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Handbook of Clinical Neurology 2021Neuroendocrine manifestations are common in Erdheim-Chester disease (ECD) patients. ECD is a rare non-Langerhans form of histiocytosis with multisystemic infiltration.... (Review)
Review
Neuroendocrine manifestations are common in Erdheim-Chester disease (ECD) patients. ECD is a rare non-Langerhans form of histiocytosis with multisystemic infiltration. The involvement of the hypothalamo-pituitary axis is common and central diabetes insipidus (CDI) is one of the most common endocrine manifestations in ECD patients. CDI is the first manifestation of ECD in 25%-48% of the cases. Suprasellar region extension, due to the infiltration of ECD lesions, can cause neurologic manifestations by mass effects, such as headache, visual disturbance, and cranial nerve palsies. Recent studies have revealed that disorders affecting anterior pituitary hormones are common in ECD patients. Secondary adrenal insufficiency, secondary hypothyroidism, (adult) growth hormone deficiency, hypogonadotropic hypogonadism, hyperprolactinemia, and hypoprolactinemia can develop as the neuroendocrine manifestations of ECD. Since the symptoms of anterior pituitary hormone deficiencies tend to be nonspecific, the diagnosis of anterior pituitary hormone dysfunctions can be delayed. Some anterior pituitary dysfunctions such as adrenocorticotropic hormone and/or thyroid-stimulating hormone deficiencies can be life-threatening without adequate hormone supplementation therapies. An endocrinological evaluation of the function of the pituitary gland should be performed at the initial diagnosis of ECD. It is important to recognize that endocrine dysfunctions can develop later during the follow-up of ECD.
Topics: Adult; Erdheim-Chester Disease; Humans; Hypopituitarism; Hypothyroidism; Pituitary Diseases; Pituitary Gland
PubMed: 34238453
DOI: 10.1016/B978-0-12-820683-6.00010-5 -
Neuroendocrinology 2020Hypophysitis is characterized by inflammation of the pituitary gland that can be primary (PH) or secondary (SH) to other diseases or following drug administration. It... (Review)
Review
Hypophysitis is characterized by inflammation of the pituitary gland that can be primary (PH) or secondary (SH) to other diseases or following drug administration. It may also be classified according to anatomical and histopathological criteria, leading to variable degrees of hypopituitarism and/or compressive symptoms to nearby structures. There has recently been an increase in the number of hypophysitis cases, raising the interest on the spectrum of its pathogenesis, clinical, biochemical/endocrinological, and imaging features. However, the use of conventional biomarkers, including currently utilized pituitary autoantibodies, has relatively limited diagnostic accuracy. Lymphocytic hypophysitis (LH) is the commonest cause of PH, whereas IgG4-related hypophysitis is increasingly being recognized. Histiocytosis and granulomatous diseases are the most frequent causes of SH, although infections and lymphoma have also been reported. The increasing use of immune checkpoint inhibitors in oncology is associated with a high incidence of hypophysitis, providing further understanding of its pathogenesis. Hypophysitis can occur silently and be easily missed, potentially leading to substantial morbidity or mortality due to adrenal insufficiency, requiring a high index of clinical suspicion and timely initiation of appropriate treatment. In most cases of LH or drug-induced hypophysitis, active surveillance along with replacement of established hormonal deficiencies is needed. In the presence of compressive and/or evolving symptoms, treatment with glucocorticoids either alone or in combination with other immunosuppressive agents can be used. Surgical decompression is reserved for nonresponsive cases with threatened vital structures. Timely diagnosis and intervention are important to minimize disease-related morbidity and mortality. We aimed to review current concepts and recent developments in the pathogenesis, diagnosis, and management of hypophysitis.
Topics: Humans; Hypophysitis; Immunoglobulin G; Immunologic Factors
PubMed: 32126548
DOI: 10.1159/000506903 -
Journal of Neuroendocrinology Mar 2021Diabetes insipidus (DI) is a well-recognised transient or permanent complication following transsphenoidal surgery for pituitary adenomas or other sellar/parasellar... (Review)
Review
Diabetes insipidus (DI) is a well-recognised transient or permanent complication following transsphenoidal surgery for pituitary adenomas or other sellar/parasellar lesions. However, data regarding the prevalence of pre-operative DI in sellar/parasellar lesions other than pituitary adenomas are scarce. We systematically reviewed the existing data for defining the prevalence of DI before any treatment in adult patients with sellar/parasellar lesions, excluding pituitary adenomas and metastatic lesions. In total, 646 patients with sellar/parasellar lesions presenting with DI at diagnosis were identified. The most common pathologies of sellar/parasellar lesions presenting with DI at diagnosis were lymphocytic hypophysitis (26.5%), craniopharyngiomas (23.4%), Langerhans's cell histiocytosis (18.9%) and Rathke's cleft cyst (12.7%), accounting for the vast majority (more than 80%) of these lesions. Overall, DI at diagnosis was found in 23.4% of all patients with sellar/parasellar lesions, albeit with a wide range from 10.6% to 76.7%, depending on the nature of the pathology. The highest prevalence of DI was found in less commonly encountered lesions namely germ-cell tumours (76.7%), abscesses (55.4%) and neurosarcoidosis (54.5%), each accounting for less than 3% of all sellar/parasellar lesions. Most DI cases (68.8%) were associated with anterior pituitary hormonal deficiencies, in contrast to pituitary adenomas that rarely present with DI. The enlargement and enhancement of the pituitary stalk were the most common findings on magnetic resonance imaging besides the loss of the high signal of the posterior pituitary on T1-weighted images. Resolution of DI spontaneously or following systemic and surgical management occurred in 22.4% of cases. Post-operative DI, not evident before surgery, was found in 27.8% of non-adenomatous sellar/parasellar lesions, and was transient in 11.6% of them. Besides distinctive imaging features and symptoms, early recognition of DI in such lesions is important because it directs the diagnosis towards a non-adenomatous sellar/parasellar tumour and the early initiation of appropriate treatment.
Topics: Adenoma; Diabetes Insipidus, Neurogenic; Humans; Pituitary Diseases; Pituitary Neoplasms; Postoperative Complications; Prevalence; Sella Turcica
PubMed: 33769630
DOI: 10.1111/jne.12954 -
Pituitary Aug 2020Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the viral strain that has caused the coronavirus disease 2019 (COVID-19) pandemic, has presented healthcare... (Review)
Review
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the viral strain that has caused the coronavirus disease 2019 (COVID-19) pandemic, has presented healthcare systems around the world with an unprecedented challenge. In locations with significant rates of viral transmission, social distancing measures and enforced 'lockdowns' are the new 'norm' as governments try to prevent healthcare services from being overwhelmed. However, with these measures have come important challenges for the delivery of existing services for other diseases and conditions. The clinical care of patients with pituitary disorders typically involves a multidisciplinary team, working in concert to deliver timely, often complex, disease investigation and management, including pituitary surgery. COVID-19 has brought about major disruption to such services, limiting access to care and opportunities for testing (both laboratory and radiological), and dramatically reducing the ability to safely undertake transsphenoidal surgery. In the absence of clinical trials to guide management of patients with pituitary disease during the COVID-19 pandemic, herein the Professional Education Committee of the Pituitary Society proposes guidance for continued safe management and care of this population.
Topics: Betacoronavirus; COVID-19; Coronavirus Infections; Delivery of Health Care, Integrated; Health Services Accessibility; Health Status; Host-Pathogen Interactions; Humans; Pandemics; Patient Care Team; Pituitary Diseases; Pneumonia, Viral; Prognosis; Risk Factors; SARS-CoV-2
PubMed: 32556793
DOI: 10.1007/s11102-020-01059-7 -
La Radiologia Medica Mar 2020Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis... (Review)
Review
Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.
Topics: Adenoma; Autoimmune Hypophysitis; Contrast Media; Diagnosis, Differential; Gadolinium; Humans; Hypophysitis; Immunoglobulin G4-Related Disease; Magnetic Resonance Imaging; Neuroradiography; Pituitary Gland; Pituitary Neoplasms; Xanthomatosis
PubMed: 31863360
DOI: 10.1007/s11547-019-01120-x -
Experimental and Clinical Endocrinology... Mar 2021Cushing's disease is a rare condition of cortisol excess attributed to a pituitary adenoma with an annual incidence of 1.2-2.4 cases per million population. It is... (Review)
Review
Cushing's disease is a rare condition of cortisol excess attributed to a pituitary adenoma with an annual incidence of 1.2-2.4 cases per million population. It is associated with several co-morbidities leading to increased mortality predominantly due to cardiovascular disease. Despite the advances in its diagnosis and management, survival can be compromised even after apparent successful treatment. Minimizing the duration and extent of exposure to hypercortisolaemia by early diagnosis and rapid, effective therapeutic interventions, as well as close monitoring and aggressive control of cardiovascular risk factors are vital for improving outcomes of the patients.
Topics: Cardiovascular Diseases; Humans; Pituitary ACTH Hypersecretion
PubMed: 32645721
DOI: 10.1055/a-1197-6380 -
Annales D'endocrinologie Apr 2023Hypopituitarism is defined as a lack or decreased secretion of one or several pituitary hormones. It can result from diseases of the pituitary gland or from pathologies... (Review)
Review
Hypopituitarism is defined as a lack or decreased secretion of one or several pituitary hormones. It can result from diseases of the pituitary gland or from pathologies of the superior regulatory center, i.e. the hypothalamus, thereby decreasing hypothalamic releasing hormones and consequently the pituitary hormones. It is still a rare disease with an estimated prevalence of 30-45 patients/100,000 and an incidence of 4-5/100,000/year. This review summarizes the currently available data with a focus on etiologies of hypopituitarism, evidence on mortality rates in patients with hypopituitarism, temporal trends in mortality , and associated diseases, pathophysiological mechanisms and risk factors that affect mortality risk in these patients.
Topics: Humans; Hypopituitarism; Pituitary Gland; Hypothalamus; Pituitary Hormones; Risk Factors
PubMed: 36809815
DOI: 10.1016/j.ando.2023.02.002 -
Handbook of Clinical Neurology 2021The diagnosis and management of mass lesions in the sellar and parasellar areas remain challenging. When approaching patients with possible sellar or hypothalamic... (Review)
Review
The diagnosis and management of mass lesions in the sellar and parasellar areas remain challenging. When approaching patients with possible sellar or hypothalamic masses, it is important not only to focus on imaging but also detect possible pituitary hormone deficits or excess, in order to establish an appropriate diagnosis and initiate treatment. The imaging modalities used to characterize hypothalamic and pituitary lesions have significantly evolved over the course of the past several years. Computed tomography (CT) and CT angiography play a major role in detecting various sellar lesions, especially in patients who have contraindications to magnetic resonance imaging (MRI) and can also yield important information for surgical planning. However, MRI has become the gold standard for the detection and characterization of hypothalamic and pituitary tumors, infections, cystic, or vascular lesions. Indeed, the imaging characteristics of hypothalamic and sellar lesions can help narrow down the differential diagnosis preoperatively. In addition, MRI can help establish the relationship of mass lesions to surrounding structures. A pituitary MRI examination should be obtained if there is concern for mass effect (including visual loss, ophthalmoplegia, headache) or if there is clinical suspicion and laboratory evidence of either hypopituitarism or pituitary hormone excess. The information obtained from MRI images also provides us with assistance in planning surgery. Using intraoperative MRI can be very helpful in assessing the adequacy of tumor resection. In addition, MRI images yield reliable data that allow for noninvasive monitoring of patients postoperatively.
Topics: Adenoma; Craniopharyngioma; Humans; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Pituitary Neoplasms
PubMed: 34225987
DOI: 10.1016/B978-0-12-819975-6.00004-2 -
Japanese Journal of Radiology Sep 2023Hypophysitis is an inflammatory disease affecting the pituitary gland. Hypophysitis can be classified into multiple types depending on the mechanisms (primary or... (Review)
Review
Hypophysitis is an inflammatory disease affecting the pituitary gland. Hypophysitis can be classified into multiple types depending on the mechanisms (primary or secondary), histology (lymphocytic, granulomatous, xanthomatous, plasmacytic/IgG4 related, necrotizing, or mixed), and anatomy (adenohypophysitis, infundibulo-neurohypophysitis, or panhypophysitis). An appropriate diagnosis is vital for managing these potentially life-threatening conditions. However, physiological morphological alterations, remnants, and neoplastic and non-neoplastic lesions may masquerade as hypophysitis, both clinically and radiologically. Neuroimaging, as well as imaging findings of other sites of the body, plays a pivotal role in diagnosis. In this article, we will review the types of hypophysitis and summarize clinical and imaging features of both hypophysitis and its mimickers.
Topics: Humans; Pituitary Diseases; Pituitary Gland; Hypophysitis; Neuroimaging; Diagnosis, Differential
PubMed: 37010787
DOI: 10.1007/s11604-023-01417-y -
Current Urology Reports Nov 2019Hypogonadism is a common endocrine dysfunction. This review focuses on the most up-to-date guideline for evaluation of pituitary function among men presenting with signs... (Review)
Review
PURPOSE OF REVIEW
Hypogonadism is a common endocrine dysfunction. This review focuses on the most up-to-date guideline for evaluation of pituitary function among men presenting with signs and symptoms of hypogonadism.
RECENT FINDINGS
The clinician must differentiate between primary (testicular) and secondary (pituitary-hypothalamic or central) hypogonadisms and be aware of adult-onset hypogonadism. If gonadotropins are low or inappropriately normal, the clinician must consider potential reversible causes in the hypothalamus-pituitary axis. Also, it is critical to understand the pitfalls of testosterone testing. When clinically indicated, evaluation of other pituitary hormone functions as well as pituitary magnetic resonance imaging may be necessary. Furthermore, it is essential to recognize that pituitary incidentalomas are common. Patients with microprolactinoma are more likely to present with symptoms of sexual dysfunction while those with macroprolactinoma are more likely to present with symptoms of mass effect. Some functional pituitary tumors respond to drug therapy while other nonfunctional tumors require surgical intervention. It is important for the clinician to understand the proper work-up of the hypogonadal patient with pituitary dysfunction and when necessary to refer to an endocrinologist or a neurosurgeon.
Topics: Gonadotropins; Humans; Hypogonadism; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Gland; Pituitary Neoplasms; Practice Guidelines as Topic; Prolactinoma; Sexual Dysfunction, Physiological; Testicular Diseases; Testosterone
PubMed: 31734886
DOI: 10.1007/s11934-019-0935-z