Inferior vena cava leiomyosarcoma: Establishment of an international registry

Correspondence

The upper segment of the IVS is less frequently involved. In the world series only 34 patients (24.3%) presented with a tumour arising from this portion, whereas 49 (34.0%) and 59 (41.7%) patients were affected by a tumour of the lower and middle segments respectively. Local spread of this tumour to the surrounding organs is uncommon. A non infiltrating growth is in fact characteristic of IVC leiomyosarcoma. Only two out of eighty-two patients (2.4%) receiving a radical operation presented with local spread to the omentum in one case and to stomach, duodenum, jejunum and pancreas in the other. Among the 14 patients submitted to a palliative resection, infiltration of the surrounding tissues was only noted in two patients (14.3%). Moreover among the 47 patients in w h o m the diagnosis was made at exploratory laparotomy or autopsy, only seven cases (18.9%) presented with a local tumour infiltration. Metastasis to the liver and/or lungs were present in 13 out of these 47 patients (27.6%), Unfortunately tumour debulking does not offer good palliation. Survival of the 14 patients receiving this treatment was very short: 10 patients had died at a median of 3 months (1-56 months). A wide resection of the IVC when the tumour involves the infrarenal portion is possible but not justified. No difference in the outcome and in the incidence of local recurrence was noted in the world series among the three different types of radical tumour resection (caval rim resection and segmental caval resection with or without resection of adjacent organs). Long-term results of radical resection are better. The overall 5 and 10-year survival rates were respectively 27.9% and 14.2%. However patients with tumour of the middle segment fared better than those with lower segment involvement. In 1991 only 19 out of 82 patients were reported as alive (12 patients free of disease at a mean follow-up of 63.1 + 56.9 months and seven patients with recurrent disease at a mean follow-up of 84 + 36 months). Most of the patients with upper segment tumour were inoperable (median survival 1 month). Only three patients have been radically operated on, 3-5 in two'cases with a

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prosthetic IVC replacement and right hepatic lobe resection for the involvement of the right hepatic vein (trisegmentectomy in one 3 and complete lobectomy in the other 4) and in the third case with a wall rim resection and Dacron patch. The reported survival of these patients was 24, 48 and 10 months, respectively. In conclusion we would like to emphasise the importance of the International Registry of IVC leiomyosarcoma. It is a real opportunity to study the pathogenesis and natural history of this tumour and develop the most appropriate treatment. Requirements for enrolling patients in the Registry include sending the patients' clinical record along with the histologic slides from the tumour specimen. For registration of cases, please contact Andrea Mingoli, M.D., 1st Dept. of Surgery, La Sapienza University, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy. Andrea Mingoli, Antonino Cavallaro, Richard J. Feldhaus*, Luca di Marzo, Maria M. Morelli and Vincenzo Sciacca

1st Department of Surgery, La Sapienza University, Rome, Italy and *Creighton University, Omaha, Nebraska.

References 1 THOMPSONMM, GRAHAMTR, BOL1AAA, [FIRMINRK, BELLPRF. Intrahepatic leiomyosarcoma of the inferior vena cava with extension into the right atrium. Eur J Vasc Surg 1993; 7: 204-207. 2 MINGOLI A, FELDHAUS RF, CAVALLAROA, STIPA S. Leiomyosarcoma of the inferior vena cava: analysis of a world series of 141 patients and report of three new cases. J Vasc Surg 1991; 14: 688-699. 3 IWATSUKIS, TODO S, STARZLTE. Right trisegmentectomy with a synthetic vena cava graft. Arch Surg 1988; 123: 1021-1022. 4 BOUSQUETJC, GOZE A, HASSANM, KIEFFERR, CURETP, PELLETJ. Leiomyosarcoma of the inferior vena cava. Ultrasonographic appearance. J Ultrasound Med 1987; 6: 7-12. 5 SMITH BM, MULHERINJL, SAWYERSJL, TURNER BI, PRAGERRL, PAN RH. Suprarenal vena caval occlusion. Principles of operative management. Ann Surg 1984; 199: 656-668.

Eur J Vasc Surg Vol 8, May 1994