Leiomyosarcoma of the inferior vena cava with renal metastasis: An unusual case and diagnostic challenge

Abstract

Leiomyosarcomas are exceedingly rare malignant tumours with a very poor prognosis. We present a 78-year-old patient with a small renal and paracaval mass. Surgery included right open partial nephrectomy and resection of the paracaval mass to treat the presumed renal cell carcinoma. Pathology revealed the tumour to be leiomyosarcoma (LMS). This is only the second documented report of an inferior vena cava LMS presenting along with an ipsilateral renal mass. Case reportA 78-year-old male with a history of intermediate-risk prostate cancer treated with neoadjuvant and adjuvant androgen deprivation therapy, as well as external beam radiotherapy 1 year ago, presented at a follow-up appointment with significant right-sided flank and abdominal pain. Physical examination revealed tenderness in the right flank without detectable peripheral lymphadenopathy. Routine blood work was normal and prostate serum antigen (PSA) was 0.91 μg/L. Computed tomography (CT) and magnetic resonance imaging revealed an enhancing exophytic lesion off the inferior pole of the right kidney measuring 2.1 cm in diameter (Fig. 1a), as well as a 1.8-cm mass anterior to inferior vena cava (IVC) (Fig. 1b) suspicious for an enlarged lymph node. The right renal vein did not appear to be involved. These lesions were not likely metastatic prostate cancer since PSA was 0.91 μg/L. Consultation with urology was initiated for further management of the renal lesion. A positron emission tomography scan was ordered to assess the activity of the caval lymph node. It revealed a hypermetabolic IVC lymph node, as well as several bilateral hypermetabolic hilar lung abnormalities.A bone scan revealed no skeletal metastasis. Repeat CT scan showed growth of the IVC node to 2.2 cm and bilateral pulmonary nodules. The suspicious renal mass remained stable at 1.8 cm.The patient agreed to an open partial nephrectomy and retroperitoneal lymph node dissection as the anterior caval mass was not amendable to biopsy. During the preoperative period, another CT scan showed the IVC node had increased in size to 2.7 cm, while the renal mass remained stable. The renal mass was removed from the right kidney. Intraoperatively, the anterior caval mass involved the IVC and about 30% of the circumference was resected. Pathology revealed a high grade 2.3-cm renal mass and a 3.8-cm caval leiomyosarcoma (LMS) (Fig. 2a). Diagnosis was confirmed with positive smooth muscle cell markers for actin, desmin and vimentin (Fig. 2b). Based on pathology, the final diagnosis was primary IVC LMS with metastasis to the ipsilateral kidney.Postoperatively the patient was seen by the medical oncology team; the patient declined any adjuvant therapy. The patient developed multifocal pulmonary metastases 3 years after his surgery and succumbed to his disease a year later. DiscussionLeiomyosarcomas are extremely rare and often aggressive malignant tumours that are associated with a dismal prognosis.1-5 Management is not well-established due to rare presentations and limited literature. 6,7 The present...

Incidentally Diagnosed Extraluminal Leiomyosarcoma of Infrarenal Inferior Vena Cava: A Case Report and Literature Review from a Radiologist’s Perspective

“…On prone imaging, these tumors show persistent contact with the wall of IVC or persistent nonvisualization of the lumen of IVC. Tumors with predominant extraluminal component commonly occur in anterior relation to the IVC and towards the right side of midline [ 9 - 11 , 17 , 19 ]. However, tumors originating posteriorly from the IVC have also been reported [ 5 , 14 ].…”

“…These tumors can be locally aggressive but distant metastases are uncommon. However, lung is the most common site of metastasis [ 2 , 11 , 14 ]. Metastasis to the liver and kidney have also been reported [ 11 , 21 ].…”

“…However, lung is the most common site of metastasis [ 2 , 11 , 14 ]. Metastasis to the liver and kidney have also been reported [ 11 , 21 ].…”

Secondary Tumors of the Kidney: A Comprehensive Clinicopathologic Analysis

“…Several other primary sites and tumor types of secondary tumors involving the kidney have also been reported in the literature as case reports. Osteogenic sarcoma,140 chondrosarcoma,141 leiomyosarcoma of the inferior vena cava,142 neuroblastoma,143 meningeal haemangiopericytoma (solitary fibrous tumor),144,145 Merkel cell carcinoma,146 and synovial sarcoma147 have all been described as a possible source for renal metastases. Finally, carcinomas of unknown primary account for 5.3% of renal metastatic tumors 6…”

<p>A Case of a Huge Inferior Vena Cava Leiomyosarcoma: Precise Preoperative Evaluation with Gadobutrol-Enhanced MRI</p>

“…Nevertheless, many case reports have used MRI for IVC LMS diagnosis. 8,[12][13][14][15][16][17][18] Compared with CT, MRI can provide more accurate information about the tumour's location, extent, collateral vessels, and adjacent structures with superior soft-tissue resolution, special sequences, and safer contrast material. The T2-weighted black blood sequence and high concentration contrast agent can overcome the limitations of CT, clearly revealing the vessel walls and lumens of IVC and collateral vessels.…”