-
Neuro-oncology Jun 2017Pituitary adenomas are one of the most common primary central nervous system tumors and have an estimated prevalence of 17%. Approximately half of pituitary adenomas... (Review)
Review
Pituitary adenomas are one of the most common primary central nervous system tumors and have an estimated prevalence of 17%. Approximately half of pituitary adenomas secrete distinct pituitary hormones (most often prolactin, growth hormone, or adrenocorticotropic hormone). While these tumors are histologically benign, they have potent endocrine effects that lead to significant morbidity and shortened lifespan. Because of their pathophysiologic endocrine secretion and anatomic location near critical neural/vascular structures, hormone-secreting pituitary adenomas require defined management paradigms that can include relief of mass effect and biochemical remission. Management of hormone-secreting pituitary adenomas involves a multidisciplinary approach that can incorporate surgical, medical, and/or radiation therapies. Early and effective treatment of hormone-secreting pituitary adenomas can reduce morbidity and mortality. Consequently, understanding clinical features as well as therapeutic options in the context of the specific biological features of each type of hormone-secreting pituitary adenoma is critical for optimal management.
Topics: Adenoma; Animals; Humans; Pituitary Hormones; Pituitary Neoplasms
PubMed: 27543627
DOI: 10.1093/neuonc/now130 -
Journal of Clinical Neuroscience :... Sep 2023Pituitary adenomas are the most common sellar tumors, and may cause adverse outcomes in terms of morbidity and mortality if left untreated or if diagnosis is delayed. No...
PURPOSE
Pituitary adenomas are the most common sellar tumors, and may cause adverse outcomes in terms of morbidity and mortality if left untreated or if diagnosis is delayed. No data exists on the prevalence, characteristics, and prognosis of pituitary adenomas in Thailand. The systematic registration of clinical characteristics may improve patient care and prognosis for this disease in Thailand.
METHODS
This was a retrospective study conducted in 11 academic referral centers. All patients diagnosed with pituitary adenomas during the 2011---2014 period were enrolled in the study. The information was correlated with two national databases.
RESULTS
A total of 1,283 pituitary adenoma patients were identified. The adenomas were: non-functioning 50.1%, prolactinoma 29.4%, acromegaly 14.7%, Cushing disease 3.8%, gonadotropin-producing tumor 1.0%, TSH-secreting tumor 0.6%. The mean age was 49.2±15.2 years. Sixty-three percent of patients were female. Most common complaint was visual impairment (27.7%). The average size of the tumor was 22.2±16.1 mm. Fifty-nine percent of patients underwent surgery. Median follow-up was 27.4 months (0-24 years). After treatment, 72.4% improved, and 10.4% were cured. Overall results of treatment in non-functioning adenoma, prolactinoma (medically treated), acromegaly, Cushing, TSH, gonadotropin producing adenoma were: improved/cured in 81/5.5, 86/5.7, 55.9/30, 54.2/31.2, 85.7/14.3, 69.2/15.4% respectively.
CONCLUSION
Pituitary adenomas in academic centers in Thailand were found predominantly in female in the 4th decade of life. After treatment 72.4% of patients improved and 10% had full recovery. A health promotion system aimed to improve patient and physician recognition and physician expertise may improve the prognosis of these diseases.
Topics: Humans; Female; Adult; Middle Aged; Male; Pituitary Neoplasms; Prolactinoma; Acromegaly; Retrospective Studies; Thailand; Adenoma; Thyrotropin
PubMed: 37572520
DOI: 10.1016/j.jocn.2023.07.026 -
Frontiers in Endocrinology 2021Pituitary tumors are very complex and heterogeneous and have a very wide range of proliferative and aggressive behaviors, and how to define and classify these tumors... (Review)
Review
Pituitary tumors are very complex and heterogeneous and have a very wide range of proliferative and aggressive behaviors, and how to define and classify these tumors remains controversial. This review summarizes the epidemiology and progress in the classification and definition of pituitary tumors, as well as controversial issues. Based on the results of radiologic and autopsy studies, the prevalence of pituitary tumors has recently increased significantly. However, the majority of pituitary tumors are incidentally discovered and asymptomatic, and such tumors are called pituitary incidentalomas. Most of these incidentalomas do not induce symptoms, remain stable in size, and do not need treatment. The recent revised classification strategies mainly depend on immunohistochemistry (IHC) to detect pituitary hormones and pituitary transcription factors; therefore, the accuracy of diagnosing pituitary tumors has improved. Although new classification strategies and definitions for pituitary tumors have been presented, there are still some controversies. The term pituitary neuroendocrine tumor (PitNET) was proposed by the International Pituitary Pathology Club, and this terminology can encompass the unpredictable malignant behavior seen in the subset of aggressive pituitary adenomas (PAs). However, some endocrinologists who oppose this change in terminology have argued that the use of tumor in the terminology is misleading, as it gives PAs a harmful connotation when the majority are not aggressive. Such terminology may add new ambiguity to the origin of PAs and unnecessary anxiety and frustration for the majority of patients with benign PAs. The classification of aggressive PAs mainly relies on subjective judgment of clinical behavior and lacks objective biomarkers and unified diagnostic criteria. However, the term "refractory" could more accurately represent the characteristics of these tumors, including their clinical behaviors, radiological features, and pathologic characteristics. Moreover, the diagnostic criteria for refractory PAs are stricter, more objective, and more accurate than those for aggressive PAs. Early identification of patients with these tumors through recognition and increased awareness of the definition of refractory PAs will encourage the early use of aggressive therapeutic strategies.
Topics: Adenoma; Diagnostic Techniques, Endocrine; Humans; Neuroendocrine Tumors; Pituitary Neoplasms; Practice Guidelines as Topic
PubMed: 33815274
DOI: 10.3389/fendo.2021.604644 -
Neuroendocrinology 2022Pituitary neuroendocrine tumors (PitNETs) are the most common intracranial neoplasms. Although generally benign, they can show a clinically aggressive course, with local... (Review)
Review
Pituitary neuroendocrine tumors (PitNETs) are the most common intracranial neoplasms. Although generally benign, they can show a clinically aggressive course, with local invasion, recurrences, and resistance to medical treatment. No universally accepted biomarkers of aggressiveness are available yet, and predicting clinical behavior of PitNETs remains a challenge. In rare cases, the presence of germline mutations in specific genes predisposes to PitNET formation, as part of syndromic diseases or familial isolated pituitary adenomas, and associates to more aggressive, invasive, and drug-resistant tumors. The vast majority of cases is represented by sporadic PitNETs. Somatic mutations in the α subunit of the stimulatory G protein gene (gsp) and in the ubiquitin-specific protease 8 (USP8) gene have been recognized as pathogenetic factors in sporadic GH- and ACTH-secreting PitNETs, respectively, without an association with a worse clinical phenotype. Other molecular factors have been found to significantly affect PitNET drug responsiveness and invasive behavior. These molecules are cytoskeleton and/or scaffold proteins whose alterations prevent proper functioning of the somatostatin and dopamine receptors, targets of medical therapy, or promote the ability of tumor cells to invade surrounding tissues. The aim of the present review is to provide an overview of the genetic and molecular alterations that can contribute to determine PitNET clinical behavior. Understanding subcellular mechanisms underlying pituitary tumorigenesis and PitNET clinical phenotype will hopefully lead to identification of new potential therapeutic targets and new markers predicting the behavior and the response to therapeutic treatments of PitNETs.
Topics: Humans; Neuroendocrine Tumors; Pituitary Neoplasms
PubMed: 33524974
DOI: 10.1159/000514862 -
Neurology India 2023Primary hypophysitis is a rare condition, usually diagnosed retrospectively after surgery for a suspected pituitary adenoma. Improved awareness of the condition and... (Review)
Review
BACKGROUND
Primary hypophysitis is a rare condition, usually diagnosed retrospectively after surgery for a suspected pituitary adenoma. Improved awareness of the condition and better imaging have resulted in more patients being diagnosed without surgery.
OBJECTIVE
A retrospective chart analysis study of hypophysitis from a single secondary endocrine and neurosurgical referral center in eastern India was conducted between 1999 and 2021 to assess the diagnostic and therapeutic challenges posed by these patients.
METHODS AND MATERIAL
Fourteen patients presented to the center between 1999 and 2021. All patients had an MRI of the head with contrast and a full clinical workup. Twelve patients had headache, of whom one had progressive visual impairment. One patient had severe weakness, attributed later to hypoadrenalism and one had sixth nerve palsy.
RESULTS
Six patients had glucocorticoids as their primary treatment, four refused treatment, and one was on glucocorticoid replacement. One patient had decompressive surgery because of progressive visual loss, and two had surgery because of a presumptive diagnosis of pituitary adenoma. There was no difference between the patients who had glucocorticoids and those who did not.
CONCLUSIONS
Our data suggest that it is possible to identify most patients with hypophysitis on clinical and radiological grounds. In the largest published series on this subject and in ours, glucocorticoid treatment did not alter the outcome.
Topics: Humans; Glucocorticoids; Pituitary Neoplasms; Retrospective Studies; Abducens Nerve Diseases; Hypophysitis
PubMed: 36861584
DOI: 10.4103/0028-3886.370481 -
Annales D'endocrinologie Jul 2015Non-functioning pituitary adenoma may be totally asymptomatic and discovered "incidentally" during radiological examination for some other indication, or else induce... (Review)
Review
Non-functioning pituitary adenoma may be totally asymptomatic and discovered "incidentally" during radiological examination for some other indication, or else induce tumoral signs with compression of the optic chiasm and pituitary dysfunction. Non-functioning adenomas are mainly gonadotroph, but may also be "silent". Treatment strategy depends on initial clinical, biological, ophthalmological and radiological findings. The present French Society of Endocrinology Consensus work-group sought to update the pitfalls associated with hormone assay and outline a hormonal exploration strategy for diagnosis and follow-up, without overlooking the particularities of silent adenoma. We also drew up basic rules for initial exploration and radiological follow-up of both operated and non-operated pituitary adenomas.
Topics: Adenoma; Consensus; Gonadotropins; Humans; Pituitary Hormones; Pituitary Neoplasms; Radiography
PubMed: 26122495
DOI: 10.1016/j.ando.2015.04.005 -
The Journal of Clinical Endocrinology... Mar 2016This perspective traces a pipeline of discovery in pituitary medicine over the past 75 years. (Review)
Review
CONTEXT
This perspective traces a pipeline of discovery in pituitary medicine over the past 75 years.
OBJECTIVE
To place in context past advances and predict future changes in understanding pituitary pathophysiology and clinical care.
DESIGN
Author's perspective on reports of pituitary advances in the published literature.
SETTING
Clinical and translational Endocrinology.
OUTCOMES
Discovery of the hypothalamic-pituitary axis and mechanisms for pituitary control, have culminated in exquisite understanding of anterior pituitary cell function and dysfunction. Challenges facing the discipline include fundamental understanding of pituitary adenoma pathogenesis leading to more effective treatments of inexorably growing and debilitating hormone secreting pituitary tumors as well as medical management of non-secreting pituitary adenomas. Newly emerging pituitary syndromes include those associated with immune-targeted cancer therapies and head trauma.
CONCLUSIONS
Novel diagnostic techniques including imaging genomic, proteomic, and biochemical analyses will yield further knowledge to enable diagnosis of heretofore cryptic syndromes, as well as sub classifications of pituitary syndromes for personalized treatment approaches. Cost effective personalized approaches to precision therapy must demonstrate value, and will be empowered by multidisciplinary approaches to integrating complex subcellular information to identify therapeutic targets for enabling maximal outcomes. These goals will be challenging to attain given the rarity of pituitary disorders and the difficulty in conducting appropriately powered prospective trials.
Topics: Adrenocorticotropic Hormone; Human Growth Hormone; Humans; Pituitary Neoplasms; Precision Medicine
PubMed: 26908107
DOI: 10.1210/jc.2015-3653 -
Ulusal Travma Ve Acil Cerrahi Dergisi =... Apr 2022Pituitary apoplexy is an emergent and potential life-threatening complication of pituitary adenomas if not managed properly. The aim of our study is to present our...
BACKGROUND
Pituitary apoplexy is an emergent and potential life-threatening complication of pituitary adenomas if not managed properly. The aim of our study is to present our series of pituitary adenomas and to focus on the clinical, radiological, and surgical characteristics of this rare complication.
METHODS
In this study, a total of 143 patients with pituitary adenoma underwent surgical treatment between 2016 and 2018. All patients were operated using endoscopic endonasal transsphenoidal (EET) technique. The data of pituitary apoplexy cases were recorded. Resection rates, hormonal results, and visual outcomes of patients with pituitary apoplexy were evaluated.
RESULTS
Of the 143 patients, 8 (5.59%) were presented with the symptoms and radiological findings of pituitary apoplexy. The mean age was 26.75 years, and 4 (50%) of them were male and 4 were female. Pre-operative mean Knosp grading score was 2.1 All of eight patients underwent emergent surgical intervention and total resection was achieved in 75% of patients with apoplexy. Hormone levels were significantly decreased after surgery (p<0.05), except prolactin (p>0.05). Cerebrospinal fluid leakage occurred in one pa-tient. None of the patient with pituitary apoplexy died in our series.
CONCLUSION
Pituitary apoplexy is an important complication of pituitary adenomas. Early diagnosis and surgical intervention provide excellent ophthalmological and hormonal outcomes. Emergent EET approach is crucial for patients with ophthalmological findings and macroadenomas.
Topics: Adenoma; Adult; Endoscopy; Female; Humans; Male; Pituitary Apoplexy; Pituitary Neoplasms; Retrospective Studies
PubMed: 35485523
DOI: 10.14744/tjtes.2021.93539 -
Molecular and Cellular Endocrinology Feb 2016Pituitary neoplasias can occur as part of a complex inherited disorder, or more commonly as sporadic (non-familial) disease. Studies of the molecular and genetic... (Review)
Review
Pituitary neoplasias can occur as part of a complex inherited disorder, or more commonly as sporadic (non-familial) disease. Studies of the molecular and genetic mechanisms causing such pituitary tumours have identified dysregulation of >35 genes, with many revealed by studies in mice, rats and zebrafish. Strategies used to generate these animal models have included gene knockout, gene knockin and transgenic over-expression, as well as chemical mutagenesis and drug induction. These animal models provide an important resource for investigation of tissue-specific tumourigenic mechanisms, and evaluations of novel therapies, illustrated by studies into multiple endocrine neoplasia type 1 (MEN1), a hereditary syndrome in which ∼ 30% of patients develop pituitary adenomas. This review describes animal models of pituitary neoplasia that have been generated, together with some recent advances in gene editing technologies, and an illustration of the use of the Men1 mouse as a pre clinical model for evaluating novel therapies.
Topics: Animals; Humans; Mice; Models, Animal; Pituitary Neoplasms; Proto-Oncogene Proteins; Rats; Zebrafish
PubMed: 26320859
DOI: 10.1016/j.mce.2015.08.024 -
Frontiers in Endocrinology 2020Pituitary adenomas (PAs) can be classified as non-secreting adenomas, somatotroph adenomas, corticotroph adenomas, lactotroph adenomas, and thyrotroph adenomas.... (Review)
Review
Pituitary adenomas (PAs) can be classified as non-secreting adenomas, somatotroph adenomas, corticotroph adenomas, lactotroph adenomas, and thyrotroph adenomas. Substantial advances have been made in our knowledge of the pathobiology of PAs. To obtain a comprehensive understanding of the molecular biological characteristics of different types of PAs, we reviewed the important advances that have been made involving genetic and epigenetic variation, comprising genetic mutations, chromosome number variations, DNA methylation, microRNA regulation, and transcription factor regulation. Classical tumor predisposition syndromes include multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4) syndromes, Carney complex, and X-LAG syndromes. PAs have also been described in association with succinate dehydrogenase-related familial PA, neurofibromatosis type 1, and von Hippel-Lindau, DICER1, and Lynch syndromes. Patients with aryl hydrocarbon receptor-interacting protein () mutations often present with pituitary gigantism, either in familial or sporadic adenomas. In contrast, guanine nucleotide-binding protein G(s) subunit alpha () and G protein-coupled receptor 101 () mutations can lead to excess growth hormone. Moreover, the deubiquitinase gene , , and mutations are associated with adrenocorticotropic hormone production. In this review, we describe the genetic and epigenetic landscape of PAs and summarize novel insights into the regulation of pituitary tumorigenesis.
Topics: Adenoma; Epigenesis, Genetic; Gene Expression Regulation; Genetic Markers; Genetic Predisposition to Disease; Humans; Mutation; Pituitary Neoplasms
PubMed: 33574795
DOI: 10.3389/fendo.2020.596554