Pericardial Teratoma

• Rare tumor arises from pericardium and includes all three germ cell layers
• Prevalence 0.17-28/10,000
• Accounts for 6.7-12.8% of diagnosed cardiac tumors but is the most common tumor diagnosed during fetal life
• Majority right sided, encapsulated and do not invade
• When diagnosed in fetal life the mortality is high
• Rapidly growing and associated with a pericardial effusion
• High risk of fetal and postnatal demise secondary to compression limiting filling, tamponade and fetal hydrops
• Can be diagnosed prenatally
• Requires very close serial surveillance given rapid tumor growth
• Fetal hydrops is common secondary to mass effect and tamponade physiology and carries a poor prognosis
• Without hydrops prognosis remains favorable
• Fetal intervention has been performed in select cases with variable success

Frequent surveillance is key as these are rapidly growing tumors that can dramatically change in short period of time. Prompt referral for treatment to multidisiplinary fetal intervention center AT TIME OF DIAGNOSIS is key as these tumors demonstrate rapid growth with high risk of intrauterine demise or early postnatal death. Consideration for fetal surgury and resection is possible in select cases with good results

Types of Fetal intervention

1. Pericardiocentesis
2. Pericardioamniotic shunt
3. Laser ablation of feeding vessels
4. EXIT procedure (ex utero intrapartum treatment) to resection
5. Open fetal surgery

Fetal Echo Evalutation:

• Evaluate tumor location, size (most commonly right sided)
• Evaluate degree of cardiac compression (most commonly right heart compression with restricted tricuspid valve inflow)
• Evaluate right heart dimensions (often with compression of right atrium and subsequent right ventricular hypoplasia
• Evaluate for tricuspid inflow obstruction
• Evaluate PDA flow (retrograde ductal flow in setting of RV hypoplasia may indicate ductal dependency postnatally)
• Assess cardiac output (declining cardiac output may indicate rapid growth and risk for hydrops
• Evaluate for fetal hydrops (pericardial effusion)
• Evaluate fetal rhythm (compression of SA node can cause fetal sinus bradycardia)
• Evaluate flow in ductus venosus and umbilical vein for evidence of elevated venous pressures in setting of restricted right heart filling
• In presence of worsening fetal cardiac status (enlarging tumor, pericardial effusion, abnormal ductus venosus or umbilical venous doppler) or presence of fetal hydrops (enlarging tumor, pericardial effusion) consideration of fetal intervention or early delivery is warranted as risk of in utero demise is high

 

References:

Rychik J, Khalek N, Gaynor JW et al. Fetal intrapericardial teratoma: natural history and managment including successful in utero surgery. Am J Obstet Gynecol. Dec 2016. 

Nassr AA, Shazly SA, Morris SA et al. Prenatal management of fetal intrapericardial teratoma: A systematic review. Prenatal Diagnosis. Sept 2017. 

• • Fetal pericardial teratoma