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endo.pheochromocytoma.v1

Pheochromocytoma / paraganglioma

endocrinologychronicacutesubacuteadultpediatricoutpatientacuteinpatienttransition

Pheochromocytoma — α-blockade BEFORE β-blockade is the cardinal rule (10–14 d preop). Plasma fractionated metanephrines preferred screening; CT/MRI for localisation; MIBG/DOTATATE for metastatic / extra-adrenal. 30–40% heritable — genetic counselling and family screening mandatory (RET, VHL, NF1, SDHx). Open: manifest, problem-package, RxCUI verification, GAPP-PASS staging absent, tests.

Entry points (5)

  • symptom
    Classic triad — paroxysmal headache + palpitations + diaphoresis (ADA 2026)
    pheo_triad
  • symptom
    Paroxysmal HTN or resistant HTN (ADA 2026)
    paroxysmal_or_resistant_htn
  • imaging
    Adrenal incidentaloma >4 cm or imaging features (high HU, intense uptake) (ADA 2026)
    adrenal_incidentaloma_with_features
  • history
    Family history MEN2 / VHL / NF1 / SDHx (ADA 2026)
    family_history_men2_vhl_nf1_sdhx
  • symptom
    Unexplained Takotsubo / catecholamine cardiomyopathy (ADA 2026)
    unexplained_takotsubo_or_HF

Required inputs (15)

  • agerequired
    demographic • used at CONTEXT
    Pediatric pheo more often hereditary (ADA 2026)
  • sbprequired
    vital • used at CONTEXT
    Crisis recognition + preop target (ADA 2026)
  • dbprequired
    vital • used at CONTEXT
    Crisis recognition (ADA 2026)
  • hrrequired
    vital • used at CONTEXT
    Tachycardia drives β-blocker timing (ADA 2026)
  • plasma_or_urine_metanephrinesrequired
    lab • used at INITIAL_WORKUP
    Screening test; both forms acceptable (ADA 2026)
  • cbcrequired
    lab • used at INITIAL_WORKUP
    Baseline; pre-op (ADA 2026)
  • cmp_glucoserequired
    lab • used at INITIAL_WORKUP
    Hyperglycemia common (catecholamine effect) (ADA 2026)
  • creatinine_egfrrequired
    lab • used at INITIAL_WORKUP
    Imaging contrast; drug dosing (ADA 2026)
  • genetic_panel
    lab • used at BRANCHING_WORKUP
    30–40% heritable; specific syndromes shape surveillance (ADA 2026)
  • adrenal_ct_or_mrirequired
    imaging • used at INITIAL_WORKUP
    Localisation (ADA 2026)
  • mibg_or_dotatate_pet
    imaging • used at BRANCHING_WORKUP
    Metastatic / extra-adrenal / multifocal (ADA 2026)
  • echo_for_cardiomyopathy
    imaging • used at BRANCHING_WORKUP
    Catecholamine cardiomyopathy assessment (ADA 2026)
  • symptom_paroxysm_patternrequired
    history • used at CONTEXT
    Diagnostic timing (ADA 2026)
  • meds_or_foods_interferingrequired
    history • used at CONTEXT
    TCAs, MAOIs, sympathomimetics, levodopa, labetalol affect metanephrines (ADA 2026)
  • current_medsrequired
    medication • used at CONTEXT
    Drug interferences + preop sequencing (ADA 2026)

12-phase flow (12)

  1. 1FRAME
    Confirm pheo / paraganglioma via fractionated metanephrines; rule out interfering drugs / mimics (ADA 2026)
    inputs: plasma_or_urine_metanephrines
    advance: Biochemical confirmation
  2. 2ENTRY
    Triad / paroxysmal / resistant HTN / adrenal mass / family history / Takotsubo (ADA 2026)
    inputs: age
    advance: Engine entered
  3. 3CONTEXT
    Symptom paroxysm pattern, drugs and foods interfering, comorbidities (ADA 2026)
    inputs: symptom_paroxysm_pattern, meds_or_foods_interfering, current_meds
    advance: Context complete
  4. 4RED_FLAGS
    Hypertensive crisis, MI, stroke, Takotsubo, cardiomyopathy (ADA 2026)
    inputs: sbp, hr
    actions: htn_emergency, aortic_dissection
    advance: Stabilised
  5. 5INITIAL_WORKUP
    Plasma fractionated metanephrines (or 24-h urine), CMP, glucose, lipid, CBC, ECG, CT/MRI adrenal (ADA 2026)
    inputs: plasma_or_urine_metanephrines, cmp_glucose, creatinine_egfr, cbc, adrenal_ct_or_mri
    actions: panel.hormone, panel.cardiac, specialty.pheochromocytoma
    advance: Localised
  6. 6BRANCHING_WORKUP
    MIBG / DOTATATE PET if metastatic suspected; genetic testing; cardiomyopathy assessment (ADA 2026)
    inputs: mibg_or_dotatate_pet, genetic_panel, echo_for_cardiomyopathy
    advance: Branch resolved
  7. 7DIFFERENTIAL
    Pheo vs paraganglioma vs cocaine / TCA / MAOI / clonidine withdrawal vs panic vs hyperthyroid vs carcinoid (ADA 2026)
    advance: Diagnosis confirmed
  8. 8RISK_STRATIFICATION
    Tumour size, location, metastasis, genetics; perioperative risk (ADA 2026)
    inputs: adrenal_ct_or_mri
    advance: Tier documented
  9. 9TREATMENT
    α-blockade BEFORE β; phenoxybenzamine 10 mg BID titrate to BP <130/80 + postural; or doxazosin/prazosin; β only after α if tachycardia persists; high Na diet + IV fluids preop; laparoscopic adrenalectomy; metastatic — MIBG, sunitinib, CVD chemo, Lu-DOTATATE (ADA 2026)
    inputs: sbp, hr, creatinine_egfr
    advance: Plan documented
  10. 10DISPOSITION
    Outpatient endo if stable; admit if crisis or preop optimisation (ADA 2026)
    advance: Disposition documented
  11. 11MONITORING
    BP home log, metanephrines post-op, recurrence surveillance, genetic counselling (ADA 2026)
    advance: Schedule documented
  12. 12FOLLOWUP
    Endo + surgery + genetics; lifelong annual surveillance (ADA 2026)
    advance: Follow-up booked