Inflammatory pseudotumor arising from the right ventricular outflow tract causing pulmonary stenosis

Area of Science:

• Cardiovascular Pathology
• Pediatric Oncology
• Surgical Pathology

Background:

• Inflammatory pseudotumor (IPT), also known as inflammatory myofibroblastic tumor (IMT), is an uncommon neoplastic-like lesion characterized by spindle cells.
• Initially identified in the lung, IPTs are now recognized across virtually all major organ systems.
• These lesions exhibit a diverse range of clinical presentations and behaviors.

Observation:

• A case is presented involving a seven-year-old male.
• The patient presented with an inflammatory pseudotumor located in the right ventricular outflow tract of the heart.
• This specific tumor involved the pulmonary valve.

Findings:

• The inflammatory pseudotumor's involvement of the pulmonary valve led to significant obstruction.
• The primary finding was the development of pulmonary stenosis secondary to the cardiac inflammatory pseudotumor.
• Histopathological analysis confirmed the spindle cell nature of the lesion.

Implications:

• This case expands the known anatomical sites for inflammatory pseudotumors.
• It highlights the potential for cardiac involvement, specifically in the right ventricular outflow tract and pulmonary valve.
• Early diagnosis and management are crucial for pediatric patients presenting with cardiac masses causing hemodynamic compromise.