Extensive Clinical Experience:Changing Patterns in Diagnosis and Therapy of Acromegaly over Two Decades

Nachtigall, Lisa B.; Delgado, Adriano; Swearingen, Brooke; Lee, Hang; Zerikly, R. Kurdi; Klibanski, Anne

doi:10.1210/jc.2007-2149

Cited by 124 publications

(94 citation statements)

References 36 publications

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“…The authors of many older series have reported a delay in the diagnosis of 7-10 years after the onset of signs and symptoms. 24 However, in a recent retrospective study Nachtigall et al 50 reported a decrease in this interval to roughly 2-3 years. Given that tumor size has been established as an important predictor of surgical outcome, early recognition and treatment are considered keys to achieving high rates of remission and avoiding long-term comorbidities.…”

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confidence: 95%

Outcomes after a purely endoscopic transsphenoidal resection of growth hormone–secreting pituitary adenomas

Campbell¹,

Kenning

Andrews³

et al. 2010

FOC

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Object Using strict biochemical remission criteria, the authors assessed surgical outcomes after endoscopic transsphenoidal resection of growth hormone (GH)–secreting pituitary adenomas and identified preoperative factors that significantly influence the rate of remission. Methods A retrospective review of a prospectively maintained database was performed. The authors reviewed cases in which an endoscopic resection of GH-secreting pituitary adenomas was performed. The cohort consisted of 26 patients who had been followed for 3–60 months (mean 24.5 months). The thresholds of an age-appropriate, normalized insulin-like growth factor–I concentration, a nadir GH level after oral glucose load of less than 1.0 μg/l, and a random GH value of less than 2.5 μg/l were required to establish biochemical cure postoperatively. Results Overall, in 57.7% of patients undergoing a purely endoscopic transsphenoidal pituitary adenectomy for acromegaly, an endocrinological cure was achieved. The mean clinical follow-up duration was 24.5 months. In patients with microadenomas (4 cases) the cure rate was 75%, whereas in patients harboring macroadenomas (22 cases) the cure rate was 54.5%. Cavernous sinus invasion (Knosp Grades 3 and 4) was associated with a significantly lower remission rate (p = 0.0068). Hardy Grade 3 and 4 tumors were also less likely to achieve biochemical cure (p = 0.013). The overall complication rate was 11.5% including 2 incidents of transient diabetes insipidus and 1 postoperative CSF leak, which were treated nonoperatively. Conclusions A purely endoscopic transsphenoidal approach to GH-secreting pituitary adenomas leads to similar outcome for noninvasive macroadenomas compared with traditional microsurgical techniques. Furthermore, this approach may often provide maximal visualization of the tumor, the pituitary gland, and the surrounding neurovascular structures.

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confidence: 95%

Outcomes after a purely endoscopic transsphenoidal resection of growth hormone–secreting pituitary adenomas

Campbell¹,

Kenning

Andrews³

et al. 2010

FOC

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“…Although the disorder has been recognized since antiquity, the pathology of pituitary "prosopectasia" was first described by Andrea Verga in 1864 and the clinical features of acromegaly by Pierre Marie in 1886. Disease pathogenesis involves growth hormone (GH) hypersecretion by tumorous pituitary somatotroph cells, and the diagnosis is invariably preceded by about 10 years of active but unrecognized disease (1)(2)(3). Clinical presentation of acromegaly, in descending frequency as determined in a study of approximately 600 patients, includes acral and facial changes, hyperhidrosis (abnormally increased perspiration), headaches, paresthesia ("pins and needles" tingling sensation), sexual dysfunction, hypertension, goiter, and rarely, visual field defects (4) (see Sidebar 1).…”

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confidence: 99%

Acromegaly pathogenesis and treatment

Melmed¹

2009

J. Clin. Invest.

608

472

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Dysregulated growth hormone (GH) hypersecretion is usually caused by a GH-secreting pituitary adenoma and leads to acromegaly -a disorder of disproportionate skeletal, tissue, and organ growth. High GH and IGF1 levels lead to comorbidities including arthritis, facial changes, prognathism, and glucose intolerance. If the condition is untreated, enhanced mortality due to cardiovascular, cerebrovascular, and pulmonary dysfunction is associated with a 30% decrease in life span. This Review discusses acromegaly pathogenesis and management options. The latter include surgery, radiation, and use of novel medications. Somatostatin receptor (SSTR) ligands inhibit GH release, control tumor growth, and attenuate peripheral GH action, while GH receptor antagonists block GH action and effectively lower IGF1 levels. Novel peptides, including SSTR ligands, exhibiting polyreceptor subtype affinities and chimeric dopaminergic-somatostatinergic properties are currently in clinical trials. Effective control of GH and IGF1 hypersecretion and ablation or stabilization of the pituitary tumor mass lead to improved comorbidities and lowering of mortality rates for this hormonal disorder.

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“…However, follow-up suggests that these patients probably did not have acromegaly, First, considering the interval between the onset of manifestations and diagnosis, with the typical phenotype already present (33,34), the absence of this phenotype and of changes in physiognomy after 5 years makes the disease unlikely. Second, spontaneous normalization or absence of an increase in IGF-1 after this period also weakens the diagnosis.…”

Section: Discussionmentioning

confidence: 97%

Long-term follow-up of patients with elevated IGF-1 and nadir GH > 0.4 µg/L but < 1 µg/L

Rosário

Calsolari

2017

Arch. Endocrinol. Metab.

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Objective: To report the results of initial investigation and after 5 years of patients with a suspicious clinical scenario for acromegaly, elevated IGF-1, and nadir GH during an oral glucose tolerance test (OGTT) > 0.4 µg/L but < 1 µg/L. Subjects and methods: Seventeen patients who had elevated IGF-1 (outside puberty and pregnancy) in two measurements and GH between 0.4 and 1 µg/L during OGTT were selected. Results: During initial assessment, only one patient had microadenoma on magnetic resonance imaging (MRI) of the pituitary. In this patient, IGF-1 returned to normal spontaneously after 5 years. In the remaining 16 patients, spontaneous normalization of IGF-1 was observed in four and IGF-1 continued to be elevated in 12 after 5 years. None of the latter patients developed a phenotype of acromegaly, changes in physiognomy or increase in IGF-1 and no tumor was detected by imaging methods. Two patients had nadir GH < 0.4 µg/L, while the nadir GH remained between 0.4 and 1 µg/L in 10 patients. Conclusion: In patients (notably young adult or adult women) without a typical phenotype in whom IGF-1 is measured due to a suspicious clinical scenario and is found to be slightly elevated, even if confirmed and in the absence of other causes, a nadir GH cut-off value of 0.4 µg/L instead of 1 µg/L in the OGTT might be inadequate for the diagnosis. Arch Endocrinol Metab. 2017;61(5):426-31

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Extensive Clinical Experience:Changing Patterns in Diagnosis and Therapy of Acromegaly over Two Decades

Cited by 124 publications

References 36 publications

Outcomes after a purely endoscopic transsphenoidal resection of growth hormone–secreting pituitary adenomas

Outcomes after a purely endoscopic transsphenoidal resection of growth hormone–secreting pituitary adenomas

Acromegaly pathogenesis and treatment

Long-term follow-up of patients with elevated IGF-1 and nadir GH > 0.4 µg/L but < 1 µg/L

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