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Otolaryngologic Clinics of North America Apr 2022Cushing disease is a disorder of hypercortisolemia caused by hypersecretion of adrenocorticotropic hormone by a pituitary adenoma and is a rare diagnosis. Cushing... (Review)
Review
Cushing disease is a disorder of hypercortisolemia caused by hypersecretion of adrenocorticotropic hormone by a pituitary adenoma and is a rare diagnosis. Cushing disease presents with characteristic clinical signs and symptoms associated with excess cortisol, but diagnosis is difficult and often relies on repeated and varied endocrinologic assays and neuroradiologic investigations. Gold standard treatment is surgical resection of adrenocorticotropic hormone-secreting pituitary adenoma, which is curative. Patients require close endocrinologic follow-up for maintenance of associated neuroendocrine deficiencies and surveillance for potential recurrence. Medications, radiation therapy, and bilateral adrenalectomy are alternative treatments for residual or recurrent disease.
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Humans; Pituitary ACTH Hypersecretion; Pituitary Neoplasms
PubMed: 35256173
DOI: 10.1016/j.otc.2021.12.006 -
Pituitary Jun 2023The majority of corticotroph adenomas are benign but some are locally invasive, demonstrate high rates of recurrence, and exhibit a relatively poor response to often... (Review)
Review
The majority of corticotroph adenomas are benign but some are locally invasive, demonstrate high rates of recurrence, and exhibit a relatively poor response to often repeated surgical, medical, and radiation treatment. Herein, we summarize the currently known somatic and genetic mutations and other molecular factors that influence the pathogenesis of these tumors and discuss currently available therapies. Although recent molecular studies have advanced our understanding of the pathogenesis and behavior of these refractory corticotroph adenomas, these insights do not reliably guide treatment choices at present. Development of additional diagnostic tools and novel tumor-directed therapies that offer efficacious treatment choices for patients with refractory corticotroph adenomas are needed.
Topics: Humans; ACTH-Secreting Pituitary Adenoma; Adenoma; Pituitary Neoplasms
PubMed: 36917358
DOI: 10.1007/s11102-023-01308-5 -
Mayo Clinic Proceedings Oct 2022To perform a population-based study of pituitary adenoma epidemiology, including longitudinal trends in disease incidence, treatment patterns, and outcomes.
OBJECTIVE
To perform a population-based study of pituitary adenoma epidemiology, including longitudinal trends in disease incidence, treatment patterns, and outcomes.
PATIENTS AND METHODS
In this study of incident pituitary adenomas in Olmsted County, Minnesota, from January 1, 1989, through December 31, 2019, we identified 785 patients who underwent primary screening, 435 of whom were confirmed as harboring incident pituitary adenomas and were included. Primary outcomes of interest included demographic characteristics, presenting features, and disease outcomes (tumor control, biochemical control, and complications).
RESULTS
Among our 435 study patients, 438 unique pituitary adenomas were diagnosed at a median patient age of 39 years (interquartile range [IQR], 27 to 58 years). Adenomas were incidentally identified in 164 of the 438 tumors (37%). Common symptomatic presentations included hyperprolactinemia (188 of 438 [43%]) and visual field deficit (47 of 438 [11%]). Laboratory tests confirmed pituitary hormone hypersecretion in 238 of the 435 patients (55%), which was symptomatic in 222. The median tumor diameter was 8 mm (IQR, 5 to 17 mm). Primary management strategies were observation (156 of 438 tumors [36%]), medication (162 of 438 tumors [37%]), and transsphenoidal resection (120 of 438 tumors [27%]). Tumor and biochemical control were achieved in 398 (95%) and 216 (91%) secreting tumors, respectively. New posttreatment pituitary or visual deficits were noted in 43 (11%) and 8 (2%); apoplexy occurred in 28 (6%). Median clinical follow-up was 98 months (IQR, 47 to 189 months). Standardized incidence rates were 3.77 to 16.87 per 100,000 population, demonstrating linear expansion over time (R=0.67). The mean overall standardized incidence rate was 10.1 per 100,000 population; final point prevalence was 175.1 per 100,000 population.
CONCLUSION
Pituitary adenoma is a highly incident disease, with prolactin-secreting and incidental lesions representing the majority of tumors. Incidence rates and asymptomatic detection appear to be increasing over time. Presenting symptoms and treatment pathways are variable; however, most patients achieve favorable outcomes with observation or a single treatment modality.
Topics: Adenoma; Adult; Humans; Incidence; Middle Aged; Pituitary Hormones; Pituitary Neoplasms; Prolactin; Retrospective Studies; Treatment Outcome
PubMed: 35753823
DOI: 10.1016/j.mayocp.2022.03.017 -
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 -
Frontiers in Endocrinology 2022Acromegaly is a disease that occurs secondary to high levels of GH, most often from a hormone-secreting pituitary adenoma, with multisystem adverse effects. Diagnosis... (Review)
Review
Acromegaly is a disease that occurs secondary to high levels of GH, most often from a hormone-secreting pituitary adenoma, with multisystem adverse effects. Diagnosis includes serum GH and IGF-1 levels, and obtaining an MRI pituitary protocol to assess for a functional pituitary adenoma. Attempted gross total resection of the GH-secreting adenoma is the gold standard in treatment for patients with acromegaly for a goal of biochemical remission. Medical and radiation therapies are available when patients do not achieve biochemical cure after surgical therapy.
Topics: Acromegaly; Adenoma; Goals; Growth Hormone-Secreting Pituitary Adenoma; Humans; Pituitary Neoplasms
PubMed: 35992136
DOI: 10.3389/fendo.2022.924589 -
Frontiers in Endocrinology 2020Pituitary adenomas, accounting for 15% of diagnosed intracranial neoplasms, are usually benign and pharmacologically and surgically treatable; however, the critical... (Review)
Review
Pituitary adenomas, accounting for 15% of diagnosed intracranial neoplasms, are usually benign and pharmacologically and surgically treatable; however, the critical location, mass effects and hormone hypersecretion sustain their significant morbidity. Approximately 35% of pituitary tumors show a less benign course since they are highly proliferative and invasive, poorly resectable, and likely recurring. The latest WHO classification of pituitary tumors includes pituitary transcription factor assessment to determine adenohypophysis cell lineages and accurate designation of adenomas, nevertheless little is known about molecular and cellular pathways which contribute to pituitary tumorigenesis. In malignant tumors the identification of cancer stem cells radically changed the concepts of both tumorigenesis and pharmacological approaches. Cancer stem cells are defined as a subset of undifferentiated transformed cells from which the bulk of cancer cells populating a tumor mass is generated. These cells are able to self-renew, promoting tumor progression and recurrence of malignant tumors, also conferring cytotoxic drug resistance. On the other hand, the existence of stem cells within benign tumors is still debated. The presence of adult stem cells in human and murine pituitaries where they sustain the high plasticity of hormone-producing cells, allowed the hypothesis that putative tumor stem cells might exist in pituitary adenomas, reinforcing the concept that the cancer stem cell model could also be applied to pituitary tumorigenesis. In the last few years, the isolation and phenotypic characterization of putative pituitary adenoma stem-like cells was performed using a wide and heterogeneous variety of experimental models and techniques, although the role of these cells in adenoma initiation and progression is still not completely definite. The assessment of possible pituitary adenoma-initiating cell population would be of extreme relevance to better understand pituitary tumor biology and to identify novel potential diagnostic markers and pharmacological targets. In this review, we summarize the most updated studies focused on the definition of pituitary adenoma stem cell phenotype and functional features, highlighting the biological processes and intracellular pathways potentially involved in driving tumor growth, relapse, and therapy resistance.
Topics: Adenoma; Animals; Cell Transformation, Neoplastic; Drug Resistance, Neoplasm; Humans; Neoplasm Recurrence, Local; Neoplastic Stem Cells; Pituitary Neoplasms
PubMed: 32153500
DOI: 10.3389/fendo.2020.00054 -
Endocrinology and Metabolism Clinics of... Sep 2020Endoscopic transsphenoidal surgery for pituitary adenoma is a safe and highly effective first-line treatment that is well tolerated by patients. Potential complications... (Review)
Review
Endoscopic transsphenoidal surgery for pituitary adenoma is a safe and highly effective first-line treatment that is well tolerated by patients. Potential complications are plenty, and there is a large variation in complexity of surgery. This article presents the philosophy, surgical techniques, and outcomes of a high-volume pituitary adenoma center. Three surgical videos illustrate some procedures. The experience has reinforced the authors' belief that experience and surgical volume are key to high quality of care.
Topics: Adenoma; Endoscopy; Humans; Neurosurgical Procedures; Pituitary Neoplasms; Postoperative Complications
PubMed: 32741484
DOI: 10.1016/j.ecl.2020.05.011 -
Neurology India 2020The most common sellar pathology that merits neurosurgical attention is the pituitary adenoma. However, some developmental, inflammatory, and neoplastic lesions may... (Review)
Review
The most common sellar pathology that merits neurosurgical attention is the pituitary adenoma. However, some developmental, inflammatory, and neoplastic lesions may primarily or secondarily involve the sella, mimicking pituitary tumors. Advances in imaging and endocrinological assessment have helped in the recognition of these less common sellar, supra sellar pathologies, which may occasionally create confusion in management. The most common developmental anomaly is the Rathke's cleft cyst and an increasingly recognized inflammatory pathology is the spectrum of hypophysitis. Neoplasms, viz. Craniopharyngioma, Germinoma, Langerhans's cell histiocytosis or metastasis, have more distinctive features in various age groups and are more likely to be correctly diagnosed on current imaging and managed accordingly. This review looks at mainly intraparenchymal pathologies, namely Rathke's Cleft Cyst and various hypophysitides, and will discuss their management strategies.
Topics: Central Nervous System Cysts; Craniopharyngioma; Diagnosis, Differential; Humans; Pituitary Diseases; Pituitary Neoplasms
PubMed: 32611908
DOI: 10.4103/0028-3886.287682 -
Neurology India 2017Clinically relevant pituitary tumors presenting with altered hormonal secretion or mass effect represent a significant proportion of patients in endocrinology clinics.... (Review)
Review
Clinically relevant pituitary tumors presenting with altered hormonal secretion or mass effect represent a significant proportion of patients in endocrinology clinics. However, in recent years, these patients are also referred to clinical genetic services due to possible germline mutations causing syndromic or isolated pituitary adenomas. While somatic mutations have been identified in GNAS, USB8, PIK3CA, GPR101 and rarely in RAS, germline mutations have been identified in MEN1, cyclin dependent kinase inhibitor genes, AIP, DICER1, PRKAR1A, PRKACA, SDH genes and GPR101. In this review, we present a short overview of pituitary adenoma classifications, pituitary development and somatic and germline genetic changes identified in these adenomas.
Topics: Adenoma; Genetic Techniques; Genetics; Humans; Mutation; Pituitary Gland; Pituitary Neoplasms
PubMed: 28488625
DOI: 10.4103/neuroindia.NI_330_17 -
Annales D'endocrinologie Jul 2015After diagnosis of non-functioning pituitary adenoma and impact assessment (pituitary deficiency, visual field disorder), the question of management arises between... (Review)
Review
After diagnosis of non-functioning pituitary adenoma and impact assessment (pituitary deficiency, visual field disorder), the question of management arises between surgery and surveillance. This part of the Consensus document aims to clarify the principal situations encountered in clinical practice (visual disorder, pituitary deficiency, asymptomatic adenoma, etc.), so as to determine which ones indicate surgery and which ones simple surveillance. Particular contexts are also dealt with (elderly patients, young women hoping for pregnancy, etc). The principal surgical techniques (microscopy, endoscopy, etc.) are also considered. Finally, in case of surgery, the principal pathologic criteria are specified (immunolabeling, proliferation markers, etc.).
Topics: Adenoma; Clinical Decision-Making; Consensus; Humans; Neurosurgery; Pituitary Neoplasms; Vision Disorders
PubMed: 26070464
DOI: 10.1016/j.ando.2015.04.007