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Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment
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Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Endocrinology & Metabolism".

Deadline for manuscript submissions: closed (31 December 2021) | Viewed by 36129

Special Issue Editor

Prof. Dr. Shereen Ezzat

E-Mail Website
Guest Editor
1. Medicine & Oncology, University of Toronto, Toronto, ON, Canada
2. Head, Endocrine Oncology Site Group, University Health Network, Toronto, ON, Canada
3. Toronto General Hospital, Endocrine Oncology, Toronto, ON, Canada
Interests: pituitary tumors; acromegaly; cushing's disease; prolactinomas; non funtioning tumors; pituitary hormone abnormalities; other tumors; diseases of the pituitary gland and/or hypothalamus

Special Issue Information

Dear Colleagues,

Gigantism in children and acromegaly in adults are diseases manifested by growth hormone (GH) excess, usually from unrestrained production by the pituitary gland. Both conditions have proven immensely instructive in providing insights into the genetics, pathophysiology, and pharmacotherapeutic opportunities underpinning GH disorders. In this issue, we hope to gather experts from around the world to review the latest developments in this evolving area of medicine. What triggers should raise clinical suspicion and promote earlier detection? Do certain population genetic features shed light on disease risks? We will explore how recent germline predispositions play a role in pituitary tumorigenesis in general and in acromegaly/gigantism in particular. How do these genetic factors contribute to specific histomorphologic tumors of the somatotroph lineage? We will also learn how these different somatotroph tumor types reveal themselves differently on MR imaging. Collectively, the spectrum of genotype and morphologic phenotypes are emerging as clinical syndromes with distinct pharmacologic and therapeutic outcomes. Can drug class selection be based on specific features that will streamline management for better clinical outcomes that are more cost-effective?  What are the neurosurgical challenges in managing these tumors? Is there a role for sequenced multimodal therapies that can improve overall outcomes?

Prof. Dr. Shereen Ezzat
Guest Editor

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Keywords

  • pituitary tumor
  • acromegaly
  • gigantism
  • growth hormone
  • insulin-like growth factor 1

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Published Papers (6 papers)

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Research

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18 pages, 1445 KiB  
Article
Neutrophil-to-Lymphocyte, Platelet-to-Lymphocyte Ratios, and Systemic Immune-Inflammation Index as Potential Biomarkers of Chronic Inflammation in Patients with Newly Diagnosed Acromegaly: A Single-Centre Study
by Joanna Szydełko, Magdalena Szydełko-Gorzkowicz and Beata Matyjaszek-Matuszek
J. Clin. Med. 2021, 10(17), 3997; https://doi.org/10.3390/jcm10173997 - 3 Sep 2021
Cited by 7 | Viewed by 2819
Abstract
Acromegaly is a rare disease caused by overproduction of growth hormone (GH) by a pituitary adenoma, and consequently increased insulin-like growth factor 1 (IGF-1) concentration. The GH/IGF-1 axis and immune cells interactions are hypothesized to be involved in subclinical inflammation. This retrospective study [...] Read more.
Acromegaly is a rare disease caused by overproduction of growth hormone (GH) by a pituitary adenoma, and consequently increased insulin-like growth factor 1 (IGF-1) concentration. The GH/IGF-1 axis and immune cells interactions are hypothesized to be involved in subclinical inflammation. This retrospective study aimed to investigate the differences in neutrophil-to-lymphocyte (NLR), platelet-to-lymphocyte (PLR) ratios, and systemic immune-inflammation index (SII) in GH-secreting adenomas compared with non-functioning pituitary adenomas (NFPAs) concerning clinical and radiological findings. After evaluation of 665 patients with pituitary tumors, 62 individuals with newly diagnosed acromegaly and 134 with NFPAs were enrolled in the analysis. The control group consisted of 120 healthy individuals. Fifty-eight patients with acromegaly were re-evaluated after medical or surgical therapies. NLR, PLR, SII values, and neutrophil count were significantly higher (p ≤ 0.001), whereas lymphocyte count was lower in acromegaly than in NFPAs (p = 0.001). No significant differences between NFPAs and controls were observed in analyzed ratios. Higher preoperative NLR, PLR, SII values were found in patients who failed to achieve a cure with surgery (p < 0.05). Although NLR, PLR, and SII values were significantly higher in acromegaly, these indices cannot be used to discriminate GH-secreting pituitary tumors from NFPAs. Treatment of acromegaly decreased the value of NLR and SII, but it requires further studies to consolidate the real clinical role of these inflammation-related ratios. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
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12 pages, 614 KiB  
Article
3D DXA Hip Differences in Patients with Acromegaly or Adult Growth Hormone Deficiency
by Luis Gracia-Marco, Sheila Gonzalez-Salvatierra, Antonia Garcia-Martin, Esther Ubago-Guisado, Beatriz Garcia-Fontana, José Juan Gil-Cosano and Manuel Muñoz-Torres
J. Clin. Med. 2021, 10(4), 657; https://doi.org/10.3390/jcm10040657 - 9 Feb 2021
Cited by 4 | Viewed by 2649
Abstract
The skeleton is regulated by and responds to pituitary hormones, especially when the circulating levels are perturbed in disease. This study aims to analyse the between-group differences in 3D dual-energy X-ray absorptiometry (DXA) parameters at the hip site among patients with acromegaly or [...] Read more.
The skeleton is regulated by and responds to pituitary hormones, especially when the circulating levels are perturbed in disease. This study aims to analyse the between-group differences in 3D dual-energy X-ray absorptiometry (DXA) parameters at the hip site among patients with acromegaly or adult growth hormone deficiency (AGHD) and a healthy control group. The current cross-sectional study includes data for 67 adults, 20 with acromegaly, 14 with AGHD and 33 healthy controls. We obtained the areal bone mineral density (aBMD) outcomes using DXA and cortical and trabecular parameters using 3D-DXA software (3D-SHAPER). The mean-adjusted 3D-DXA parameters did not differ between acromegaly patients and the controls (p > 0.05); however, we found cortical bone impairment (−7.3% to −8.4%; effect size (ES) = 0.78) in AGHD patients (p < 0.05). Differences in the cortical bone parameters were more evident when comparing AGHD patients (−8.5% to −16.2%; ES = 1.22 to 1.24) with acromegaly patients (p < 0.05). In brief, the 3D mapping highlighted the trochanter as the site with greater cortical bone differences between acromegaly patients and the controls. Overall, AGHD patients displayed lower cortical parameters at the trochanter, femoral neck and intertrochanter compared to the controls and acromegaly patients. To sum up, 3D-DXA provided useful information about the characteristics of bone involvement in growth hormone (GH)-related disorders. Patients with AGHD showed distinct involvement of the cortical structure. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
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Review

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16 pages, 9353 KiB  
Review
An Update on Pituitary Neuroendocrine Tumors Leading to Acromegaly and Gigantism
by Sylvia L. Asa and Shereen Ezzat
J. Clin. Med. 2021, 10(11), 2254; https://doi.org/10.3390/jcm10112254 - 22 May 2021
Cited by 20 | Viewed by 7797
Abstract
An excess of growth hormone (GH) results in accelerated growth and in childhood, the clinical manifestation is gigantism. When GH excess has its onset after epiphyseal fusion at puberty, the overgrowth of soft tissue and bone results in acromegaly. Persistent GH excess in [...] Read more.
An excess of growth hormone (GH) results in accelerated growth and in childhood, the clinical manifestation is gigantism. When GH excess has its onset after epiphyseal fusion at puberty, the overgrowth of soft tissue and bone results in acromegaly. Persistent GH excess in gigantism also causes acromegalic features that become evident in the adult years. The causes of GH excess are primarily lesions in the pituitary, which is the main source of GH. In this review, we provide an update on the clinical, radiological and pathologic features of the various types of pituitary neuroendocrine tumors (PitNETs) that produce GH. These tumors are all derived from PIT1-lineage cells. Those composed of somatotrophs may be densely granulated, resembling normal somatotrophs, or sparsely granulated with unusual fibrous bodies. Those composed of mammosomatotrophs also produce prolactin; rare plurihormonal tumors composed of cells that resemble mammosomatotrophs also produce TSH. Some PitNETs are composed of immature PIT1-lineage cells that do not resemble differentiated somatotrophs, mammosomatotrophs, lactotroph or thyrotrophs; these tumors may cause GH excess. An unusual oncocytic PIT1-lineage tumor known as the acidophil stem cell tumor is predominantly a lactotroph tumor but may express GH. Immature PIT1-lineage cells that express variable amounts of hormones alone or in combination can sometimes cause GH excess. Unusual tumors that do not follow normal lineage differentiation may also secrete GH. Exceptional examples of acromegaly/gigantism are caused by sellar tumors composed of hypothalamic GHRH-producing neurons, alone or associated with a sparsely granulated somatotroph tumor. Each of these various tumors has distinct clinical, biochemical and radiological features. Data from careful studies based on morphologic subtyping indicate that morphologic classification has both prognostic and predictive value. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
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24 pages, 780 KiB  
Review
Genetics of Acromegaly and Gigantism
by Anna Bogusławska and Márta Korbonits
J. Clin. Med. 2021, 10(7), 1377; https://doi.org/10.3390/jcm10071377 - 29 Mar 2021
Cited by 34 | Viewed by 8683
Abstract
Growth hormone (GH)-secreting pituitary tumours represent the most genetically determined pituitary tumour type. This is true both for germline and somatic mutations. Germline mutations occur in several known genes (AIP, PRKAR1A, GPR101, GNAS, MEN1, CDKN1B, SDHx [...] Read more.
Growth hormone (GH)-secreting pituitary tumours represent the most genetically determined pituitary tumour type. This is true both for germline and somatic mutations. Germline mutations occur in several known genes (AIP, PRKAR1A, GPR101, GNAS, MEN1, CDKN1B, SDHx, MAX) as well as familial cases with currently unknown genes, while somatic mutations in GNAS are present in up to 40% of tumours. If the disease starts before the fusion of the epiphysis, then accelerated growth and increased final height, or gigantism, can develop, where a genetic background can be identified in half of the cases. Hereditary GH-secreting pituitary adenoma (PA) can manifest as isolated tumours, familial isolated pituitary adenoma (FIPA) including cases with AIP mutations or GPR101 duplications (X-linked acrogigantism, XLAG) or can be a part of systemic diseases like multiple endocrine neoplasia type 1 or type 4, McCune–Albright syndrome, Carney complex or phaeochromocytoma/paraganglioma-pituitary adenoma association. Family history and a search for associated syndromic manifestations can help to draw attention to genetic causes; many of these are now tested as part of gene panels. Identifying genetic mutations allows appropriate screening of associated comorbidities as well as finding affected family members before the clinical manifestation of the disease. This review focuses on germline and somatic mutations predisposing to acromegaly and gigantism. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
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8 pages, 226 KiB  
Review
Towards an Earlier Diagnosis of Acromegaly and Gigantism
by Jill Sisco and Aart J. van der Lely
J. Clin. Med. 2021, 10(7), 1363; https://doi.org/10.3390/jcm10071363 - 26 Mar 2021
Cited by 7 | Viewed by 5229
Abstract
Acromegaly is a rare disease and the clinical features of acromegaly develop insidiously; its diagnosis is often significantly delayed. Therefore, earlier diagnosis will improve the quality of life of the patient and reduce the need for other therapies to control the initial and [...] Read more.
Acromegaly is a rare disease and the clinical features of acromegaly develop insidiously; its diagnosis is often significantly delayed. Therefore, earlier diagnosis will improve the quality of life of the patient and reduce the need for other therapies to control the initial and ongoing damage that acromegaly presents. In this chapter, we describe the view of the patient and the clinician on the importance of earlier diagnosis, as well as on what can be done to speed up this process. Earlier diagnosis will not only improve quality of life and the burden of disease in acromegaly patients, but it will also have a positive impact in the economic burden of this rare disease. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
8 pages, 249 KiB  
Review
The Biochemical Diagnosis of Acromegaly
by Amit Akirov, Hiba Masri-Iraqi, Idit Dotan and Ilan Shimon
J. Clin. Med. 2021, 10(5), 1147; https://doi.org/10.3390/jcm10051147 - 9 Mar 2021
Cited by 23 | Viewed by 6554
Abstract
Background: The diagnosis of acromegaly still poses a clinical challenge, and prolonged diagnostic delay is common. The most important assays for the biochemical diagnosis and management of acromegaly are growth hormone (GH) and insulin-like growth factor-1 (IGF-1). Objective: Discuss the role of IGF-1, [...] Read more.
Background: The diagnosis of acromegaly still poses a clinical challenge, and prolonged diagnostic delay is common. The most important assays for the biochemical diagnosis and management of acromegaly are growth hormone (GH) and insulin-like growth factor-1 (IGF-1). Objective: Discuss the role of IGF-1, basal serum GH, and nadir GH after oral glucose tolerance test (OGTT) for the diagnosis, management, and treatment of patients with acromegaly. Methods: We performed a narrative review of the published data on the biochemical diagnosis and monitoring of acromegaly. An English-language search for relevant studies was conducted on PubMed from inception to 1 January 2021. The reference lists of relevant studies were also reviewed. Results: Serum IGF-1 levels, basal GH values, and nadir GH after OGTT play a major role in the diagnosis, management, and treatment of patients with acromegaly. Measurement of IGF-1 levels is the key factor in the diagnosis and monitoring of acromegaly, but basal and nadir GH following OGTT are also important. However, several factors may significantly influence the concentrations of these hormones, including assay methods, physiologic and pathologic factors. In some cases, discordant GH and IGF-1 levels may be challenging and usually requires additional data and monitoring. Conclusion: New GH and IGF-1 standards are much more precise and provide more accurate tools to diagnose and monitor patients with acromegaly. However, all these biochemical tools have their limitations, and these should be taken under consideration, along with the history, clinical features and imaging studies, when assessing patients for acromegaly. Full article
(This article belongs to the Special Issue Acromegaly and Gigantism: Latest Developments in Pathogenesis and Treatment)
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