Inferior Vena Cava Leiomyosarcoma: Case Report and Review of Radiologic Findings
Poster #: 068
Session/Time: A
Author:
Avery Seward, BA
Mentor:
Trenton Taros, MD
Research Type: Case Report
Abstract
INTRODUCTION:
Inferior vena cava (IVC) leiomyosarcomas are rare, slow-growing primary malignancies of the smooth muscle of the IVC. They represent approximately 0.5% of adult soft tissue sarcomas, with a female-to-male ratio of 3:1 and a peak incidence in the sixth decade of life. As patients may be asymptomatic or present with only nonspecific symptoms such as abdominal pain, prompt and accurate radiologic characterization of these tumors is essential to ensure timely diagnosis and management.
CASE INFORMATION:
A 38 year old woman with past medical history of hypertension, gastroesophageal reflux disease, and endometriosis presented to the clinic with a 4-month history of progressively worsening centrally-located abdominal and back pain. The patient reported that the pain was initially post-prandial and nocturnal lasting for a few minutes at a time, but had progressed to lasting for hours at a time over the past 3 weeks. Associated symptoms included nausea, vomiting, and diarrhea. She was started on a heparin drip with some improvement in her pain and multiple imaging modalities were obtained, suggesting an intra-renal IVC thrombus with soft tissue lesion concerning for primary leiomyosarcoma. The patient's case was discussed at a multi-disciplinary tumor board with decision to proceed with surgical resection of the probable primary IVC leiomyosarcoma. During surgery, the IVC mass and peri-aortic tissue were biopsied to establish a definitive diagnosis, confirming grade 2 leiomyosarcoma.
DISCUSSION/CLINICAL FINDINGS:
Radiologically, the presence of a soft tissue mass that is localized to and expanding into the IVC is suggestive of a leiomyosarcoma. IVC leiomyosarcoma can be visualized with ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI). On ultrasound, they often appear as hypoechoic intraluminal masses with high vascularity and abnormal flow. However, in our patient, there was limited evaluation on ultrasound due to overlying bowel gas. On CT, IVC leiomyosarcomas commonly present as filling defects will loss of visualization of the IVC wall at the site of tumor contact. Furthermore, there can be enhancing filling defects if there is an intraluminal component. In our patient, the initial CT showed distortion of the right renal artery with perivascular haziness posterior to the IVC and narrowing of the left renal vein. On subsequent CTA Abdomen/Pelvis, there was a notable juxtarenal IVC filling defect abutting the abdominal aorta and right renal artery with severe narrowing of the left renal vein. On MRI, IVC leiomyosarcomas typically appear as heterogeneous and irregular soft tissue masses. In our patient, her MRI showed peripheral enhancing lobular wall thickening with absence of central enhancement, and with confluent soft tissue abutting the anterior wall of the abdominal aorta. There was also notable markedly narrowed distal left renal vein adjacent to IVC. Taken together, these radiographic findings were most consistent with a diagnosis of IVC leiomyosarcoma, and facilitated timely surgical intervention.
CONCLUSION:
Unfortunately, prognosis for IVC leiomyosarcoma is poor with five-year survival less than 50%. However, recognition and accurate radiographic characterization is critical, as this enables prompt intervention and may improve patient outcomes.
Inferior vena cava (IVC) leiomyosarcomas are rare, slow-growing primary malignancies of the smooth muscle of the IVC. They represent approximately 0.5% of adult soft tissue sarcomas, with a female-to-male ratio of 3:1 and a peak incidence in the sixth decade of life. As patients may be asymptomatic or present with only nonspecific symptoms such as abdominal pain, prompt and accurate radiologic characterization of these tumors is essential to ensure timely diagnosis and management.
CASE INFORMATION:
A 38 year old woman with past medical history of hypertension, gastroesophageal reflux disease, and endometriosis presented to the clinic with a 4-month history of progressively worsening centrally-located abdominal and back pain. The patient reported that the pain was initially post-prandial and nocturnal lasting for a few minutes at a time, but had progressed to lasting for hours at a time over the past 3 weeks. Associated symptoms included nausea, vomiting, and diarrhea. She was started on a heparin drip with some improvement in her pain and multiple imaging modalities were obtained, suggesting an intra-renal IVC thrombus with soft tissue lesion concerning for primary leiomyosarcoma. The patient's case was discussed at a multi-disciplinary tumor board with decision to proceed with surgical resection of the probable primary IVC leiomyosarcoma. During surgery, the IVC mass and peri-aortic tissue were biopsied to establish a definitive diagnosis, confirming grade 2 leiomyosarcoma.
DISCUSSION/CLINICAL FINDINGS:
Radiologically, the presence of a soft tissue mass that is localized to and expanding into the IVC is suggestive of a leiomyosarcoma. IVC leiomyosarcoma can be visualized with ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI). On ultrasound, they often appear as hypoechoic intraluminal masses with high vascularity and abnormal flow. However, in our patient, there was limited evaluation on ultrasound due to overlying bowel gas. On CT, IVC leiomyosarcomas commonly present as filling defects will loss of visualization of the IVC wall at the site of tumor contact. Furthermore, there can be enhancing filling defects if there is an intraluminal component. In our patient, the initial CT showed distortion of the right renal artery with perivascular haziness posterior to the IVC and narrowing of the left renal vein. On subsequent CTA Abdomen/Pelvis, there was a notable juxtarenal IVC filling defect abutting the abdominal aorta and right renal artery with severe narrowing of the left renal vein. On MRI, IVC leiomyosarcomas typically appear as heterogeneous and irregular soft tissue masses. In our patient, her MRI showed peripheral enhancing lobular wall thickening with absence of central enhancement, and with confluent soft tissue abutting the anterior wall of the abdominal aorta. There was also notable markedly narrowed distal left renal vein adjacent to IVC. Taken together, these radiographic findings were most consistent with a diagnosis of IVC leiomyosarcoma, and facilitated timely surgical intervention.
CONCLUSION:
Unfortunately, prognosis for IVC leiomyosarcoma is poor with five-year survival less than 50%. However, recognition and accurate radiographic characterization is critical, as this enables prompt intervention and may improve patient outcomes.