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endo.acromegaly.core.v1

Acromegaly

endocrinologychronicadultoutpatientinpatient
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Acromegaly dossier — GH-secreting pituitary adenoma; gigantism if pre-epiphyseal. Two-step biochemical diagnosis: elevated age/sex-adjusted IGF-1 + failure of GH suppression on 75 g OGTT (modern ultrasensitive-assay nadir cutoff ~<0.4 µg/L; legacy <1 µg/L). Transsphenoidal surgery first-line at a high-volume centre; SRL (octreotide LAR/lanreotide), pasireotide for SRL-resistant (hyperglycemia caution), pegvisomant (LFT + tumour MRI surveillance), cabergoline adjunct, stereotactic radiotherapy for residual. Excess mortality driven by cardiomyopathy/CHF, arrhythmia, HTN, OSA, glucose intolerance, colon neoplasia — aggressive parallel comorbidity bundle + lifelong surveillance. Screen MEN1/AIP/X-LAG/Carney if young/familial; rare ectopic GHRH NET when MRI negative with biochemical disease. Manifest pointer is a PLACEHOLDER (reuses endo.cushing_syndrome.v1 manifest) — no acromegaly manifest/problem-package on disk yet. Open: dedicated manifest + problem-package, RxNav RxCUI verification (none embedded), acromegaly-specific workup/calculator ids absent (mapped to whitelisted hypopituitarism/men_screening/thyroid_nodule/apoplexy + NEWS2/qSOFA), Bayesian LRs deferred.

Entry points (5)

  • symptom
    Enlarging hands/feet (ring + shoe size up), coarse facial features, prognathism, frontal bossing, macroglossia, hyperhidrosis, oily skin (Endocrine Society 2014)
    acral_facial_coarsening
  • symptom
    New large-joint arthralgia, bilateral carpal tunnel, snoring/witnessed apnea, headache, visual change (Acromegaly Consensus 2018/2020)
    arthralgia_carpal_tunnel_osa
  • lab_abnormality
    Age/sex-adjusted IGF-1 above reference range on routine or workup labs (Endocrine Society 2014)
    elevated_igf1
  • imaging
    Incidental pituitary macroadenoma / sellar mass on imaging (Acromegaly Consensus 2020)
    incidental_pituitary_macroadenoma
  • problem_list
    Young/unexplained cluster: T2DM + resistant HTN + cardiomyopathy + OSA + colon polyps (Acromegaly Consensus 2020)
    unexplained_t2dm_htn_cardiomyopathy_cluster

Required inputs (14)

  • agerequired
    demographic • used at CONTEXT
    IGF-1 reference is age/sex-adjusted; young/aggressive disease triggers MEN1/AIP/X-LAG/Carney genetic screen; pre-epiphyseal onset = gigantism
  • sexrequired
    demographic • used at CONTEXT
    IGF-1 reference range is sex-specific; estrogen status affects GH/IGF-1 axis interpretation
  • somatic_change_historyrequired
    symptom • used at CONTEXT
    Insidious acral/facial change, ring/shoe size progression, hyperhidrosis, macroglossia define the phenotype; old photos anchor onset (often 7–10 yr lag to diagnosis)
  • igf1required
    lab • used at INITIAL_WORKUP
    Best single screening test — age/sex-adjusted IGF-1 elevation is the entry biochemical criterion and the remission target
  • ogtt_gh_nadirrequired
    lab • used at INITIAL_WORKUP
    Confirmatory — failure of GH suppression to <0.4 µg/L (modern assay; legacy <1 µg/L) on 75 g OGTT confirms autonomous GH secretion
  • random_gh
    lab • used at INITIAL_WORKUP
    Markedly elevated random GH supports diagnosis and is a post-treatment remission/cure surrogate (random GH <1 µg/L)
  • anterior_pituitary_panelrequired
    lab • used at INITIAL_WORKUP
    Prolactin (co-secretion / stalk effect), TSH+FT4, 0800 cortisol/ACTH, LH/FSH/testosterone or estradiol — screen mass-effect hypopituitarism
  • glucose_a1crequired
    lab • used at INITIAL_WORKUP
    Impaired glucose tolerance / T2DM is a core comorbidity and OGTT-confounding factor; pasireotide worsens it
  • pituitary_mrirequired
    imaging • used at BRANCHING_WORKUP
    Localizes adenoma; macroadenoma common; defines cavernous-sinus invasion, optic chiasm compression, surgical resectability
  • formal_visual_fields
    imaging • used at BRANCHING_WORKUP
    Macroadenoma with suprasellar extension threatens chiasm — bitemporal hemianopia mandates expedited surgery
  • echocardiogram
    imaging • used at RISK_STRATIFICATION
    Acromegalic cardiomyopathy (biventricular hypertrophy, diastolic then systolic dysfunction) drives excess mortality
  • family_or_syndromic_featuresrequired
    history • used at CONTEXT
    MEN1 (hyperparathyroidism/pancreatic NET), AIP / familial isolated pituitary adenoma, X-LAG (infantile gigantism), Carney complex stigmata
  • osa_screen
    history • used at CONTEXT
    OSA in 60–80%; soft-tissue + macroglossia; perioperative airway risk and CV mortality contributor
  • current_meds
    medication • used at CONTEXT
    Estrogen/somatostatin/dopaminergic agents alter IGF-1; baseline before SRL/pegvisomant; QT/diabetes interactions

12-phase flow (12)

  1. 1FRAME
    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)
    inputs: age, somatic_change_history
    advance: Clinical suspicion framed; population/setting fixed
  2. 2ENTRY
    Acral/facial coarsening, arthralgia/carpal tunnel/OSA cluster, elevated IGF-1, incidental pituitary macroadenoma, or young unexplained T2DM+HTN+cardiomyopathy cluster (Endocrine Society 2014)
    inputs: age, sex
    advance: Engine entered via a recognized trigger
  3. 3CONTEXT
    Onset timeline (pre- vs post-epiphyseal → gigantism vs acromegaly), old-photo comparison, family/syndromic features (MEN1/AIP/X-LAG/Carney), OSA screen, current meds (Acromegaly Consensus 2018)
    inputs: somatic_change_history, family_or_syndromic_features, osa_screen, current_meds
    advance: Context, syndromic risk and comorbidity scope captured
  4. 4RED_FLAGS
    Acute mass effect / visual loss, pituitary apoplexy in a GH adenoma (thunderclap headache + ophthalmoplegia + visual loss ± adrenal crisis), decompensated acromegalic cardiomyopathy/CHF, severe OSA with hypoventilation (Acromegaly Consensus 2020)
    inputs: random_gh
    actions: workup.pituitary_apoplexy, calc.news2
    advance: Emergent mass-effect / cardiac / apoplexy screen completed and escalated if positive
  5. 5INITIAL_WORKUP
    Age/sex-adjusted IGF-1, 75 g OGTT GH nadir, random GH, full anterior pituitary panel (PRL, TSH/FT4, 0800 cortisol/ACTH, gonadal axis), glucose/A1c, metabolic + cardiac panels (Endocrine Society 2014)
    inputs: igf1, ogtt_gh_nadir, random_gh, anterior_pituitary_panel, glucose_a1c
    actions: panel.hormone, panel.glucose_a1c, panel.metabolic, panel.cardiac, panel.thyroid
    advance: IGF-1 elevated AND GH not suppressed on OGTT — biochemical diagnosis confirmed
  6. 6BRANCHING_WORKUP
    Pituitary MRI (size, cavernous-sinus invasion, chiasm), formal visual fields if macroadenoma; mass-effect hypopituitarism workup; if pituitary MRI negative with biochemical disease → serum GHRH + chest/abdomen imaging for ectopic GHRH NET; genetic testing if young/familial (Acromegaly Consensus 2020)
    inputs: pituitary_mri, formal_visual_fields
    actions: workup.hypopituitarism, workup.men_screening, panel.cbc
    advance: Tumour localized (or ectopic GHRH source identified) and pituitary reserve characterized
  7. 7DIFFERENTIAL
    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)
    advance: Etiology and syndromic context assigned
  8. 8RISK_STRATIFICATION
    Quantify systemic morbidity/mortality drivers: echo for acromegalic cardiomyopathy, BP, glucose/A1c, sleep study, colonoscopy (baseline + surveillance), thyroid US for nodules, DXA/vertebral fracture; tumour aggressiveness (size, invasion, granulation, resectability) (Acromegaly Consensus 2020)
    inputs: echocardiogram, glucose_a1c
    actions: calc.news2, panel.cardiac
    advance: Comorbidity burden + tumour aggressiveness staged; surgical candidacy and predicted remission documented
  9. 9TREATMENT
    Transsphenoidal surgery FIRST-LINE at a high-volume pituitary centre targeting biochemical remission. If not cured: first-generation SRL (octreotide LAR / lanreotide), pasireotide for SRL-resistant (monitor hyperglycemia), pegvisomant when IGF-1 not normalized (LFT + tumour surveillance), cabergoline modest adjunct; stereotactic radiotherapy for residual/recurrent. Treat comorbidities aggressively (cardiac, OSA, glucose, colonoscopy, bone/joint). Peri-op + pregnancy nuances (Acromegaly Consensus 2020; Endocrine Society 2014)
    inputs: pituitary_mri, igf1, glucose_a1c
    advance: Surgical and/or medical/radiotherapy plan + comorbidity bundle documented
  10. 10DISPOSITION
    Outpatient endocrinology + neurosurgery referral for elective transsphenoidal surgery; admit for surgery, decompensated cardiomyopathy/CHF, severe OSA with hypoventilation, visual-threatening mass, apoplexy, or pasireotide-induced severe hyperglycemia (Acromegaly Consensus 2020)
    advance: Disposition and surgical pathway documented
  11. 11MONITORING
    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)
    inputs: igf1, glucose_a1c
    actions: panel.hormone, panel.glucose_a1c, panel.metabolic
    advance: Remission criteria assessed (normal age-matched IGF-1 + suppressed GH) and surveillance schedule set
  12. 12FOLLOWUP
    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)
    advance: Lifelong follow-up + surveillance plan booked