Intravenous Leiomyomatosis with Extension to the Heart Associated with Disseminated Peritoneal Leiomyomatosis / 대한내과학회지

Leiomyomatosis refers to benign smooth muscle cell tumors that often arise from unusual growth patterns and include benign metastasizing leiomyoma, disseminated peritoneal leiomyomatosis, and intravenous leiomyomatosis. Intravenous leiomyomatosis is the extension of a vascular tumor into the venous channels, whereas disseminated peritoneal leiomyomatosis is characterized by multiple leiomyomas growing along the submesothelial tissues of the abdominopelvic peritoneum. It is extremely rare for intravenous leiomyomatosis and disseminated peritoneal leiomyomatosis to occur simultaneously. A 42-year-old female presented with disseminated peritoneal leiomyomatosis and intravenous leiomyomatosis extended through the inferior vena cava into the right side of the heart. The patient underwent one-stage surgery under simultaneous sternotomy and laparotomy, and radical excision of the tumor was achieved using cardiopulmonary bypass. Here we describe a case in which complete removal of a leiomyomatosis with an unusual growth pattern was successfully performed using one-stage surgery.

A Case of Noonan Syndrome Presenting with Malignant Hypertension in an Adult / 대한내과학회지

Noonan syndrome is an autosomal dominant disorder characterized by dysmorphic facial features, congenital heart defects and short stature. To date, renal artery stenosis has not been associated with Noonan syndrome. We report the case of a 27-year old male who presented with malignant hypertension associated with renal artery stenosis, dysmorphic facial features, pectus excavatum, pulmonary stenosis and hypertrophic cardiomyopathy who was diagnosed with Noonan syndrome.

Acute ST Elevated Myocardial Injury due to Coronary Thrombosis during Thoracic Endovascular Aortic Repair in Patient with Protein S Deficiency

A 71-year-old woman who had suffered from pulmonary thromboembolism with deep vein thrombosis for 12 years presented the hospital with a huge thoracic aortic aneurysm. During thoracic endovascular therapy, she had a sudden coronary artery occlusion without having organized stenosis or plaque rupture even under the dual antiplatelet treatment and heparinization. She turned out to be having a protein S deficiency. A procedure related thrombotic adverse event in patient with protein S deficiency is very rare, so we report a case with literature review.