Primary Leiomyosarcoma of the Inferior Vena Cava Presenting as a Renal Mass

CASE REVIEW Primary Leiomyosarcoma of the Inferior Vena Cava Presenting as a Renal Mass Oleg Shvarts, MD, Ken-Ryu Han, MD, John S. Lam, MD, Arie S. Belldegrun, MD, FACS Department of Urology, David Geffen School of Medicine at UCLA, Los Angeles, CA Leiomyosarcoma of the inferior vena cava (IVC) is an extremely rare entity. We present the case of a 62-year-old woman who was found to have a large right upper quadrant mass upon examination by her primary care physician in evaluation for diffuse abdominal pain accompanied by anorexia and weight loss. A computed tomographic scan and magnetic resonance imaging demonstrated a 13-cm retroperitoneal lesion that appeared to stem from the right kidney and yielded a tumor thrombus up to the level of the hepatic venous confluence. The patient underwent a right radical nephrectomy and IVC thrombectomy for treatment of a presumed renal cell carcinoma. Instead, pathology revealed the tumor to be a leiomyosarcoma of the IVC. We document this unusual presentation of an extremely rare tumor entity. [Rev Urol. 2004;6(1):39-42] © 2004 MedReviews, LLC Key words: Leiomyosarcoma • Inferior vena cava • Nephrectomy 62-year-old woman presented to her primary care physician with complaints of diffuse abdominal pain over the span of several weeks, accompanied by anorexia and a 30-lb weight loss. The patient denied fevers, chills, nausea, vomiting, hematuria, or dysuria. Her past medical history was consistent with anxiety, and her past surgical history was noncontributory. Upon examination A VOL. 6 NO. 1 2004 REVIEWS IN UROLOGY 39 Leiomyosarcoma of the IVC continued Figure 1. Abdominal computed tomography scan demonstrating a 13-cm renal tumor involving the inferior vena cava and invading the liver. Figure 2. Abdominal computed tomography scan demonstrating extension of an inferior vena cava thrombus to hepatic venous confluence. by the primary care physician, a large right upper quadrant mass was noted. Aside from an elevated alkaline phosphatase level of 197 U/L, the patient’s laboratories, including a hematologic panel, chemistry, and liver function tests, were found to be normal. The patient underwent a computed tomography (CT) scan with intravenous contrast, which demonstrated a 13-cm lobulated mass arising from the upper pole of the right kidney and contiguous with the undersurface of the right lobe of the liver (Figure 1). The lesion was noted to contain a massive tumor thrombus extending through the renal vein and into the inferior vena cava (IVC) up to the hepatic venous confluence (Figure 2). Magnetic resonance imaging (MRI) was subsequently performed, which confirmed a tumor 40 VOL. 6 NO. 1 2004 REVIEWS IN UROLOGY thrombus extending up the IVC to the level of the atrial junction. Given the appearance of the tumor on CT and MRI, extensive renal cell carcinoma was diagnosed and the patient underwent a metastatic workup (including bone scan and lung and head CT scans), which was significant for small bilateral pulmonary nodules. After urology and oncology consultations, the patient was advised to undergo radical nephrectomy, IVC thrombectomy, and adjuvant immunotherapy. During the nephrectomy, the tumor was found to be extensive, directly invading into the liver and adhering to the duodenum and small bowel. The IVC was also found to be full of tumor to a diameter of 8 cm to 9 cm. The tumor was freed from the liver and duodenum. Upon opening the IVC, attempts were made to shell the tumor out, but it was found to be invading into the wall, requiring resection of a segment of IVC during the en bloc resection of the tumor mass and right kidney. An extensive lymphadenectomy was performed. Subsequently, through the thoracoabdominal incision, several excisional biopsies were taken of lesions noted in the right lung. Review of the pathology specimens demonstrated a 12.5-cm tumor with 25% necrosis. The kidney specimen demonstrated extension through the capsule into adjacent adipose tissue and lymph nodes and into the adrenal gland. The tumor was also found to extend into the collecting system. Surprisingly, microscopic examination of the lesion demonstrated high-grade sarcomatoid cells consistent with leiomyosarcoma (Figure 3). This diagnosis was confirmed when smooth muscle markers (desmin and smooth muscle actin) were positive. Per pathology, the tumor was thought to arise from the IVC. The excisional lung biopsy was also found to be consistent with metastatic leiomyosarcoma. Discussion Retroperitoneal leiomyosarcoma is a rare entity, accounting for 5% to 15% of all retroperitoneal tumors.1 Leiomyosarcomas of the IVC are extremely rare, documented in the surgical literature mostly as case reports rather than organized series. To our knowledge, there has been no mention in the urologic literature of a leiomyosarcoma of the IVC presenting as a renal mass like a renal cell carcinoma. Leiomyosarcomas of the IVC can arise in the lower (below the renal vessels), middle (renal vessels to retrohepatic IVC), and upper (suprahepatic) portions of the IVC. Given their location deep in the retroperitoneum, these tumors usually have an Leiomyosarcoma of the IVC Figure 3. Microscopic examination of the primary tumor demonstrating high-grade sarcomatoid cells consistent with leiomyosarcoma. insidious presentation, with symptoms occurring at late stages of the disease.2 Presenting symptoms can include abdominal pain or mass, Budd-Chiari syndrome, weight loss, fever, weakness, anorexia, vomiting, night sweats, and dyspnea.3 Leiomyosarcomas tend to enlarge via intraluminal or extraluminal growth rather than infiltration. Imaging usually demonstrates an IVC full of tumor, as well as extraluminal tumor in the retroperitoneum. If they involve adjacent organs, these tumors are often mistaken for primary leiomyosarcoma arising from retro- peritoneal smooth muscle rather than the IVC. However, such a tumor should be classified as a leiomyosarcoma of the IVC if it has predominantly intraluminal growth or if a segment of the involved IVC needs to be resected with the extraluminal tumor.4 Historically, leiomyosarcomas of the IVC have been resistant to chemotherapy and radiation.3 As a result, radical en bloc resection with assurance of adequate margins serves as the only adequate treatment.2 Unfortunately, the recurrence rate with these tumors is high. Even following a complete primary resection, more than 50% of patients develop a late recurrence.3,5 Secondary resection is recommended if the recurrence is amenable to surgical intervention and if no metastases are present.5 In the case of pulmonary metastasis, resection is recommended if a solitary lesion is noted.6 Some success has been achieved with aggressive surgical management of these tumors. The largest literature review of case reports to date, including 218 patients, reported 5- and 10-year survival rates of 49.4% and 29.5%, respectively.4 Given the proximity of the middle section of the IVC to numerous retroperitoneal organs and the extensive innervation in that area, tumors involving this portion of the IVC generally are symptomatic earlier and more often. Because of this earlier presentation, these tumors generally have a better prognosis, with 5- and 10-year survival rates of 56.7% and 47.3%, respectively.4 Other indicators of a favorable prognosis include the presence of abdominal pain, absence of a palpable mass, and surgical resection. In contrast, high-grade tumors, those involving the upper portion of the IVC, and those associated with BuddChiari syndrome or IVC occlusion have particularly bad prognoses.3,4 Main Points • Leiomyosarcomas of the inferior vena cava (IVC) are extremely rare and may arise in the lower (below the renal vessels), middle (renal vessels to retrohepatic IVC), and upper (suprahepatic) portions of the IVC. • Presenting symptoms of leiomyosarcomas of the IVC include abdominal pain or mass, Budd-Chiari syndrome, weight loss, fever, weakness, anorexia, vomiting, night sweats, and dyspnea. • Historically, leiomyosarcomas of the IVC have been resistant to chemotherapy and radiation. Radical en bloc resection with assurance of adequate margins serves as the only adequate treatment of this tumor entity. • Because they are symptomatic earlier and more often, tumors involving the middle portion of the IVC generally have a better prognosis; other indicators of a favorable prognosis include the presence of abdominal pain, absence of a palpable mass, and surgical resection. • Tumors that are high-grade, involve the upper portion of the IVC, or are associated with Budd-Chiari syndrome or IVC occlusion have less favorable prognoses. VOL. 6 NO. 1 2004 REVIEWS IN UROLOGY 41 Leiomyosarcoma of the IVC continued References 1. 3. Felix EL, Wood DK, Das Gupta TK. Tumors of the retroperitoneum. Curr Probl Cancer. 1981;6:1-47. Shindo S, Matsumoto H, Ogata K, et al. Surgical treatment of retroperitoneal leiomyosarcoma invading the inferior vena cava: report of three cases. Surg Today. 2002;32:929-933. Mingoli A, Feldhaus RJ, Cavallaro A, Stipa S. 42 VOL. 6 NO. 1 2004 2. REVIEWS IN UROLOGY 4. Leiomyosarcoma of the inferior vena cava: analysis and search of world literature on 141 patients and report of three new cases. J Vasc Surg. 1991;14:688-699. Mingoli A, Cavallaro A, Sapienza P, et al. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res. 1996;16(5B):3201-3205. 5. 6. van Doorn RC, Gallee MP, Hart AA, et al. Resectable retroperitoneal soft tissue sarcomas: the effect of extent of resection and postoperative radiation therapy on local tumor control. Cancer. 1994;73:637-642. Arbeit JM, Flye MW, Mundinger GH Jr, Webber BL. Latent pulmonary embolus from a retroperitoneal sarcoma. Cancer. 1980;46:1492-1498.