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endo.acromegaly.core.v1PRODUCTION
endo.acromegaly.core.v1

Acromegaly

endocrinologychronicadult
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Recognize acromegaly as an insidious, often years-delayed GH-excess syndrome whose untreated course carries ~2× excess mortality; goal = biochemical control + comorbidity reversal (Acromegaly Consensus 2020)

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Clinical suspicion framed; population/setting fixed

Patient inputs (14)

Localizes adenoma; macroadenoma common; defines cavernous-sinus invasion, optic chiasm compression, surgical resectability

IGF-1 reference is age/sex-adjusted; young/aggressive disease triggers MEN1/AIP/X-LAG/Carney genetic screen; pre-epiphyseal onset = gigantism

IGF-1 reference range is sex-specific; estrogen status affects GH/IGF-1 axis interpretation

Insidious acral/facial change, ring/shoe size progression, hyperhidrosis, macroglossia define the phenotype; old photos anchor onset (often 7–10 yr lag to diagnosis)

MEN1 (hyperparathyroidism/pancreatic NET), AIP / familial isolated pituitary adenoma, X-LAG (infantile gigantism), Carney complex stigmata

Best single screening test — age/sex-adjusted IGF-1 elevation is the entry biochemical criterion and the remission target

Confirmatory — failure of GH suppression to <0.4 µg/L (modern assay; legacy <1 µg/L) on 75 g OGTT confirms autonomous GH secretion

Prolactin (co-secretion / stalk effect), TSH+FT4, 0800 cortisol/ACTH, LH/FSH/testosterone or estradiol — screen mass-effect hypopituitarism

Impaired glucose tolerance / T2DM is a core comorbidity and OGTT-confounding factor; pasireotide worsens it

Macroadenoma with suprasellar extension threatens chiasm — bitemporal hemianopia mandates expedited surgery

OSA in 60–80%; soft-tissue + macroglossia; perioperative airway risk and CV mortality contributor

Estrogen/somatostatin/dopaminergic agents alter IGF-1; baseline before SRL/pegvisomant; QT/diabetes interactions

Markedly elevated random GH supports diagnosis and is a post-treatment remission/cure surrogate (random GH <1 µg/L)

Acromegalic cardiomyopathy (biventricular hypertrophy, diastolic then systolic dysfunction) drives excess mortality

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationallife_threateningacromegalic_cardiomyopathy_chf
    Decompensated acromegalic cardiomyopathy / CHF — biventricular hypertrophy with systolic dysfunction, pulmonary congestion, or malignant arrhythmia (Acromegaly Consensus 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningvisual_loss_mass_effect
    Progressive visual field loss / bitemporal hemianopia from macroadenoma compressing the optic chiasm (Acromegaly Consensus 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpituitary_apoplexy_gh_adenoma
    Pituitary apoplexy in a GH-secreting adenoma — thunderclap headache, acute ophthalmoplegia/visual loss, ± hemodynamic collapse from acute ACTH deficiency (Acromegaly Consensus 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_osa_hypoventilation
    Severe OSA with daytime hypoventilation / hypoxemia or perioperative airway risk in acromegaly (Acromegaly Consensus 2018)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepasireotide_severe_hyperglycemia
    Pasireotide-induced severe hyperglycemia / hyperglycemic crisis (Acromegaly Consensus 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepegvisomant_hepatotoxicity
    Transaminases >3× ULN on pegvisomant (Endocrine Society 2014)
    Trigger could not be auto-evaluated — needs clinician judgement.

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RED_FLAGSoptionalDrives severity classification
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Recommended regimen

Acromegaly — surgery-first ladder → SRL → pegvisomant → cabergoline → radiotherapy → comorbidity bundle (Acromegaly Consensus 2020)
axis: acromegaly_surgery_first_ladderstep 1 - Step 1 — Transsphenoidal surgery (FIRST-LINE, high-volume centre)
Selected step "Step 1 — Transsphenoidal surgery (FIRST-LINE, high-volume centre)" — Resectable somatotroph adenoma without prohibitive operative risk; goal = biochemical remission (normal age-matched IGF-1 + suppressed GH)
  • endoscopic transsphenoidal adenomectomy
    first line
    pituitary_surgery
    surgical • once (definitive)
    triggers: resectable_adenoma, surgical_candidate, visual_threat
    Endocrine Society 2014 / Consensus 2020 — first-line; cure rate higher for microadenoma and non-invasive macroadenoma at experienced centres; debulking improves subsequent medical response

outpatient playbook — drug actions (4)

  1. 1. octreotide LAR or lanreotide depot
    octreotide LAR 20 mg IM q4 wk OR lanreotide 90 mg SC q4 wk, titrate by IGF-1 • IM/SC • q4 weeks
    trigger: Not biochemically cured post-op or unresectable (Acromegaly Consensus 2020)
    First medical therapy — IGF-1 control + tumour shrinkage (Endocrine Society 2014)
  2. 2. pasireotide LAR
    40 mg IM q4 wk, up to 60 mg • IM • q4 weeks
    trigger: First-generation SRL resistant (Acromegaly Consensus 2020)
    Broader SSTR coverage; monitor hyperglycemia (Acromegaly Consensus 2020)
  3. 3. pegvisomant
    10 mg SC daily load then titrate by IGF-1 • SC • daily
    trigger: IGF-1 not normalized on SRL (Endocrine Society 2014)
    Best IGF-1 normalization; LFT + tumour MRI surveillance (Endocrine Society 2014)
  4. 4. cabergoline
    0.5 mg PO twice weekly, titrate • PO • 2× weekly
    trigger: Mild elevation or co-secreting prolactin (Endocrine Society 2014)
    Oral adjunct, modest effect (Endocrine Society 2014)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Enlarging hands/feet (ring + shoe size up), coarse facial features, prognathism, frontal bossing, macroglossia, hyperhidrosis, oily skin (Endocrine Society 2014); New large-joint arthralgia, bilateral carpal tunnel, snoring/witnessed apnea, headache, visual change (Acromegaly Consensus 2018/2020); Age/sex-adjusted IGF-1 above reference range on routine or workup labs (Endocrine Society 2014).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Acromegaly** (endo.acromegaly.core.v1).
Phenotype framing: Somatotroph macro/microadenoma vs mixed GH/PRL adenoma vs ectopic GHRH (chest/abdomen NET) vs ectopic GH (rare); MEN1/AIP/X-LAG/Carney syndromic forms; pseudo-acromegaly (insulin-mediated, minoxidil, severe insulin resistance) excluded biochemically (Endocrine Society 2014)
Scope: Recognize acromegaly as an insidious, often years-delayed GH-excess syndrome whose untreated course carries ~2× excess mortality; goal = biochemical control + comorbidity reversal (Acromegaly Consensus 2020)

No severity triggers fired against current inputs.

Plan

Regimen axis: **Acromegaly — surgery-first ladder → SRL → pegvisomant → cabergoline → radiotherapy → comorbidity bundle (Acromegaly Consensus 2020)** — step "Step 1 — Transsphenoidal surgery (FIRST-LINE, high-volume centre)".
1. endoscopic transsphenoidal adenomectomy surgical once (definitive) (pituitary_surgery, first line) — Endocrine Society 2014 / Consensus 2020 — first-line; cure rate higher for microadenoma and non-invasive macroadenoma at experienced centres; debulking improves subsequent medical response

Setting playbook (outpatient) — Confirm biochemical diagnosis, localize tumour, refer for transsphenoidal surgery, start/titrate medical therapy if not cured, run lifelong comorbidity + surveillance bundle (Acromegaly Consensus 2020)
2. octreotide LAR or lanreotide depot octreotide LAR 20 mg IM q4 wk OR lanreotide 90 mg SC q4 wk, titrate by IGF-1 IM/SC q4 weeks — Not biochemically cured post-op or unresectable (Acromegaly Consensus 2020) (First medical therapy — IGF-1 control + tumour shrinkage (Endocrine Society 2014))
3. pasireotide LAR 40 mg IM q4 wk, up to 60 mg IM q4 weeks — First-generation SRL resistant (Acromegaly Consensus 2020) (Broader SSTR coverage; monitor hyperglycemia (Acromegaly Consensus 2020))
4. pegvisomant 10 mg SC daily load then titrate by IGF-1 SC daily — IGF-1 not normalized on SRL (Endocrine Society 2014) (Best IGF-1 normalization; LFT + tumour MRI surveillance (Endocrine Society 2014))
5. cabergoline 0.5 mg PO twice weekly, titrate PO 2× weekly — Mild elevation or co-secreting prolactin (Endocrine Society 2014) (Oral adjunct, modest effect (Endocrine Society 2014))

Non-pharmacologic actions:
- Neurosurgery referral for elective transsphenoidal adenomectomy (Acromegaly Consensus 2020)
- Stereotactic radiotherapy referral for residual/recurrent tumour (Acromegaly Consensus 2020)
- CPAP for confirmed OSA (Acromegaly Consensus 2018)
- Baseline + surveillance colonoscopy (Acromegaly Consensus 2020)
- Cardiac, glucose, BP, bone/joint optimization; pituitary MDT (Acromegaly Consensus 2020)

AVOID / contraindication checks:
- Pasireotide hyperglycemia caution baseline and serial glucose A1c (Acromegaly Consensus 2020)
- Pegvisomant LFT monitoring baseline then serial hold if transaminases >3xULN (Endocrine Society 2014)
- Pegvisomant not tumour directed require interval MRI for tumour growth (Acromegaly Consensus 2020)
- SRL gallstone risk baseline and symptomatic gallbladder US (Endocrine Society 2014)
- Radiotherapy late hypopituitarism lifelong axis surveillance (Acromegaly Consensus 2020)
- Cabergoline high cumulative dose valvulopathy awareness (Endocrine Society 2014)

Monitoring

Regimen monitoring:
- IGF-1 age/sex-adjusted (± random/nadir GH) ~12 wk post-op then per therapy (Endocrine Society 2014)
- LFTs baseline then serial on pegvisomant (Endocrine Society 2014)
- Glucose + A1c baseline then serial, intensified on pasireotide (Acromegaly Consensus 2020)
- Pituitary MRI periodic — more frequent on pegvisomant and post-radiotherapy (Acromegaly Consensus 2020)
- Surveillance colonoscopy baseline then interval (Acromegaly Consensus 2020)
- Echocardiogram periodic for acromegalic cardiomyopathy (Acromegaly Consensus 2020)
- Gallbladder ultrasound on SRL therapy (Endocrine Society 2014)
- Sleep study for OSA at diagnosis and after biochemical control (Acromegaly Consensus 2018)

Setting (outpatient) monitoring:
- IGF-1 ± random/nadir GH per therapy interval (Endocrine Society 2014)
- Glucose/A1c, LFTs (on pegvisomant), gallbladder US (on SRL) (Acromegaly Consensus 2020)
- Pituitary MRI periodically; visual fields if macroadenoma (Acromegaly Consensus 2020)

Follow-up plan: Lifelong endocrine surveillance — biochemical relapse, hypopituitarism replacement, comorbidity reassessment (colonoscopy interval, cardiac, OSA, bone/joint), tumour-regrowth MRI; pituitary MDT for recurrence (Acromegaly Consensus 2020)
- Close-out criterion: Lifelong follow-up + surveillance plan booked

Monitoring phase: IGF-1 (± random/nadir GH) ~12 wk post-op then per therapy; on pegvisomant LFTs + IGF-1 + interval MRI for tumour growth; on SRL/pasireotide glucose/A1c + gallbladder US; periodic echo, sleep study, BP, colonoscopy interval, thyroid US (Acromegaly Consensus 2018/2020)

Disposition

Current setting: outpatient — Confirm biochemical diagnosis, localize tumour, refer for transsphenoidal surgery, start/titrate medical therapy if not cured, run lifelong comorbidity + surveillance bundle (Acromegaly Consensus 2020)

Disposition criteria:
- Continue ambulatory diagnostic/surgical pathway and titration; surveillance lifelong (Acromegaly Consensus 2020)

Escalation triggers (move to higher acuity):
- New/worsening visual field loss or acute headache → urgent imaging + neurosurgery (apoplexy pathway) (Acromegaly Consensus 2020)
- Decompensated cardiomyopathy/CHF or severe OSA hypoventilation → admit (Acromegaly Consensus 2020)
- Pasireotide-induced severe hyperglycemia/DKA-range → urgent glucose management (Acromegaly Consensus 2020)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Decompensated acromegalic cardiomyopathy / CHF — biventricular hypertrophy with systolic dysfunction, pulmonary congestion, or malignant arrhythmia (Acromegaly Consensus 2020)
- [LIFE_THREATENING] Progressive visual field loss / bitemporal hemianopia from macroadenoma compressing the optic chiasm (Acromegaly Consensus 2020)
- [LIFE_THREATENING] Pituitary apoplexy in a GH-secreting adenoma — thunderclap headache, acute ophthalmoplegia/visual loss, ± hemodynamic collapse from acute ACTH deficiency (Acromegaly Consensus 2020)

Citations

- 2014 Endocrine Society Acromegaly Guideline (Katznelson) + 2018/2020 Acromegaly Consensus (Giustina, Pituitary Society); 2021-2025 updates [PMID:25356808](https://pubmed.ncbi.nlm.nih.gov/25356808/)
- Cited evidence (PMID 30050156) [PMID:30050156](https://pubmed.ncbi.nlm.nih.gov/30050156/)
- Cited evidence (PMID 31606735) [PMID:31606735](https://pubmed.ncbi.nlm.nih.gov/31606735/)
- Cited evidence (PMID 24423324) [PMID:24423324](https://pubmed.ncbi.nlm.nih.gov/24423324/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2014 Endocrine Society Acromegaly Guideline (Katznelson) + 2018/2020 Acromegaly Consensus (Giustina, Pituitary Society); 2021-2025 updates — PMID:25356808
  • Cited evidence (PMID 30050156) — PMID:30050156
  • Cited evidence (PMID 31606735) — PMID:31606735
  • Cited evidence (PMID 24423324) — PMID:24423324