SESSION TITLE: Cancer Student/Resident Case Report Posters III
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Primary sarcomas of the great vessels are rare. Presented is a case of an undifferentiated intimal sarcoma of the inferior vena cava (IVC) with extension to the right atrium, adrenal, and renal veins.
CASE PRESENTATION: A 52-year-old woman presented with a one month history of fatigue, dyspnea and bilateral lower extremity edema. She was not in distress and hemodynamically stable. An echocardiogram demonstrated a 4.5 cm right atrial mass with extension into the IVC. An abdominal MRI demonstrated a 15.2 x 5.7 x 6.8 cm solid, heterogeneously enhancing IVC tumor extending 4 cm distal to the right renal vein, 1.2 cm into the right renal vein, 1.0 cm into the left renal vein, and upper IVC with a 1.5 cm nodule within the inferior right atrium. A cardiac MRI showed tumor within the upper IVC extending posteriorly to the right atrium with a 2 cm thrombus adherent to the intra-cardiac portion of the tumor. A multidisciplinary approach resulted in IVC resection from the right atrium to the infrarenal IVC with composite graft reconstruction, right nephrectomy and adrenalectomy, left nephrectomy with autotransplantaton, total hepatectomy and liver autotransplantation. The tumor was comprised of spindled cells in a fasicular growth pattern with cigar-shaped nuclei and exhibited marked nuclear pleomorphism. Immunohistochemistry had negative staining for epithelial differentiation, nerve sheath differentiation, endothelial cell markers, and smooth muscle marker, but Fli-1 positive (endothelial cell marker). Ki-67 or the proliferative index measured 60%. Given the histologic features and immunohistochemical staining pattern, this tumor was classified as a high grade undifferentiated intimal sarcoma. Postoperatively, the patient received chemotherapy and remains free of tumor recurrence.
DISCUSSION: Sarcomas of the IVC have a female predominance typically presenting in the fifth decade. (1) They are most often derived from medial smooth muscle, but intimal sarcomas, leiomyomas, synovial sarcoma, angiosarcoma, and rhabdomyosarcoma have been reported. Intimal sarcomas have been reported in the superior vena cava, IVC, and brachiocephalic vein. (2) There have been five cases of venous undifferentiated intimal sarcomas reported in the literature, two of which occurred in the IVC. Burke et.al reviewed 16 IVC sarcoma cases. 12 of the 16 patients were women and presented with symptoms of shortness of breath, pain, thrombosis, and IVC syndrome. (1)
CONCLUSIONS: Primary IVC sarcomas need to be included as part of a complete differential diagnosis in patients with atrial masses.
Reference #1: Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic study. Cancer 1993;71(5):1761-73.
Reference #2: Sebenik M, Ricci J, DiPasquale B, Mody K, Pytel P, Jee K, Knuutila S, Scholes J. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005; 29(9): 1184-1193.
DISCLOSURE: The following authors have nothing to disclose: Aasim Afzal, Jamil Alsahhar, Adam Mora
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