290.3 🏥 內科專科考前版

290.3.1 Mechanistic Deep Dive

290.3.1.1 Myxoma Pathology

  • Gelatinous, mucopolysaccharide matrix
  • Stellate cells, multinucleated
  • Multipotent mesenchymal origin
  • IL-6 secretion → constitutional symptoms
  • Carney complex: PRKAR1A loss → cAMP/PKA pathway dysregulation

290.3.1.2 Angiosarcoma Pathology

  • Vascular endothelial origin
  • Pleomorphic, vascular channels
  • Often bloody pericardial effusion
  • VEGF, MET pathway involvement
  • KDR (VEGFR2) mutations
  • Aggressive biology

290.3.1.3 Rhabdomyoma Pathology

  • Mature striated muscle origin
  • TS: hamartomatous proliferation
  • mTOR pathway hyperactive
  • mTOR inhibitor (everolimus, rapamycin) → regression

290.3.2 Recent Trials & Updates

290.3.2.1 Pazopanib for Angiosarcoma (Phase II)

  • VEGFR/PDGFR inhibitor
  • Partial responses
  • Used in refractory

290.3.2.2 Immune Checkpoint Inhibitors in Sarcomas

  • Limited efficacy in cardiac sarcoma
  • Trials ongoing

290.3.2.3 Everolimus for Rhabdomyoma in TSC (TESSTAL trial)

  • Significant tumor reduction
  • Approved in pediatric TS with neurologic indications

290.3.2.4 CMR Advances (2024)

  • Quantitative T1/T2 mapping
  • Diffusion-weighted CMR for differentiation
  • AI-assisted lesion characterization

290.3.2.5 PET-CT for Cardiac Tumors

  • Improved sensitivity for malignancy
  • Pre-op staging

290.3.3 High-Yield Specialist Points

290.3.3.1 Surgical Approach to Myxoma

  • Median sternotomy + cardiopulmonary bypass
  • Resection with attached endocardium (prevent recurrence)
  • Patch closure of defect
  • Excellent long-term outcomes
  • Carney complex: bilateral atrial + ventricular monitoring

290.3.3.2 Papillary Fibroelastoma Management 2024

  • Symptomatic (especially stroke) → surgical excision
  • Left-sided + > 1 cm → consider surgery
  • Right-sided / small → antiplatelet
  • Increasing transcatheter / robotic excision

290.3.3.3 Pediatric Cardiac Tumor Care

  • Rhabdomyoma + TSC: workup brain MRI, renal US, ophthalmology
  • Fibroma: assess for Gorlin syndrome (BCC, jaw cysts, hyperthyroidism etc.)
  • Multidisciplinary pediatric oncology, cardiology, surgery

290.3.3.4 Sarcoma Management (2024)

  • Multidisciplinary sarcoma board
  • Neoadjuvant chemo for resectable
  • Adjuvant chemo + radiation
  • Doxorubicin-based regimens
  • Immunotherapy + targeted (pazopanib) in clinical trials

290.3.3.5 Cardiac Lymphoma

  • B-cell, large diffuse most common
  • Right-sided predominance
  • HIV / EBV association
  • R-CHOP chemotherapy
  • Survival 50% at 1 year

290.3.3.6 Cardiac Metastases — Treatment

  • Treat primary aggressively
  • Pericardial drainage if tamponade
  • Pericardial sclerosis (bleomycin) for recurrent
  • Surgery rarely beneficial
  • Palliative goals

290.3.3.7 Pre-Op CMR for Cardiac Tumor

  • Define extent, invasion, function impact
  • Plan resection
  • Distinguish from thrombus

290.3.3.8 Special: IVC Tumor Thrombus

  • Renal cell carcinoma extending up IVC
  • Hepatocellular carcinoma
  • Surgical resection (Mayo classification of extension)
  • Multidisciplinary

290.3.3.9 Genetic Counseling

  • Carney complex: family screening + multi-organ surveillance
  • TSC: skin, brain, renal screening; genetic testing
  • Gorlin syndrome: skin BCC screening

290.3.3.10 Future Directions

  • CAR-T for primary cardiac lymphoma
  • Personalized molecular targeting
  • 3D-printed pre-op models
  • Robotic resection
  • Minimally invasive cardiac surgery

290.3.4 Pearls

  • Myxoma: LA fossa ovalis 75%; triad (obstruction + embolism + constitutional); surgery curative
  • Papillary fibroelastoma: most common valvular tumor; aortic valve > mitral; embolic risk
  • Rhabdomyoma + TSC: 50%+ of TS have rhabdomyoma; mTOR inhibitor option
  • Angiosarcoma: most common primary malignant; RA; poor prognosis; multimodal therapy
  • Metastatic > primary 20-40x: melanoma highest cardiac metastasis rate
  • CMR + PET-CT = gold standard for characterization
  • Carney complex (PRKAR1A): NAME / LAMB syndromes; multiple recurrent myxomas