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Journal of Neuro-oncology Jun 2024Functioning pituitary adenomas (FPAs) include most frequently prolactinomas, somatotroph or corticotroph adenomas, while thyrotroph and gonadotroph adenomas are very... (Review)
Review
CONTEXT
Functioning pituitary adenomas (FPAs) include most frequently prolactinomas, somatotroph or corticotroph adenomas, while thyrotroph and gonadotroph adenomas are very rare. Despite their benign histological nature (aggressive tumors are rare and malignant ones exceptional), FPAs could cause significant morbidity and increased mortality due to complications associated with hormonal excess syndromes and/or mass effect leading to compression of adjacent structures. This mini review will focus on the increasing role of medical therapy in the multimodal treatment, which also includes transsphenoidal surgery (TSS) and radiotherapy.
EVIDENCE SYNTHESIS
Most patients with prolactinomas are treated only with medications, but surgery could be considered for some patients in a specialized pituitary center, if higher chances of cure. Dopamine agonists, especially cabergoline, are efficient in reducing tumor size and normalizing prolactin. TSS is the first-line treatment for all other FPAs, but most patients require complex adjuvant treatment, including a combination of therapeutic approaches. Medical therapy is the cornerstone of treatment in all patients after unsuccessful surgery or when surgery cannot be offered and includes somatostatin receptor ligands and dopamine agonists (almost all FPAs), growth hormone receptor antagonists (acromegaly), adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers (Cushing's disease). Novel medical treatments, especially for acromegaly and Cushing's disease are under research.
CONCLUSIONS
An enlarged panel of effective drugs available with increased knowledge of predictive factors for response and/or adverse effects will enhance the possibility to offer a more individualized treatment. This would not only improve disease control and prognosis, but also quality of life.
Topics: Humans; Pituitary Neoplasms; Adenoma; Combined Modality Therapy; Clinical Trials as Topic
PubMed: 38760632
DOI: 10.1007/s11060-024-04670-x -
JCEM Case Reports May 2024Acromegaly is a rare pituitary condition stemming from hypersecretion of growth hormone (GH). Classic presentation involves enlarged hands, feet, and coarse facial...
Acromegaly is a rare pituitary condition stemming from hypersecretion of growth hormone (GH). Classic presentation involves enlarged hands, feet, and coarse facial features. However, late-onset cardiac manifestations develop in the absence of disease control. Of the various cardiac complications, heart failure is the rarest (3%-4% of cases). Here we present a case of acromegaly diagnosed after the patient exhibited symptoms of heart failure, with eventual placement of a left ventricular assist device (LVAD) as a bridge to orthotopic heart transplant. The 37-year-old patient originally presented with exercise intolerance and "heavy heartbeats" but was found to be in acute decompensated heart failure, with an ejection fraction (EF) of 15%. The acromegaly diagnosis was confirmed with labs, and he began treatment with lanreotide 120 mg weekly along with 0.5 mg cabergoline twice weekly. EF improved up to 30%. Soon after, he was lost to follow-up during the COVID-19 pandemic and returned with worsening EF. An LVAD was placed to support recovery while the patient awaited heart transplant. While LVADs are a common measure of cardiac support for ischemic cardiomyopathy, they can also be successful options in the setting of GH-driven cardiomegaly.
PubMed: 38746051
DOI: 10.1210/jcemcr/luae072 -
Cureus Apr 2024Acrochordons are polypoid, skin-colored lesions usually present at the site of skin folds. They are extremely rare in the preputial area of the penis and do not tend to...
Acrochordons are polypoid, skin-colored lesions usually present at the site of skin folds. They are extremely rare in the preputial area of the penis and do not tend to grow. To the best of our knowledge, in English literature, this report presents the first case of an androgen receptor-positive penile acrochordon, which is located on the penis and showed rapid growth along with body development during puberty with no underlying causes such as acromegaly, diabetes, obesity, and trauma.
PubMed: 38741806
DOI: 10.7759/cureus.58177 -
Clinical Case Reports May 2024Our case highlights the importance of recognizing acromegaly as a potential risk factor for venous thromboembolism (VTE). Despite a thorough thrombophilia workup...
KEY CLINICAL MESSAGE
Our case highlights the importance of recognizing acromegaly as a potential risk factor for venous thromboembolism (VTE). Despite a thorough thrombophilia workup yielding unremarkable results, further research is warranted to elucidate the underlying mechanisms linking acromegaly and thrombophilia. This understanding will aid in improving risk assessment and management strategies for patients with acromegaly.
ABSTRACT
Acromegaly, a rare disorder characterized by excessive growth hormone secretion, is associated with various comorbidities including hypertension, diabetes mellitus, and obstructive sleep apnea. While previous studies have identified abnormalities in hemostatic factors in acromegaly patients, the association between acromegaly and venous thromboembolism (VTE) remains poorly understood. We present the case of a 36-year-old male with a history of acromegaly who presented with acute dyspnea, chest pain, and cough. Despite a prior trans-sphenoidal hypophysectomy, his acromegaly symptoms persisted. Upon evaluation, he was found to have bilateral pulmonary embolism. Thorough thrombophilia workup was unremarkable, suggesting acromegaly as a potential risk factor for VTE.
PubMed: 38736578
DOI: 10.1002/ccr3.8867 -
Oral Diseases May 2024
PubMed: 38735856
DOI: 10.1111/odi.15000 -
The Journal of Medical Investigation :... 2024Pancreatic GHRHomas (pGHRHomas) with acromegaly have unique conditions, harboring the existence of multiple endocrine neoplasia type 1 (MEN 1). Moreover, pituitary... (Review)
Review
Pancreatic GHRHomas (pGHRHomas) with acromegaly have unique conditions, harboring the existence of multiple endocrine neoplasia type 1 (MEN 1). Moreover, pituitary lesions are affected by both protracted ectopic GHRH and loss of menin function. Of significance is the clarification of clinicopathological aspects of pGHRHomas in patients with or without MEN 1. From 1977-2016, thirty-six patients with pGHRHomas were reported. Twenty-two out of 36 patients (61%) had pGHRHomas with MEN 1 and 14 patients did not. The former had a tendency of male predominance, benign tumor behavior and fewer metastasis rather than the latter. The latter is a single pGHRHoma accompanied by pituitary enlargement with somatotroph hyperplasia (hyperplasia) caused by protracted ectopic GHRH. Nine patients with MEN 1 underwent transsphenoidal surgery (TSS). The hyperplasia associated with various pituitary adenomas (PAs) including three GH-related adenomas was observed in seven subjects (32%). In these patients, the resection of their pGHRHomas was feasible. Furthermore, all patients with acromegaly due to pGHRHomas without MEN 1 had non-TSS, whereas approximately 70% of those with MEN 1 had unnecessary TSS. The association with hyperplasia and various PAs suggested that formation of the three GH-related adenomas may be induced by the foundations of MEN 1 gene mutations. J. Med. Invest. 71 : 1-8, February, 2024.
Topics: Humans; Multiple Endocrine Neoplasia Type 1; Pancreatic Neoplasms; Male; Female; Acromegaly; Middle Aged; Adult; Aged
PubMed: 38735704
DOI: 10.2152/jmi.71.1 -
Journal of Neurological Surgery. Part... Jun 2024Surgery is the treatment of choice for growth hormone (GH)-secreting pituitary adenoma. The remission of random GH depends on various factors. We aimed to evaluate...
Surgery is the treatment of choice for growth hormone (GH)-secreting pituitary adenoma. The remission of random GH depends on various factors. We aimed to evaluate the predictors related to remission of random GH following surgical treatment. We collected the data retrospectively from the chart review from a single unit of neurosurgery. The diagnostic criteria for remission were a random GH < 1 ng/mL or nadir GH < 0.4 ng/mL after an oral glucose tolerance test. Data from a total of 110 (females 62 [56.4%]) patients were available for follow-up and were analyzed. The mean age was 36.5 years (14-69 years). Vision impairments were seen in 39 (35.5%) patients. The mean duration of symptoms before surgery was 34 months. The mean volume of the tumor was 7.2 mL (0.44-109.8 mL). Knosp grade 3 and 4 tumors were seen in 41.5% of cases. The mean preoperative random GH level was 68.9 ng/mL. Transsphenoidal surgery was done in 107 (97.3%) cases. The gross total resection could be done in 36 (32.7%) cases. At 3 months, 25 (26%) patients had a biochemical remission. In univariable analysis, lower Knosp grade, preoperative GH level < 40 ng/mL, gross total resection, and male gender were associated with remission at 3 months. In regression analysis, preoperative GH and male gender were related to remission at 3 months. The preoperative GH level < 40 ng/mL is associated with higher chances of remission after surgery for GH-secreting pituitary adenoma.
PubMed: 38721370
DOI: 10.1055/s-0043-57233 -
Ginekologia Polska May 2024
PubMed: 38717221
DOI: 10.5603/gpl.97745 -
Journal of the Endocrine Society Apr 2024Fracture rate is increased in patients with active acromegaly and those in remission. Abnormalities of bone microstructure are present in patients with active disease...
CONTEXT
Fracture rate is increased in patients with active acromegaly and those in remission. Abnormalities of bone microstructure are present in patients with active disease and persist despite biochemical control after surgery. Effects of treatment with the GH receptor antagonist pegvisomant on bone microstructure were unknown.
METHODS
We studied 25 patients with acromegaly (15 men, 10 women). In 20, we evaluated areal bone mineral density (BMD) by dual-energy X-ray absorptiometry and bone turnover markers (BTMs) longitudinally, before and during pegvisomant treatment. After long-term pegvisomant in 17, we cross-sectionally assessed volumetric BMD, microarchitecture, stiffness, and failure load of the distal radius and tibia using high-resolution peripheral quantitative computed tomography (HRpQCT) and compared these results to those of healthy controls and 2 comparison groups of nonpegvisomant-treated acromegaly patients, remission, and active disease, matched for other therapies and characteristics.
RESULTS
In the longitudinal study, areal BMD improved at the lumbar spine but decreased at the hip in men after a median ∼7 years of pegvisomant. In the cross-sectional study, patients on a median ∼9 years of pegvisomant had significantly larger bones, lower trabecular and cortical volumetric density, and disrupted trabecular microarchitecture compared to healthy controls. Microstructure was similar in the pegvisomant and acromegaly comparison groups. BTMs were lowered, then stable over time.
CONCLUSION
In this, the first study to examine bone microstructure in pegvisomant-treated acromegaly, we found deficits in volumetric BMD and microarchitecture of the peripheral skeleton. BTM levels remained stable with long-term therapy. Deficits in bone quality identified by HRpQCT may play a role in the pathogenesis of fragility in treated acromegaly.
PubMed: 38715589
DOI: 10.1210/jendso/bvae079 -
Journal of Clinical Medicine Research Apr 2024Biliary complications are frequent in patients with acromegaly. These complications may be secondary either to acromegaly or to somatostatin analogs (SAs). We aimed in...
BACKGROUND
Biliary complications are frequent in patients with acromegaly. These complications may be secondary either to acromegaly or to somatostatin analogs (SAs). We aimed in this paper to assess the prevalence of biliary complications in patients with acromegaly at diagnosis and after treatment with SAs.
METHODS
We conducted an analytical and descriptive retrospective study of 26 patients followed up for acromegaly over 7 years. Biliary complications were screened at diagnosis and follow-up by abdominal ultrasound, biliary magnetic resonance imaging (MRI), and endoscopic ultrasonography (EUS). Data were analyzed using SPSS 21.
RESULTS
The mean age of the patients was 49.6 ± 14 years, with a female predominance (53.8%). The evaluation of biliary complications showed vesicular biliary tract lithiasis and/or sludge in seven patients (29%), including two patients at the time of diagnosis of acromegaly and five patients after an average medical treatment duration of 3 years. Six female patients (24%) had dilation of the bile ducts without the presence of obstruction on biliary MRI and EUS and lithiasis/sludge of the common bile duct, tumor or external compression have been excluded. This condition was discovered incidentally at the diagnosis in five patients and during the follow-up in one patient. The preoperative insulin-like growth factor 1 (IGF-1) levels, disease duration, and female sex were significantly correlated with biliary tract dilation occurrence. Dyslipidemia, the preoperative IGF-1 level, and lanreotide treatment duration were significantly correlated with the occurrence of biliary lithiasis (P < 0.05).
CONCLUSION
Biliary stones are a frequent biliary adverse effect in patients with acromegaly undergoing SAs treatment. However, primary bile duct dilation has never been reported in acromegaly to the best of our knowledge. This condition could be considered as a complication or a feature of the disease.
PubMed: 38715563
DOI: 10.14740/jocmr5140