnosis 11 years), of whom 68 (28%) were without a paid job. Patients who had acromegaly, Cushing's disease, (pan)hypopituitarism, radiotherapy, were single, less educated, lower HRQoL, and increased disease burden were more often without a paid job (p < 0.05). Among those with paid jobs, 41% reported health-related absenteeism in the previous year. The three work incapacitating problems reported by the largest proportion of patients were within the mental and social domain (WRFQ).
CONCLUSION
Work disability among patients treated for a pituitary tumor is substantial. As impact on social functioning is high, it is strongly advised to incorporate work disability during clinical guidance of patients.
Topics: Absenteeism; Adolescent; Adult; Aged; Chi-Square Distribution; Cross-Sectional Studies; Female; Health Status; Humans; Male; Middle Aged; Pituitary Diseases; Pituitary Neoplasms; Quality of Life; Socioeconomic Factors; Surveys and Questionnaires; Young Adult
PubMed: 30288666
DOI: 10.1007/s11102-018-0913-3 -
Internal Medicine (Tokyo, Japan) Feb 2018We herein report the case of a 25-year-old woman who presented with severe headache and visual field defects after childbirth. Magnetic resonance imaging revealed marked...
We herein report the case of a 25-year-old woman who presented with severe headache and visual field defects after childbirth. Magnetic resonance imaging revealed marked swelling of the pituitary gland, and an endocrinological examination revealed panhypopituitarism and diabetes insipidus. An immunohistological analysis of a transsphenoidal biopsy sample of the pituitary gland showed the significant accumulation of an immunogloblin G4 (IgG4)-positive population, leading to the diagnosis of IgG4-related hypophysitis. The patient was treated with prednisolone, which markedly reduced the swelling of the pituitary gland, in association with recovery of the pituitary function. This is a rare case of biopsy-proven IgG4-related hypophysitis with a postpartum onset.
Topics: Adult; Autoimmune Hypophysitis; Female; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Postpartum Period; Prednisolone; Treatment Outcome
PubMed: 29093382
DOI: 10.2169/internalmedicine.8446-16