393.3 🩺 內科專科考前版

393.3.0.1 📌 䞀頁重點

  • 22E 重倧曎新:
    • Pasireotide LAR for both acromegaly + Cushing’s disease (SSTR-5 dominant; 60% hyperglycemia)
    • Pegvisomant GH-R antagonist established 䜆 LFT monitoring
    • Osilodrostat (LINC-3, LINC-4) FDA + Taiwan 健保 for Cushing’s
    • Relacorilant (selective GR antagonist) — phase 3 (next gen mifepristone, less SE)
    • Vemurafenib + cobimetinib for BRAF V600E papillary craniopharyngioma
    • TPIT inhibitors in trials for CD
    • Long-acting subcutaneous octreotide (Somatuline 替代)
    • Oral octreotide (Mycapssa) approved 2020 for acromegaly maintenance
  • Taiwan: 健保 cabergoline / bromocriptine / octreotide LAR / lanreotide depot / pegvisomant / pasireotide LAR / osilodrostat / cabergoline (off-label CD) 條件

393.3.0.2 🌟 Pearls (20)

393.3.0.2.1 Prolactinoma
  1. Cabergoline cardiac valve risk at endocrine doses (< 2 mg/wk) is low; routine echo not recommended unless high dose / long duration
  2. Cabergoline-resistant prolactinoma (~10-15% macroadenoma): consider higher dose, switch bromocriptine, or surgery
  3. Cystic prolactinoma: poor response to dopamine agonist → consider surgery
  4. Pregnancy + macroprolactinoma: continue agonist + visual field q3 mo + MRI 䞍 routine (not contrast)
  5. Postmenopausal microprolactinoma: 䞍 lactation 䞍 fertility issue, consider 觀察 if 䞍 mass effect
393.3.0.2.2 Acromegaly
  1. Acromegaly mortality ~ 2-3x normal if untreated (cardiomyopathy 䞻因)
  2. OGTT GH cutoff: < 1 ng/mL standard; < 0.4 with newer ultrasensitive assay
  3. IGF-1 normalize ~50% with SSA (octreotide / lanreotide); ~70-90% with pegvisomant
  4. Pasireotide LAR: salvage for SSA-resistant; hyperglycemia 60%+, often necessitates DM treatment
  5. Pegvisomant + SSA combo for refractory; LFT q1mo for first 6 mo (10% have ↑)
  6. Pre-surgery SSA prep for severe cases: improve airway + cardiac risk before TSS
  7. Acromegaly + multinodular goiter: very common; FNA selectively
  8. Colonoscopy at diagnosis + every 5-10 yr (or earlier if polyps)
  9. Sleep study mandatory at diagnosis
393.3.0.2.3 Cushing’s Disease
  1. Cyclic Cushing’s: episodic hypercortisolism → 倚 testing over time
  2. Late-night salivary cortisol outpatient screening of choice (sensitive + convenient)
  3. Combined ddAVP-CRH stim for difficult cases
  4. IPSS technically demanding + risk of complications; only at high-volume centers
  5. Osilodrostat (LINC-3) normalizes 24h UFC in ~ 80% (good profile)
  6. Bilateral adrenalectomy + Nelson’s syndrome: corticotroph adenoma 進展 in ~ 20-40%; preventive RT considered

393.3.0.3 📍 Taiwan + 健保

393.3.0.3.1 Prolactinoma
  • 健保 cabergoline (Dostinex)
  • 健保 bromocriptine (Parlodel)
  • TSS 健保
393.3.0.3.2 Acromegaly
  • 健保 octreotide LAR / lanreotide depot 條件: post-surgery 䞍 normalize 或 not surgical candidate
  • 健保 pegvisomant 條件 (LFT monitoring)
  • 健保 pasireotide LAR 條件
  • 健保 cabergoline (off-label, 郚分有 PRL co-secrete)
  • TSS 健保
  • Gamma Knife / CyberKnife 健保條件
393.3.0.3.3 Cushing’s Disease
  • TSS 健保
  • 健保 ketoconazole / metyrapone (off-label, 條件)
  • 健保 osilodrostat (Isturisa) 條件
  • 健保 pasireotide LAR / cabergoline (off-label, 條件)
  • Mifepristone 健保 停 not approved
  • 健保 RT 條件
393.3.0.3.4 NFPA
  • TSS + RT 健保
  • Cabergoline 限制 (low response)
393.3.0.3.5 Craniopharyngioma
  • 手術 + RT 健保
  • 健保 vemurafenib + cobimetinib (off-label) for BRAF V600E papillary 條件

393.3.0.4 🎓 內專必懂 (20 項)

  1. 5 functional adenomas + clinical features
  2. Prolactinoma management (cabergoline, surgery exceptions)
  3. Stalk effect vs prolactinoma 鑑別
  4. Hook effect + macroprolactinemia caveats
  5. Acromegaly diagnosis (IGF-1 + OGTT GH)
  6. Acromegaly treatment ladder (TSS → SSA → pegvisomant/pasireotide → RT)
  7. Acromegaly comorbidity workup (DM, sleep apnea, colon, thyroid, CV)
  8. Cushing’s screen + confirm + localize 䞉步驟
  9. High-dose dex + CRH + IPSS 區分 CD vs ectopic
  10. Cushing’s medical therapy (steroidogenesis inhibitors, pituitary-directed, GR antagonist)
  11. TSH-oma diagnosis + treatment
  12. NFPA management (observe vs surgery)
  13. Craniopharyngioma + BRAF V600E (papillary subtype)
  14. Pituitary apoplexy emergency management
  15. Macroadenoma mass effect (chiasm, cavernous sinus, hypopituitarism)
  16. Pregnancy + pituitary tumor monitoring
  17. Replacement therapy after surgery (cortisol always first)
  18. 22E new drugs: pasireotide LAR, osilodrostat, relacorilant, oral octreotide, vemurafenib for cranio
  19. MEN1 association (pituitary + parathyroid + pancreas)
  20. Bilateral adrenalectomy + Nelson’s syndrome considerations

393.3.0.5 ⚙ Detailed Treatment Decision Tree

393.3.0.5.1 Acromegaly Post-TSS
1. Post-op IGF-1 + GH (OGTT) 3 mo
   - Normal → cured, surveillance
   - Persistent ↑ → medical therapy
2. Medical: SSA (octreotide/lanreotide) 6 mo trial
   - Normalize → continue
   - Partial → switch pasireotide OR add pegvisomant OR add cabergoline
   - 侍 response → switch pegvisomant (most effective)
3. Refractory → RT (Gamma Knife / CyberKnife)
4. Combination therapy common
393.3.0.5.2 Cushing’s Disease Post-TSS
1. Day 2-3 AM cortisol
   - < 5 → cured, replacement temp
   - 5-15 → equivocal
   - > 15 → not cured
2. 6 wk f/u: dynamic testing
   - Persistent CD → repeat TSS / RT / medical
3. Medical:
   - 1st: osilodrostat or ketoconazole/metyrapone
   - 2nd: pasireotide LAR
   - 3rd: GR antagonist (mifepristone, relacorilant) for DM-related
4. Bilateral adrenalectomy: last resort + preventive RT for Nelson's

393.3.0.6 ⚠ 內專芁害

  • Acromegaly heart failure / sudden cardiac death if 䞍治
  • Cushing’s untreated mortality very high (CV, infection, suicide)
  • Cabergoline-resistant prolactinoma decisions: surgery vs higher dose
  • Pregnancy + functioning macroadenoma management
  • Pituitary apoplexy — never delay steroid for confirmation
  • Co-secretion common (GH + PRL; NFPA + mild PRL ↑)
  • Hypopituitarism post-treatment — ALWAYS assess + replacement order

⚠ AI 草皿。