Leiomyosarcoma of the Inferior Vena Cava: Report of Two Cases and Review of the Literature

Abstract

Case ReportsLeiomyosarcoma of the Inferior Vena Cava: Report of Two Cases and Review of the Literature Adnan B. Mofti, FRCS, FRCS(Glasg) Mohammed Zarrar, and FRCS(Ed) Mervat S. Al-SalehFRCS(Ed) Adnan B. Mofti Address reprint requests and correspondence to Dr. Mofti: Department of Surgery, King Khalid University Hospital, P.O. Box 7805, Riyadh 11472, Riyadh. From the Department of Surgery, College of Medicine, King Saud University, Riyadh Search for more papers by this author , Mohammed Zarrar From the Department of Surgery, College of Medicine, King Saud University, Riyadh Search for more papers by this author , and Mervat S. Al-Saleh From the Department of Surgery, College of Medicine, King Saud University, Riyadh Search for more papers by this author Published Online:1 Jul 1989https://doi.org/10.5144/0256-4947.1989.397SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionLeiomyosarcoma of vascular origin is a rare and usually lethal tumor. It arises more commonly from large veins than arteries, and more than one half occur in the inferior vena cava.1 Most of these tumors are diagnosed at autopsy or laparotomy. Surgical resection offers the best prospect of cure; however, the majority of patients die from metastatic lesions into the liver and lungs.The purpose of this report is to describe two cases of leiomyosarcoma of the inferior vena cava.CASECase 1A 35-year-old Saudi woman was admitted with a 14-year history of an intermittent dull ache in the right hypochondrium. The pain had no relationship to meals, and there was no history of weight loss or other relevant complaints. Physical examination revealed a large, nontender, firm abdominal mass in the right flank. Results of routine hematological investigations, including complete blood cell count, erythrocyte sedimentation rate, urea, and electrolytes, and liver function tests were all normal. Ultrasound examination of the abdomen revealed a 9 × 5 × 9-cm solid mass in the right flank and a single stone in the right kidney. The liver and spleen appeared normal, and there were no other masses in the abdomen or pelvis. Findings on subsequent radiologic investigations, which included chest x-ray, liver and spleen isotope scan, intravenous urogram, and barium enema, were all reported normal.At laparotomy the mass was retroperitoneal, arising from the inferior vena cava at the level of the right renal vein and extending superiorly to within 2.5 cm of the most inferior hepatic vein. The left renal vein was not involved in the tumor mass. The inferior vena cava was completely occluded. The mass was separated from surrounding structures by sharp and blunt dissection, then completely resected together with the right kidney. The two ends of the inferior vena cava were closed with a continuous suture. There were no other abnormal findings in the abdomen. Postoperative recovery was uneventful. Histopathologic examination of the resected mass showed leiomyosarcoma arising from the inferior vena cava.The patient is well and alive 23 months later with no signs of metastases or lower limb edema, and renal function is normal.Case 2A 55-year-old Saudi woman was admitted for further investigations of right flank pain, which was vague in nature and associated with indigestion and increasing constipation of 7 months' duration. Three months before admission, there was increasing puffiness of the face and swelling of both lower limbs. There was no history of jaundice or shortness of breath, nor was there any history of gynecologic problems. The patient was a known diabetic and had been treated with oral hypoglycemic drugs for the last 9 years. She was moderately obese, and apart from the puffiness of the face and minor edema of the lower limbs, the physical examination was normal.Laboratory studies showed normal blood cell count, but the ESR was high at 66 mm/h. Blood urea and creatinine were elevated at 12 mmol/L and 185 mmol/L, respectively. The creatinine clearance was 65 mL/min. The liver function tests were normal. Urine examination showed moderate proteinuria.Ultrasound examination of the abdomen revealed a hypoechoic mass measuring 6 × 14 × 7 cm, closely related to the inferior vena cava. The liver, spleen, and both kidneys were normal, and there were no other masses in the abdomen or pelvis. The CT scan showed a lobulated mass in the region of the inferior vena cava anterior to the right kidney and extending behind the head of the pancreas upward to the level of the right suprarenal gland.An isotope renogram was normal. Fine-needle aspiration cytology from the mass under ultrasound control was inconclusive. At laparotomy there were two abnormal findings: (1) 5 × 4-cm rounded firm mass at the mesenteric border of the transverse colon near the hepatic flexure, and (2) a large lobulated retroperitoneal mass arising from and occluding the inferior vena cava at the level just above the right renal vein and extending upward behind the duodenum. Mobilization of the mass from the duodenum and pancreas revealed the superior margin to be just below the entrance of the hepatic veins.It proved possible to perform a complete surgical clearance by resecting the inferior vena cava from just distal to the hepatic veins above to just proximal to the renal veins below. The two ends of the inferior vena cava were closed with a continuous suture. The two renal veins were thus entering the blind distal stump of the inferior vena cava, but it was clinically apparent that an effective collateral venous circulation had already been well established. The separate mass at the colonic mesenteric border was then excised with a wedge of the transverse colon. The colonic continuity was reestablished by end-to-end anastomosis.Histopathologic examination of the resected masses showed the inferior vena cava mass to be a leiomyosarcoma and the colonic mass to be a bilharzial granuloma. Postoperative recovery and progress were uneventful, and the patient is alive and well 12 months later. The mild lower limb edema is well controlled by elastic stockings, and renal function remains satisfactory.DISCUSSIONPerl2 first described a leiomyosarcoma of the inferior vena cava found at autopsy in 1871. It remains a rare entity, and in a recent review by Bruyninckx and Derksen,3 only 70 cases could be found in the world literature. Of these, 83% occurred in women, and this female preponderance has led to speculation as to whether the caval lesions are secondary to a primary uterine source.4 Neither of our two patients had uterine fibromyoma. The tumor can occur at any level in the inferior vena cava, and about one third are sited in the midcaval segment.Leiomyosarcoma of the inferior vena cava invariably causes diagnostic difficulty, and in only 6% of the reported cases was the lesion suspected preoperatively.3 The symptomatology is nonspecific, and ill-defined abdominal pain is the most constant complaint. A palpable abdominal mass is present in only one third of the cases. Edema of the legs or other consequences of inferior vena cava obstruction are surprisingly rare, presumably because the slow advancement of the obstructing lesion allows efficient collateral venous development.1,3,5 Tumors of the upper third of the inferior vena cava are, however, more overt and often present as a variant of the Budd-Chiari syndrome.5–12 In patients presenting with an abdominal mass, ultrasonography and CT scan should demonstrate the anatomical proximity of the tumor and inferior vena cava. Venacavography will confirm the site of origin and assess the operability. Although our experience with percutaneous fine-needle aspiration cytology was not encouraging, we believe that with increasing experience in this technique, proper preoperative histologic diagnosis will be possible. Sonographic and/or isotope liver scans and chest roentgenography are invaluable in detecting metastatic lesions in the liver and lungs, which are the commonest sites for metastasis. Laboratory investigations are of little help in suggesting the diagnosis except for abnormal values which reveal secondary disorders due to hepatic or renal venous obstruction.5,13Untreated, the lesion is ultimately fatal with widespread metastases.14,15 Surgical excision offers the only hope of palliation or even cure and is usually feasible in the mid or lower caval segments. Reconstruction of the excised vena caval segment is unnecessary because of the developed collateral circulation. In few reported cases, prosthetic replacement of the inferior vena cava was performed but with very discouraging results.16,17 In all other reported cases and in our two cases, the continuity of the inferior vena cava was not reestablished, but the procedures were tolerated well and the lower limb edema was infrequent.We conclude that surgeons should be suspicious of such a tumor involving the inferior vena cava, and surgical excision should be tried as long as the tumor does not involve the upper segment of the inferior vena cava. Adjuvant radiotherapy and/or chemotherapy may be necessary in the presence of metastatic lesions or incomplete resection.ARTICLE REFERENCES:1. Kevorkian J, Cento DP. "Leiomyosarcoma of large arteries and veins" . Surgery. 1973; 73:390–400. Google Scholar2. Perl L. "Ein fall von sarkom der vena cava inferior" . Virchows Arch Pathol Anat. 1871; 53:378–83. Google Scholar3. Bruyninckx CM, Derksen OS. 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Patel JK, Englander LS. "Leiomyosarcoma of the inferior vena cava" . J Surg Oncol. 1982; 21(4):238–40. Google Scholar16. Smith DE, Hammon J, Anane-Sefah J, et al. "Segmental venous replacement: a comparison of biological and synthetic substitutes" . J Thorac Cardiovasc Surg. 1975; 69(4):589–98. Google Scholar17. Dale WA, Harris J, Terry RB. "Polytetrafluoroethylene reconstruction of the inferior vena cava" . Surgery. 1984; 95(5):625–30. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 9, Issue 4July 1989 Metrics History Accepted10 December 1988Published online1 July 1989 InformationCopyright © 1989, Annals of Saudi MedicinePDF download