290.1 🎓 醫孞生版

290.1.0.1 📌 䞀頁重點

290.1.0.1.1 Overview
290.1.0.1.1.1 Epidemiology
  • Primary cardiac tumors: rare (~ 0.02% autopsy prevalence)
  • Secondary (metastatic): 20-40x more common than primary
  • All ages but primary tumors have specific age peaks
290.1.0.1.1.2 Classification
  • Primary:
    • Benign (75%): myxoma, papillary fibroelastoma, lipoma, rhabdomyoma, fibroma, hemangioma
    • Malignant (25%): angiosarcoma (most common), rhabdomyosarcoma, leiomyosarcoma, primary cardiac lymphoma
  • Secondary (metastatic):
    • Direct extension: lung, esophagus, mediastinal
    • Hematogenous: breast, melanoma, lymphoma, leukemia
    • Pericardial seeding
290.1.0.1.2 Primary Benign Cardiac Tumors
290.1.0.1.2.1 Myxoma (Most Common Primary, ~ 50%)
  • Location: LA (~ 75%, near fossa ovalis), RA (15-20%), ventricles (5%)
  • Demographics: 30-60 yo, F > M (3:1)
  • Familial / syndromic: 7% — Carney complex (PRKAR1A gene)
    • NAME syndrome: Nevi, Atrial myxoma, Myxoid neurofibromata, Ephelides
    • LAMB syndrome: Lentigines, Atrial myxoma, Blue nevi
  • Clinical triad:
    1. Obstructive symptoms (most common): dyspnea, syncope, HF, position-dependent symptoms; “tumor plop” (early diastolic sound), murmur (mimics MS)
    2. Embolic phenomena: systemic emboli (50% to brain → stroke), retina, peripheral vessels; histology in thrombectomy
    3. Constitutional symptoms: fever, weight loss, fatigue, malaise, arthralgia, ↑ ESR, ↑ IL-6 (paraneoplastic)
  • Diagnosis: TTE > TEE; CMR shows characteristic appearance (T1 isointense, T2 hyperintense, gadolinium enhancement)
  • Treatment: Surgical excision (curative); recurrence rare (1-5%); familial higher recurrence
  • Prognosis: excellent post-resection
290.1.0.1.2.2 Papillary Fibroelastoma
  • Most common valvular tumor
  • Small, papillary, mobile (frond-like appearance)
  • Aortic > mitral > tricuspid > pulmonic
  • Clinical: incidental finding OR systemic embolism (stroke, MI from coronary embolism), TIA
  • Treatment: surgical excision if symptomatic, left-sided, or large; antiplatelet if asymptomatic
  • 2024 ACC/AHA: surgery for symptomatic or left-sided > 1 cm
290.1.0.1.2.3 Lipoma
  • 2nd most common primary benign
  • Encapsulated fat
  • Often subepicardial; LA most common
  • Usually asymptomatic; surgery if symptomatic
290.1.0.1.2.4 Rhabdomyoma (Most Common in Children)
  • Pediatric tumor
  • Tuberous sclerosis association (TSC1/TSC2 mutations) — > 50% of TS have rhabdomyoma
  • Multiple, ventricular
  • Often regresses spontaneously
  • Symptomatic: arrhythmia, obstruction
  • Treatment: mTOR inhibitors (everolimus) — novel
  • Surgery rarely needed
290.1.0.1.2.5 Fibroma
  • 2nd most common pediatric cardiac tumor
  • Ventricular myocardium
  • Asymptomatic or arrhythmia, syncope
  • Surgery for symptomatic
290.1.0.1.2.6 Hemangioma
  • Vascular tumor
  • Rare
  • Surgery for symptomatic
290.1.0.1.3 Primary Malignant Cardiac Tumors
290.1.0.1.3.1 Angiosarcoma (Most Common Primary Malignant)
  • Location: RA most common (most), aggressive
  • Demographics: 30-50 yo
  • Clinical: pericardial effusion (often bloody), HF, embolic
  • Prognosis: poor (median survival 6-12 months)
  • Treatment: surgical resection + chemotherapy (doxorubicin + ifosfamide); radiation; immunotherapy emerging
  • 2024 Updates: pazopanib, immune checkpoint inhibitors (limited data)
290.1.0.1.3.2 Rhabdomyosarcoma
  • 2nd most common primary malignant in children
  • Multiple, infiltrative
  • Median survival 9-12 months
  • Surgery + chemo (vincristine, dactinomycin, cyclophosphamide)
290.1.0.1.3.3 Leiomyosarcoma
  • LA most common
  • Mimics myxoma
  • Poor prognosis
290.1.0.1.3.4 Primary Cardiac Lymphoma
  • Rare; usually B-cell, large diffuse
  • HIV / immunocompromise risk
  • Chemotherapy (R-CHOP)
  • Better prognosis if responsive
290.1.0.1.4 Secondary (Metastatic) Cardiac Tumors
290.1.0.1.4.1 Most Common Sources
  • Lung (most common — 35%)
  • Breast (25%)
  • Melanoma (highest incidence to heart in any cancer — 50-70% of metastatic melanoma!)
  • Lymphoma / leukemia
  • Esophageal (direct extension)
  • Renal cell carcinoma (extending up IVC)
290.1.0.1.4.2 Pathways
  • Direct extension (lung, esophagus, mediastinal)
  • Hematogenous (breast, melanoma, kidney)
  • Lymphatic (lymphoma)
  • Trans-venous (renal cell, hepatocellular)
290.1.0.1.4.3 Clinical
  • Pericardial effusion (often bloody, tamponade)
  • Arrhythmias
  • HF, conduction issues
  • Often diagnosed at autopsy
290.1.0.1.4.4 Treatment
  • Treat primary malignancy
  • Pericardial drainage if tamponade
  • Local: surgical resection if isolated metastasis (rare)
  • Chemotherapy / immunotherapy
  • Pericardial window for recurrent effusion
290.1.0.1.5 Diagnostic Imaging
290.1.0.1.5.1 Echocardiography
  • First-line screening
  • TTE: most masses visible
  • TEE: better for atrial, valve, small masses
  • Distinguishes: thrombus vs tumor vs vegetation
  • 3D echo: spatial relationships
290.1.0.1.5.2 Cardiac MRI (CMR)
  • Gold standard for tissue characterization
  • T1, T2, FLAIR signals
  • LGE: enhancement patterns
  • First-pass perfusion (vascular vs avascular)
  • Cine: function impact
  • Helps distinguish:
    • Thrombus: non-enhancing, low signal
    • Myxoma: T2 hyperintense, isointense T1, enhances
    • Lipoma: high T1, fat suppression
    • Angiosarcoma: heterogeneous, vascular
290.1.0.1.5.3 CT
  • Calcification (fibroma)
  • Anatomy
  • Staging for malignancy
290.1.0.1.5.4 PET-CT (FDG)
  • Distinguishes benign vs malignant
  • Malignant: high FDG avidity
  • Thrombus: no FDG
290.1.0.1.5.5 Echo + CMR + PET combination
  • 2024 gold standard for cardiac tumor workup
290.1.0.1.6 Differential Diagnosis
290.1.0.1.6.1 Thrombus
  • AF, anterior MI, LV aneurysm
  • Non-enhancing on CMR
  • Anticoagulation responsive
290.1.0.1.6.2 Vegetation
  • Endocarditis context
  • Blood cultures
  • Often valvular
290.1.0.1.6.3 Pseudotumor
  • Lipomatous hypertrophy of interatrial septum (LHIS) — benign fat accumulation
  • Caseous mitral annular calcification
  • Hiatus hernia

290.1.0.2 🩺 床邊速查

  • Myxoma: most common primary tumor; LA 75%; triad (obstruction + embolism + constitutional); surgical excision curative
  • Papillary fibroelastoma: most common valvular tumor; embolic risk; surgical excision if symptomatic, large, or left-sided
  • Rhabdomyoma: pediatric; tuberous sclerosis association; mTOR inhibitor option
  • Angiosarcoma: most common primary malignant; RA; poor prognosis
  • Metastatic: 20-40x more common than primary; melanoma highest cardiac metastasis rate
  • Imaging: echo + CMR (tissue) + PET (malignancy)