Benign mediastinal lymphadenopathy presenting as cor triatriatum

Area of Science:

• Cardiology
• Pediatric Oncology
• Thoracic Surgery

Background:

• Superior mediastinal masses and intrathoracic lymphadenopathy can present diagnostic challenges in pediatric oncology.
• Cardiovascular abnormalities can sometimes manifest with atypical thoracic findings.

Observation:

• A pediatric patient presented with a superior mediastinal mass and extensive intrathoracic lymphadenopathy, initially referred for oncologic care.
• An echocardiographic evaluation revealed an unexpected diagnosis of a highly obstructive left atrial cor triatriatum.
• The patient underwent surgical repair of cor triatriatum and lymph node biopsy.

Findings:

• Biopsies of the lymph nodes showed reactive changes with lymphatic dilation, excluding inflammatory or neoplastic processes.
• The extensive mediastinal lymphadenopathy was found to be a consequence of severe pulmonary venous hypertension secondary to cor triatriatum.
• This case is the first reported pediatric instance of mediastinal lymphadenopathy mimicking malignancy due to pulmonary venous hypertension.

Implications:

• This case underscores the importance of considering cardiac anomalies in the differential diagnosis of pediatric mediastinal masses.
• It highlights a rare presentation where a congenital heart defect can lead to secondary thoracic abnormalities that mimic oncologic conditions.
• Early diagnosis and surgical intervention for cor triatriatum are crucial for resolving secondary pulmonary and lymphatic complications.