{"id":24605,"date":"2026-02-28T22:54:14","date_gmt":"2026-03-01T02:54:14","guid":{"rendered":"https:\/\/cliniqueomicron.ca\/gigantisme\/"},"modified":"2026-03-09T17:04:11","modified_gmt":"2026-03-09T21:04:11","slug":"gigantism","status":"publish","type":"page","link":"https:\/\/cliniqueomicron.ca\/en\/gigantisme\/","title":{"rendered":"Gigantism and acromegaly: causes, diagnosis and treatment | Clinique Omicron"},"content":{"rendered":"<div data-elementor-type=\"wp-page\" data-elementor-id=\"24605\" class=\"elementor elementor-24605\" data-elementor-post-type=\"page\">\n\t\t\t\t<div class=\"elementor-element elementor-element-de26559 e-flex e-con-boxed e-con e-parent\" data-id=\"de26559\" data-element_type=\"container\" data-e-type=\"container\" data-settings=\"{&quot;ekit_has_onepagescroll_dot&quot;:&quot;yes&quot;}\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t\t\t<div class=\"elementor-element elementor-element-06ac8b3 elementor-widget elementor-widget-html\" data-id=\"06ac8b3\" data-element_type=\"widget\" data-e-type=\"widget\" data-settings=\"{&quot;ekit_we_effect_on&quot;:&quot;none&quot;}\" data-widget_type=\"html.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<!DOCTYPE html>\n<html lang=\"fr\">\n<head>\n<meta charset=\"UTF-8\">\n<meta name=\"viewport\" content=\"width=device-width, initial-scale=1.0\">\n<title>Gigantism and acromegaly: causes, diagnosis and treatment | Clinique Omicron<\/title>\n<meta name=\"description\" content=\"Gigantism is caused by an excess of growth hormone prior to cartilage fusion. Pituitary adenoma, IGF-1, transsphenoidal surgery and management in Quebec.\">\n<meta name=\"keywords\" content=\"gigantisme traitement, gigantisme hormone de croissance, acrom\u00e9galie gigantisme, ad\u00e9nome hypophysaire GH, IGF-1 \u00e9lev\u00e9, octr\u00e9otide lanr\u00e9otide gigantisme, chirurgie transsph\u00e9no\u00efdale hypophyse, gigantisme Qu\u00e9bec\">\n<link rel=\"preconnect\" href=\"https:\/\/fonts.googleapis.com\">\n<link href=\"https:\/\/fonts.googleapis.com\/css2?family=Cinzel:wght@600&family=Poppins:wght@400;500;600;700&display=swap\" rel=\"stylesheet\">\n<style>\n.co-wrap * { font-family: 'Poppins', sans-serif; box-sizing: border-box; }\n.co-wrap { max-width: 1100px; margin: 0 auto; padding: 30px 0 60px; }\n.co-label { font-family: 'Cinzel', serif; font-size: 14px; font-weight: bold; letter-spacing: 1px; text-transform: uppercase; color: #4D6577; margin-bottom: 14px; display: block; }\n.co-wrap h1 { font-size: 32px; font-weight: 500; color: #323C52; margin: 0 0 22px; line-height: 1.2; }\n.co-intro { font-size: 16px; line-height: 1.75; color: #4D6577; margin-bottom: 36px; padding-bottom: 32px; border-bottom: 1px solid rgba(77,101,119,.2); }\n.co-wrap h2 { font-size: 20px; font-weight: 600; color: #323C52; margin: 32px 0 12px; }\n.co-wrap p { font-size: 15px; color: #4D6577; line-height: 1.7; margin-bottom: 14px; }\n.co-list { list-style: none; padding: 0; margin: 12px 0 24px; }\n.co-list li { font-size: 15px; color: #4D6577; padding: 10px 14px 10px 38px; margin-bottom: 8px; border-radius: 6px; position: relative; background: rgba(77,101,119,.06); border-left: 3px solid #4D6577; }\n.co-list li::before { content: \"\u2713\"; position: absolute; left: 12px; font-weight: 700; color: #4D6577; }\n.co-table { width: 100%; border-collapse: collapse; margin: 14px 0 22px; font-size: 14px; border-radius: 8px; overflow: hidden; table-layout: fixed; }\n.co-table thead tr { background: #323C52; color: #fff; }\n.co-table thead th { padding: 11px 16px; text-align: left; font-weight: 600; font-size: 13px; }\n.co-table tbody tr:nth-child(even) { background: rgba(77,101,119,.06); }\n.co-table tbody tr:nth-child(odd) { background: #fff; }\n.co-table td { padding: 10px 16px; color: #4D6577; border-bottom: 1px solid rgba(77,101,119,.12); font-size: 14px; vertical-align: top; }\n.co-table td:first-child { font-weight: 600; color: #323C52; }\n.co-infobox { display: flex; gap: 12px; background: rgba(77,101,119,.06); border-radius: 8px; border-left: 4px solid #4D6577; padding: 14px 18px; margin: 18px 0 28px; font-size: 14px; color: #4D6577; line-height: 1.65; }\n.co-infobox .ico { font-size: 18px; flex-shrink: 0; }\n.co-urgence { background: #fff8f8; border-left: 5px solid #c0392b; border-radius: 6px; padding: 20px 26px; margin: 24px 0 32px; }\n.co-urgence .co-urgence-titre { font-size: 13px; font-weight: 700; color: #c0392b; letter-spacing: 1.5px; text-transform: uppercase; margin-bottom: 10px; }\n.co-urgence p { color: #5a2020; font-size: 14px; margin: 0 0 10px; line-height: 1.7; }\n.co-urgence p:last-child { margin-bottom: 0; }\n.co-disclaimer { font-size: 13px; color: #8a9aaa; font-style: italic; border-top: 1px solid rgba(77,101,119,.15); padding-top: 24px; margin-top: 40px; line-height: 1.6; }\n<\/style>\n<\/head>\n<body>\n<div class=\"co-wrap\">\n  <span class=\"co-label\">Endocrinology &amp; Neurosurgery &amp; Family Medicine<\/span>\n  <h1>Gigantism and acromegaly<\/h1>\n\n  <div class=\"co-intro\">\n    Gigantism is a syndrome caused by excessive secretion of growth hormone (GH) that occurs before the epiphyseal growth plates have fused, leading to excessive height growth. When GH hypersecretion occurs after the fusion of the growth plates\u2014that is, in adults\u2014the condition is called acromegaly, characterized by enlargement of the extremities (hands, feet, jaw) and progressive visceral changes. These two conditions represent the two clinical manifestations of the same pathophysiological process: chronic GH hypersecretion, responsible for a sustained elevation of IGF-1 (insulin-like growth factor 1\u2014somatomedin C), the primary mediator of GH\u2019s anabolic and growth effects. In more than 95% of cases, the cause is a somatotrophic (benign) pituitary adenoma\u2014a microadenoma (&lt;10 mm) or a macroadenoma (&gt;10 mm). Pediatric forms of gigantism are particularly rare (estimated incidence of 5\u201310 cases per million children) and may be associated with genetic conditions (multiple endocrine neoplasia type 1\u2014MEN1, Carney syndrome, XLAG syndrome\u2014X-linked acrogigantism associated with duplications of the GPR101 gene). Without treatment, gigantism leads to a significantly increased adult height (often &gt;2.0\u20132.5 m) as well as serious metabolic and cardiovascular complications, significantly reducing life expectancy. Acromegaly, which is more common (prevalence 40\u201370 per million), is often diagnosed late (average delay of 7\u201310 years between initial symptoms and diagnosis) due to the insidious progression of morphological changes.\n  <\/div>\n\n  <h2>Etiologies, clinical presentation, and biological diagnosis<\/h2>\n  <ul class=\"co-list\">\n    <li><strong>Etiologies and genetic forms of gigantism and acromegaly:<\/strong> Sporadic somatotropic pituitary adenoma (&gt;95% of cases): microadenoma (&lt;10 mm\u2014more common in acromegaly) or macroadenoma (&gt;10 mm\u2014more common in pediatric gigantism) \u2014 autonomous GH secretion by tumor somatotrope cells \u2014 GNAS somatic mutation (Gs alpha protein \u2014 constitutive activation of adenylate cyclase): present in 40% of sporadic somatotropic adenomas (Gs-alpha+ adenomas) \u2014 smaller tumors + better response to somatostatin analogs; genetic forms and associated syndromes (10\u201315% of GH-secreting adenomas): MEN1 (multiple endocrine neoplasia type 1 \u2014 MENIN mutation \u2014 11q13): pituitary adenomas (GH + prolactin + corticotropes) + parathyroid adenomas + pancreatic endocrine tumors (insulinomas + gastrinomas) \u2192 routine screening for MEN1 in all young patients with GH-secreting pituitary adenomas; Carney syndrome (PRKAR1A mutation \u2014 17q24): GH-secreting pituitary adenomas + cardiac myxomas + cutaneous lentiginosis + pigmented adrenal nodules (PPNAD); XLAG syndrome (X-linked acrogigantism): duplication of the GPR101 gene on the X chromosome (Xq26.3) \u2192 pediatric gigantism with very early onset (before 2\u20134 years of age) + mixed GH\/prolactin pituitary macroadenoma \u2192 adult height often &gt;2 m; AIP (aryl hydrocarbon receptor interacting protein \u2014 11q13): germline mutations in AIP in 20% of familial somatotropin-secreting adenomas (FIPA \u2014 familial isolated pituitary adenoma) \u2014 invasive adenomas with early onset \u2014 poor response to somatostatin analogs; Rare causes of ectopic hypersecretion of GH or GHRH: carcinoid tumors (lung + pancreas) secreting GHRH (growth hormone-releasing hormone) \u2192 pituitary stimulation \u2192 somatotrope cell hyperplasia \u2192 very rare (&lt;1% of acromegaly cases)<\/li>\n    <li><strong>Clinical Presentation of Gigantism (Child) and Acromegaly (Adult):<\/strong> Gigantism (excessive GH secretion before cartilage fusion): accelerated height growth (growth velocity &gt;3 standard deviations for age) + progressive tall stature (often &gt;97th percentile from childhood onward + potential adult height &gt;2.0 m if untreated) + enlargement of hands and feet + progressive coarse facial features (bulging forehead + prognathism) + macroglossia + deep voice + hyperhidrosis + headaches + signs of pituitary tumor compression if macroadenoma (bitemporal hemianopia + oculomotor paralysis) + puberty: delayed or precocious puberty depending on the mass effect on the pituitary gland + fatigue + early joint pain; acromegaly (GH hypersecretion after cartilage fusion \u2014 adult): hand signs (ring sign\u2014increased ring size + rings needing to be resized) + increased shoe size + macroglossia + prognathism + dental diastema (gapped teeth) + bulging forehead (frontal bumps) + coarsening of facial features + hyperhidrosis + carpal tunnel syndrome (compression of the median nerve \u2014 present in 40\u201350% of acromegaly cases) + arthropathies + headaches (30\u201350% \u2014 mechanism not exclusively related to tumor size) + visual field defects if macroadenoma with chiasmatic compression + snoring + obstructive sleep apnea syndrome (OSAS \u2014 macroglossia + pharyngeal soft tissue hypertrophy \u2014 present in 60\u201380% of cases) + hypertension + cardiomegaly + cardiomyopathy + diabetes or glucose intolerance (insulin resistance induced by excess GH) + reddish-brown skin discoloration (melanotrophy) + acanthosis nigricans if marked insulin resistance; diagnostic delay for acromegaly: 7\u201310 years on average (progressive morphological changes largely unnoticed by close associates) \u2014 often discovered during evaluation for hypertension + diabetes + OSA + polyarthropathy + bilateral carpal tunnel syndrome + or during brain imaging performed for another reason<\/li>\n    <li><strong>Biological diagnosis and pituitary imaging :<\/strong> IGF-1 measurement (IGF-1 \u2014 insulin-like growth factor 1 \u2014 somatomedin C): first-line test \u2014 elevated in cases of chronic GH excess \u2014 interpretation based on age and sex (normal values vary by age \u2014 physiological peak at puberty + gradual decline with age) \u2014 elevated IGF-1 for age and sex is the best screening test for acromegaly\/gigantism \u2014 high PPV + normal IGF-1 virtually rules out the diagnosis except in cases of exclusively pulsatile secretion; GH suppression test using an oral glucose load (75 g OGTT \u2014 diagnostic gold standard): in a normal subject, the glucose load suppresses GH to &lt;0.4 ng\/mL (GH nadir) \u2014 in acromegaly\/gigantism: absence of GH suppression (nadir &gt;1 ng\/mL according to older criteria + nadir &gt;0.4 ng\/mL according to the current 2014 Endocrine Society criteria) or, paradoxically, an elevation in GH following glucose (paradoxical response \u2014 present in 10\u201320% of acromegalic patients) \u2014 GH samples at T0 + T30 + T60 + T90 + T120 min after glucose ingestion; Random basal GH: of limited use alone (pulsatile secretion) \u2014 useful if very high (&gt;20\u201330 ng\/mL) to quickly guide the diagnosis; Pituitary MRI with gadolinium: gold-standard morphological examination \u2014 T1 + T2 + FLAIR sequences + dynamic sequence after gadolinium injection \u2014 microadenoma: delayed contrast uptake compared to normal pituitary tissue + T1-hypointense signal \u2014 macroadenoma: mass &gt;10 mm + suprasellar extension + chiasmatic compression + cavernous sinus invasion (Knosp grades I\u2013IV) \u2014 fundus examination + visual field via automated perimetry: mandatory if macroadenoma + suprasellar extension \u2192 bitemporal hemianopia if optic chiasm compression; comprehensive pituitary workup (assessment of associated hypopituitarism \u2014 common with macroadenomas): cortisol + ACTH + Synacthen test (adrenal insufficiency) + TSH + T4L (central hypothyroidism) + FSH + LH + testosterone or estradiol (hypogonadism) + prolactin (hyperprolactinemia due to stalk effect or GH\/PRL co-secretion)<\/li>\n  <\/ul>\n\n  <h2>Treatment of Gigantism and Acromegaly<\/h2>\n  <table class=\"co-table\">\n    <colgroup><col style=\"width:200px;\"><col style=\"width:42%;\"><col><\/colgroup>\n    <thead>\n      <tr><th>Therapeutic modality<\/th><th>Technique, dosage, and mechanism<\/th><th>Results, complications, and follow-up<\/th><\/tr>\n    <\/thead>\n    <tbody>\n      <tr>\n        <td>Transsphenoidal surgery<br><small style=\"font-weight:400;color:#7a8fa0;\">1st line \u2014 endoscopy \u2014 tumor resection<\/small><\/td>\n        <td>Transsphenoidal surgery (TSS) is the first-line treatment for gigantism and acromegaly in the vast majority of cases\u2014it offers the possibility of immediate cure if the resection is complete; Surgical technique: endoscopic endonasal approach (current standard technique in most centers\u2014gradually replacing microsurgery under a microscope): access to the sphenoid sinus via the nasal fossae \u2192 opening of the anterior wall of the sella turcica \u2192 tumor resection under direct endoscopic visualization (4K high definition) + preoperative MRI-guided neuronavigation \u2192 closure of the sella (abdominal fat + biological glue + mucosal graft) \u2014 advantages vs. the microscopic approach: better visualization of blind spots + reduced complications (rhinorrhea + olfactory disturbances) + better control of lateral extensions; postoperative biochemical cure criteria (assessed at 3 months): GH nadir &lt;0.4 ng\/mL on the glucose tolerance test + age- and sex-normalized IGF-1 \u2014 cure rates according to tumor size: microadenoma: 70\u201385% % biochemical cure rate as first-line treatment \u2014 macroadenoma without cavernous sinus invasion (Knosp 0\u20132): 50\u201365% % \u2014 invasive macroadenoma (Knosp 3\u20134 \u2014 cavernous sinus invasion): 10\u201330% % (total resection virtually impossible if cavernous invasion \u2192 mandatory adjunctive medical therapy); preoperative preparation: preoperative somatostatin analogs (3\u20136 months): used if large macroadenoma + high anesthetic risk (severe OSA + cardiomyopathy) \u2192 reduction in tumor size (10\u201320 % on average) + improvement in cardiovascular comorbidities + improvement in OSA \u2192 no demonstrated benefit on surgical cure rates in meta-analyses \u2192 not routinely recommended preoperatively in all patients<\/td>\n        <td>Complications of transsphenoidal surgery: transient diabetes insipidus (10\u201315% of cases \u2014 ADH deficiency \u2192 massive polyuria + polydipsia \u2192 treatment with desmopressin \u2014 resolution within a few days to weeks \u2014 permanent in &lt;1% of cases) + cerebrospinal rhinorrhea (CSF leak through the sphenoid sinus \u2014 1\u20133% of cases \u2014 risk of meningitis \u2192 reoperation if persistent) + postoperative hypopituitarism (5\u201315% of cases \u2014 normal anteropituitary function intraoperatively \u2192 adrenal insufficiency + hypothyroidism + hypogonadism \u2192 complete pituitary workup 3 months postoperatively) + bacterial meningitis (rare \u2014 &lt;1 % \u2014 IV antibiotic therapy) + intrasellar hemorrhage + vascular injury (internal carotid artery \u2014 exceptional) + visual disturbances (worsening or onset of hemianopia due to traction on the chiasm \u2014 rare if careful technique is used); immediate postoperative follow-up: pituitary MRI 72 hours post-op (assessment of resection) + 3 months post-op (post-operative baseline MRI \u2014 before resorption of the packing fat) + hormonal workup on Day 3 (morning cortisol) + at 3 months (complete pituitary workup + IGF-1 + GH-stimulated pituitary function test) + systematic corticosteroid replacement therapy (hydrocortisone) in the first postoperative days (prevention of acute adrenal insufficiency); persistence or recurrence after surgery: tumor recurrence: annual MRI \u00d7 5 years then every 2\u20133 years if stable \u2014 biochemical recurrence: IGF-1 and GH-OGH annually for 10 years \u2192 medical treatment (ASN) or radiosurgery if recurrence is documented<\/td>\n      <\/tr>\n      <tr>\n        <td>Somatostatin analogues (SSA)<br><small style=\"font-weight:400;color:#7a8fa0;\">Octreotide \u2014 lanreotide \u2014 pasireotide<\/small><\/td>\n        <td>Somatostatin analogs (SSAs) are the first-line medical treatment for acromegaly and gigantism\u2014they bind to somatostatin receptors (SSTR\u2014primarily SSTR2 and SSTR5) expressed by tumor somatotrope cells \u2192 inhibition of GH secretion + reduction in tumor volume in 30\u201350% of cases; octreotide LP (Sandostatin LAR\u2014Novartis): extended-release formulation \u2014 10\u201340 mg deep IM (buttock) every 4 weeks \u2014 starting dose: 20 mg\/4 weeks \u2192 adjustment based on IGF-1 and GH \u2014 available in Canada + covered by RAMQ with exception criteria for acromegaly; lanreotide autogel (Somatuline Depot \u2014 Ipsen): pre-filled syringe in hydrogel formulation \u2014 60\u2013120 mg deep SC every 4 weeks (or every 6\u20138 weeks in well-controlled patients \u2014 dosing flexibility) \u2014 self-injection possible by the patient or home care nurse \u2014 non-inferiority to octreotide demonstrated \u2014 available in Canada + covered by RAMQ; pasireotide LAR (Signifor LAR \u2014 Recordati): second-generation analog \u2014 affinity for SSTR1 + SSTR2 + SSTR3 + SSTR5 (broader profile than octreotide and lanreotide, which primarily target SSTR2) \u2192 more effective on adenomas with little or no SSTR2 expression \u2014 40\u201360 mg IM every 4 weeks \u2014 PAOLA trial (Lacroix 2018): biochemical control (normalized IGF-1 and GH) in 15\u201320% of cases resistant to octreotide\/lanreotide \u2014 adverse effects: hyperglycemia ++ (present in 57\u201373% of patients \u2014 mechanism: inhibition of insulin secretion by pancreatic SSTR5) \u2192 close glycemic monitoring + metformin or other antidiabetics if diabetes is induced; side effects common to NSAIDs: gastrointestinal intolerance (nausea + cramps + diarrhea \u2014 transient at the start of treatment) + gallstones (25\u201330% of treated patients \u2014 mechanism: inhibition of gallbladder motility \u2192 biliary stasis) \u2192 annual gallbladder ultrasound<\/td>\n        <td>Efficacy of SSAs in acromegaly: biochemical control (normal IGF-1 + GH &lt;1 ng\/mL): 55\u201360% of patients on octreotide or lanreotide (Caron 2014 meta-analysis) \u2014 reduction in tumor volume: 10\u201320 % on average (reduction \u226520 % in 35\u201340 % of cases) \u2014 predictive factors for a good response to SSS: strong SSTR2 staining on immunohistochemistry (on surgical specimen) + GNAS+ (gs-alpha) mutation + microadenoma + lower baseline GH level; resistance to first-generation SSS (octreotide + lanreotide): 40\u201345% of patients \u2014 definition: Persistent IGF-1 &gt;1.3 \u00d7 ULN (upper limit of normal) after 3\u20136 months of treatment at maximum dose \u2192 options: dose escalation + switch to long-acting semaglutide (LAR) + addition of cabergoline + switch to pegvisomant; follow-up on SRS: IGF-1 + GH every 3 months (titration phase) \u2192 every 6 months (stable phase) + annual pituitary MRI (monitoring of residual tumor growth) + annual biliary ultrasound + fasting blood glucose + HbA1c (pasireotide); AS in pediatric acromegaly: limited data (small pediatric populations) \u2014 used post-surgery if residual tumor present + uncontrolled GH\/IGF-1 \u2014 effects on growth velocity: reduction but not cessation of growth if growth plates are still open \u2014 combination with GnRH analogs (to delay bone maturation) discussed in certain pediatric protocols<\/td>\n      <\/tr>\n      <tr>\n        <td>Pegvisomant and GH receptor antagonists<br><small style=\"font-weight:400;color:#7a8fa0;\">Somavert \u2014 AIDS resistance<\/small><\/td>\n        <td>Pegvisomant (Somavert \u2014 Pfizer) is a GH receptor antagonist \u2014 it binds to the GH receptor (GHR) without activating it \u2192 blocks GH signaling \u2192 reduces hepatic IGF-1 production \u2192 normalizes IGF-1 levels; unique mechanism of action (distinct from SASTs): pegvisomant does not act on the pituitary tumor itself \u2014 it blocks the peripheral effects of GH \u2192 IGF-1 decreases \u2192 serum GH may paradoxically increase under pegvisomant (absence of negative feedback from IGF-1 on the pituitary) \u2192 do not use GH as a monitoring marker during pegvisomant treatment (use IGF-1 exclusively); dosage: loading dose of 80 mg SC followed by 10\u201330 mg SC\/day \u2014 dose adjusted based on IGF-1 (target: IGF-1 within the normal range for age and sex); Efficacy: normalization of IGF-1 in 97% of patients treated at optimal doses (ACROSTUDY studies \u2014 international registry) \u2014 pegvisomant is the most effective treatment for normalizing IGF-1 in acromegaly \u2014 indications: resistance or intolerance to first- and second-generation SSS + combination with SSS (combined SSS + pegvisomant treatment): allows for a reduction in the pegvisomant dose (cost + frequency of injections) + improves control in patients partially controlled on SSS alone \u2014 SSS + pegvisomant combination therapy: positive data (Franck-Raue 2017 meta-analysis + ACROSTUDY registry); Adverse effects of pegvisomant: injection site reactions + elevated transaminases (5\u201310 times the upper limit of normal [ULN] \u2014 monthly liver function tests for the first 6 months, then every 6 months \u2014 discontinue if AST &gt;5\u00d7 ULN) + possible tumor growth (MRI monitoring \u2014 the absence of negative IGF-1 feedback may lift tumor growth inhibition in some cases \u2014 mandatory annual MRI monitoring) + lipodystrophy at the injection site (rotation of injection sites recommended); coverage in Canada: pegvisomant covered by most private insurance plans (high cost: 15,000\u201330,000 CAD\/year depending on the dose) + Pfizer Canada special access program if coverage is insufficient<\/td>\n        <td>Combination therapy with pegvisomant + ASS \u2014 clinical practice in Quebec: indications for the combination: acromegaly partially controlled with ASA alone (IGF-1 between 1.0 and 2.0 \u00d7 ULN) \u2014 large residual macroadenoma requiring tumor control (ASA) + optimal biochemical control (pegvisomant) \u2014 cost of the combination is lower than full-dose pegvisomant alone (reduction in daily pegvisomant dose by 30\u201350 %); comparison of medical strategies in post-surgical residual acromegaly: octreotide\/lanreotide alone: 55\u201360% biochemical control + long-acting pasireotide (LAR): 15\u201320% additional control in patients resistant to first-generation ASS + pegvisomant alone: 97 % of IGF-1 normalization + combination of ASS + pegvisomant: 90\u201395 % of IGF-1 control at reduced doses \u2014 individualized decision based on: side effect profile (diabetes \u2192 avoid pasireotide) + cost + adherence (daily pegvisomant injection vs. monthly ASS) + presence of residual tumor (ASS for tumor control); cabergoline (dopamine agonist \u2014 Dostinex): 3rd-line treatment for acromegaly \u2014 effective especially in cases of GH + prolactin co-secretion or D2R-expressing tumors \u2014 normalization of IGF-1 in 20\u201335% of patients (results from meta-analyses) \u2014 dosage: 0.5\u20133.5 mg\/week PO \u2014 well tolerated + low cost \u2014 attractive option for mild to moderate acromegaly or in combination with SSNIs<\/td>\n      <\/tr>\n      <tr>\n        <td>Radiotherapy and stereotactic radiosurgery<br><small style=\"font-weight:400;color:#7a8fa0;\">Gamma Knife \u2014 CyberKnife \u2014 adjuvant treatment<\/small><\/td>\n        <td>Radiotherapy and stereotactic radiosurgery are third-line treatment options for acromegaly and gigantism \u2014 they are reserved for patients with post-surgical residual tumor that is not controlled by medical treatment, or who are not candidates for further surgery; Stereotactic radiosurgery (Gamma Knife \u2014 Leksell Gamma Knife + CyberKnife \u2014 Accuray): delivery of a high-dose focused radiation in a single session (Gamma Knife) or in multiple fractions (CyberKnife \u2014 stereotactic fractionation) \u2014 principle: ablative dose to the residual tumor + maximum protection of adjacent structures (optic chiasm \u2014 dose limit 8 Gy + cranial nerves of the cavernous sinus) \u2014 condition: minimum distance of 3\u20135 mm between the residual tumor and the optic chiasm (otherwise conventional fractionated radiotherapy) \u2014 efficacy: biochemical control (normalization of IGF-1) in 40\u201360% of cases at 5\u201310 years \u2014 time to response: 2\u201310 years (slow \u2014 requires bridging medical therapy during the waiting period); fractionated conformal radiotherapy (IMRT \u2014 intensity-modulated radiation therapy): administered in 25\u201330 fractions over 5\u20136 weeks (total dose 45\u201354 Gy) \u2014 indicated if tumor residue is near the chiasm or infiltrating surrounding structures \u2192 second-line treatment compared to stereotactic radiosurgery \u2014 efficacy: biochemical control 50\u201360% at 10 years (Minniti meta-analysis 2011); proton therapy: available at a few centers in Canada (TRIUMF Vancouver) \u2192 irradiation with protons (Bragg peak \u2192 precise dose delivery) \u2192 reduction in irradiated surrounding structures + data on acromegaly similar to stereotactic radiosurgery; main complication of pituitary radiation therapy: radiation-induced hypopituitarism: adrenal insufficiency + hypothyroidism + hypogonadism \u2192 onset: 2\u201315 years after irradiation \u2192 cumulative incidence: 50\u201380% at 10 years \u2192 annual hormonal monitoring for life following irradiation<\/td>\n        <td>Indications for stereotactic radiosurgery in acromegaly in Quebec: visible post-surgical tumor residue on MRI + not controlled by medical treatment (somatostatin analogs + pegvisomant) + chiasm-tumor distance \u22653 mm (otherwise fractionated radiotherapy) + patient refusing or unable to benefit from a second surgery + residual invasive cavernous sinus macroadenoma (Knosp 3-4) after surgery + unreimbursed or intolerable medical treatment; Quebec stereotactic radiosurgery centers: CHUM (CyberKnife + Gamma Knife) + Montreal General Hospital (CUSM \u2014 Gamma Knife) + CHUS Sherbrooke + H\u00f4tel-Dieu de Qu\u00e9bec (CHU de Qu\u00e9bec); post-radiosurgery follow-up: IGF-1 + complete pituitary assessment every 6 months \u00d7 5 years + annual pituitary MRI \u00d7 5 years then every 2 years \u2014 medical treatments (somatostatin analogs + pegvisomant) must be maintained until biochemical evidence of post-radiosurgical cure (often 3-7 years after irradiation) \u2192 risk of rebound if premature cessation of somatostatin analogs; biochemical cure criteria after radiosurgery: normal IGF-1 for age and sex + GH nadir &lt;0.4 ng\/mL on OGTT (2 successive controls 6 months apart) \u2192 medical treatment withdrawal possible if these criteria are met + lifelong surveillance maintained (late recurrences documented up to 15-20 years post-irradiation)<\/td>\n      <\/tr>\n      <tr>\n        <td>Systemic complications and multidisciplinary follow-up<br><small style=\"font-weight:400;color:#7a8fa0;\">Cardiovascular \u2014 Metabolic \u2014 Quality of Life<\/small><\/td>\n        <td>Untreated or inadequately treated acromegaly and gigantism are associated with multiple systemic complications that significantly reduce life expectancy (mortality 2\u20133 times higher than in the general population in uncontrolled acromegaly) \u2014 their screening and management are an integral part of multidisciplinary follow-up; cardiovascular complications (leading cause of death in acromegaly): hypertension (30\u201340 mmHg \u2014 mechanism: sodium retention + activation of the renin-angiotensin-aldosterone system [RAAS] + functional hyperaldosteronism) \u2192 standard antihypertensive treatment \u2192 target BP &lt;130\/80 mmHg + cardiomegaly (acromegalic cardiomyopathy): thickening of the septum + posterior wall (concentric hypertrophy) \u2192 diastolic dysfunction \u2192 heart failure \u2192 TEE (transthoracic echocardiogram) at the start of follow-up + every 2\u20133 years + arrhythmias (atrial fibrillation + ventricular tachycardias) + annual ECG; metabolic complications: diabetes mellitus or glucose intolerance (30\u201350% of acromegalic patients \u2014 insulin resistance induced by GH antagonism on insulin sensitivity + inhibition of insulin secretion by pasireotide) \u2192 fasting blood glucose + HbA1c every 6 months + HOMA-IR \u2192 metformin \u00b1 insulin depending on severity; obstructive sleep apnea syndrome (OSAS): 60\u201380% of acromegaly patients \u2014 mechanism: macroglossia + pharyngeal soft tissue hypertrophy + craniofacial deformity \u2192 polysomnography if snoring + daytime sleepiness + CPAP if OSA confirmed \u2192 partial improvement with medical treatment (reduction in macroglossia); colorectal cancer: 2\u20133 times increased risk (frequent adenomatous polyposis due to IGF-1 hypersecretion \u2192 colonocyte growth factor) \u2192 colonoscopy upon diagnosis + every 5 years if active acromegaly + every 10 years if controlled; Acromegalic arthropathies: thickened articular cartilage + ligament laxity + early osteoarthritis (knees + hips + spine) \u2192 physical therapy + NSAIDs + analgesics \u2192 joint replacement if severe, disabling osteoarthritis<\/td>\n        <td>Multidisciplinary care for acromegaly and gigantism in Quebec: multidisciplinary team: endocrinologist (coordination + medical treatment) + pituitary neurosurgeon (transsphenoidal surgery) + neuroradiologist (high-resolution pituitary MRI) + radiation oncologist (stereotactic radiosurgery) + ophthalmologist (visual field + fundus examination) + cardiologist (echocardiogram + cardiovascular monitoring) + pulmonologist\/sleep specialist (OSAS + polysomnography) + gastroenterologist (colonoscopy) + rheumatologist (arthropathies) + ENT specialist (macroglossia + snoring) + maxillofacial surgeon (severe prognathism - orthognathic surgery if acromegaly is controlled) + psychologist (psychosocial impact + quality of life) + coordinating nurse; Quebec reference centers: Centre de neuroendocrinologie hypophysaire du CHUM (pituitary tumors) + CHU Sainte-Justine (pediatric gigantism) + CHUS Sherbrooke + CHU de Qu\u00e9bec; patient associations: Acrom\u00e9galie Qu\u00e9bec + Acromegaly Community (international) + Cushing's Support and Research Foundation (for pituitary tumors); quality of life in acromegaly: even in biochemical remission, many patients report altered quality of life (chronic fatigue + joint pain + cognitive disorders + depression + morphological sequelae) \u2192 psychological support + physical rehabilitation + validated questionnaires (AcroQoL \u2014 Acromegaly Quality of Life Questionnaire) at diagnosis + annual follow-up \u2192 adaptation of the therapeutic plan based on functional impact.<\/td>\n      <\/tr>\n    <\/tbody>\n  <\/table>\n\n  <div class=\"co-infobox\">\n    <span class=\"ico\">\u2139\ufe0f<\/span>\n    <span><strong>Pediatric Gigantism \u2014 XLAG Syndrome and Early Diagnosis:<\/strong> XLAG (X-linked acrogigantism) syndrome, linked to a duplication of the GPR101 gene on the X chromosome, is the best-characterized genetic cause of very early-onset gigantism (before 2-4 years of age). These children present with explosive statural growth, macrocephaly and a mixed GH\/prolactin pituitary macroadenoma that is often large at diagnosis. In any child presenting an abnormal statural acceleration with a growth rate exceeding +3 DS for age, an IGF-1 assay and pituitary MRI should be carried out without delay. Early diagnosis is crucial: every year of delay in diagnosis translates into several centimetres of irreversible excessive statural growth.<\/span>\n  <\/div>\n\n  <div class=\"co-urgence\">\n    <div class=\"co-urgence-titre\">Situations requiring urgent medical assessment<\/div>\n    <p><strong>Sudden visual deficit (hemianopsia) + intense headaches in a patient with known or suspected acromegaly<\/strong> \u2192 pituitary apoplexy (intratumoral hemorrhage) \u2192 emergency brain MRI + neurosurgery + IV corticosteroids (acute corticotropic insufficiency).<\/p>\n    <p><strong>Child with statural growth velocity &gt;+3 SD + acromegaly (large hands + feet) + headaches<\/strong> \u2192 gigantism on pituitary adenoma \u2192 IGF-1 urgent + pituitary MRI + pediatric endocrinology + neurosurgery opinion.<\/p>\n    <p><strong>Postoperative transsphenoidal surgery: polyuria &gt;3 L\/24h + polydipsia + very dilute urine<\/strong> \u2192 postoperative central diabetes insipidus \u2192 desmopressin (DDAVP) SC or nasal + ionogram + urinary and plasma osmolality + endocrinology.<\/p>\n    <p><strong>Known acromegaly + decompensated heart failure + severe OSA + uncontrolled HTN<\/strong> \u2192 severe cardiovascular complications of uncontrolled acromegaly \u2192 hospitalization + cardioprotection + optimization of anti-GH therapy + polysomnography + CPAP.<\/p>\n  <\/div>\n\n  <h2>Consult at Clinique Omicron<\/h2>\n  <p>Clinique Omicron physicians evaluate patients presenting with signs suggestive of gigantism or acromegaly (increased hand or foot size in adults, facial feature changes, prognathism, bilateral carpal tunnel syndrome, persistent headaches), order IGF-1 levels, and refer to the appropriate pituitary neuroendocrinology center. Follow-up for comorbidities (hypertension, diabetes, sleep apnea) in treated patients is managed in coordination with specialized teams at several service points in Quebec. To make an appointment, visit <a href=\"https:\/\/cliniqueomicron.ca\">cliniqueomicron.ca<\/a>.<\/p>\n\n  <p class=\"co-disclaimer\">The content of this page is for informational purposes only and does not replace the advice of an endocrinologist or neurosurgeon specializing in pituitary tumors. The management of gigantism and acromegaly requires an experienced multidisciplinary team.<\/p>\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Gigantisme et acrom\u00e9galie : causes, diagnostic et traitement | Clinique Omicron Endocrinologie &amp; Neurochirurgie &amp; M\u00e9decine de famille Gigantisme et acrom\u00e9galie Le gigantisme est un syndrome d&rsquo;exc\u00e8s de s\u00e9cr\u00e9tion d&rsquo;hormone de croissance (GH \u2014 growth hormone) survenant avant la fusion des cartilages de croissance \u00e9piphysaires, entra\u00eenant une croissance staturale excessive. Lorsque l&rsquo;hypers\u00e9cr\u00e9tion de GH survient&hellip;&nbsp;<a href=\"https:\/\/cliniqueomicron.ca\/en\/gigantisme\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Gigantism and acromegaly: causes, diagnosis and treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"Gigantisme et acrom\u00e9galie : | Brossard | Clinique Omicron","_metasync_otto_description":"Le gigantisme est caus\u00e9 par un exc\u00e8s d'hormone de croissance avant la fusion des cartilages. 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