400.2 📚 國考版醫垫國考 / PGY OSCE

400.2.0.1 📌 Cram Sheet

400.2.0.1.1 🔥 高 yield 12
  1. Pheo (adrenal) + PGL (extra-adrenal) = PPGL
  2. 40% hereditary (revised from 10%)
  3. 遺傳症候矀: MEN2 (RET), VHL, NF1, SDHx, TMEM127, MAX, FH
  4. SDHB → highest metastatic risk (30-50%)
  5. Triad: HA + 心悞 + 倚汗 + paroxysmal HTN
  6. Plasma fractionated metanephrines 1st line (99% sensitivity)
  7. Imaging order: CT/MRI → MIBG / DOTATATE PET
  8. Pre-op α-blocker 10-14d → β-blocker after → high-Na/fluid → surgery
  9. β-blocker first 倧忌 (unopposed α → crisis)
  10. Post-op: hypotension + hypoglycemia in first 24h
  11. Genetic testing for all PPGL
  12. Metastatic: MIBG therapy, PRRT (¹⁷⁷Lu-DOTATATE), sunitinib, CVD chemo
400.2.0.1.2 🔢 必背
項目 æ•žå­—
Hereditary % 40%
Plasma fractionated met sensitivity 99%
Pre-op α-blocker duration 10-14 d
Pre-op high-Na duration 7 d
Phenoxybenzamine 10-30 mg BID
Doxazosin 2-16 mg/d
Goal pre-op BP < 130/80 sitting
Malignant pheo ~10-15%
SDHB metastatic risk 30-50%

400.2.0.2 ⭐ 高 yield

400.2.0.2.1 Hereditary Syndromes
Syndrome Gene Pheo Other
MEN2A RET 50% MTC, parathyroid
MEN2B RET (M918T) 50% MTC (aggressive), mucosal neuroma, Marfan
VHL VHL 10-20% Hemangioblastoma, RCC, pancreatic NET
NF1 NF1 1-5% Neurofibroma, café-au-lait, Lisch
SDHB SDHB high Multiple PGL, metastatic, high-risk
SDHC SDHC varied Head/neck PGL
SDHD SDHD varied Head/neck PGL (paternal inheritance)
TMEM127, MAX rare varied
FH FH varied Leiomyomatosis + RCC
400.2.0.2.2 Catecholamine Profile
Adrenal pheo Extra-adrenal PGL SDHx
Epi + − −
NE + + +
Dopamine − varied +
400.2.0.2.3 Triggers (蚘!)
  • β-blocker without α
  • TCA, sympathomimetic, MAOI
  • Tyramine-rich foods (cheese, red wine, banana)
  • Anesthesia, surgery, palpation
  • Contrast, glucagon, metoclopramide, opioid
400.2.0.2.4 Pre-op Sequence (蚘順序!)
  1. α-blocker 10-14 d before (phenoxybenzamine or doxazosin)
  2. High-Na + fluid 7 d
  3. β-blocker after α established (control reflex tachycardia)
  4. CCB adjunct if needed
  5. Pre-op IV fluid
  6. Skilled team intra-op
  7. Post-op: 泚意 hypotension + hypoglycemia
400.2.0.2.5 Imaging Quick
Test Use
CT adrenal washout 1st line localization
MRI T2 bright alternative
MIBG (¹²³I-MIBG) extra-adrenal, metastatic
DOTATATE PET (Ga-68) SDHx, head/neck, metastatic
FDG-PET metastatic, SDHB
400.2.0.2.6 Drug Quick (Treatment)
Stage Drug
Pre-op α Phenoxybenzamine, doxazosin
Pre-op β Propranolol, atenolol (after α)
Intra-op crisis Nitroprusside, phentolamine
Post-op hypotension Phenylephrine, norepinephrine
Hypertensive crisis Phentolamine IV
Metastatic ¹³¹I-MIBG, ¹⁷⁷Lu-DOTATATE PRRT, sunitinib, CVD chemo, belzutifan (VHL)

400.2.0.3 🎯 自我檢枬

  1. PPGL hereditary %? → 40%
  2. Triad? → HA + 心悞 + 倚汗
  3. Plasma fractionated met sensitivity? → 99%
  4. SDHB metastatic risk? → 30-50%
  5. Pre-op α-blocker timing? → 10-14 d before
  6. β-blocker before α? → 倧忌 (unopposed α crisis)
  7. Phenoxybenzamine vs doxazosin? → Phenoxybenzamine irreversible non-selective; doxazosin selective α1
  8. Imaging 1st line? → CT/MRI adrenal
  9. Functional imaging extra-adrenal? → MIBG / DOTATATE PET
  10. Post-op concerns? → Hypotension + hypoglycemia
  11. Metastatic treatment? → MIBG, PRRT, sunitinib, CVD chemo
  12. MEN2A gene + pheo %? → RET, 50%
  13. VHL associated? → Hemangioblastoma, RCC, pancreatic NET
  14. NF1 features? → Neurofibroma, café-au-lait, Lisch
  15. Genetic testing in PPGL? → All patients

⚠ AI 草皿。