A vena cava inferior leiomyosarcomája

Pinczés István, Kovács Attila, Kardos Magdolna, Gara Ákos, Gyory Gabriella, F. Szalay, G. Speer

Research output: Article

Abstract

INTRODUCTION - Primary neoplasm of the great blood vessels is rarely reported. The tumour most commonly presents in the inferior vena cava. In general, the prognosis is very poor, since intimal sarcomas metastasise early, and mural sarcomas grow silently and remain hidden for a long time. Also, because this type of tumour is so uncommon, it is often not considered for diagnosis. CASE REPORT - We report on the case of a 77-year-old woman with progressive symmetric oedema of the lower limbs. The investigations revealed a tumour thrombus that obliterated the inferior vena cava in its entire length. No surgery was possible because of the advanced stage of the disease. Later, Budd-Chiari syndrome developed and the patient died of progressive hepatic failure. The tumour was initially thought to be that of extravascular origin breaking into the vein. However, autopsy revealed a primary tumour of vascular origin, which histologically proved to be a leiomyosarcoma. CONCLUSION - Today, the recognition of the insidiously growing vascular mural sarcomas is assisted by an entire diagnostic arsenal. It is important, however, that the possibility of this rare disease is considered in time to permit surgical removal, the only treatment that can prolong survival. The authors would like to contribute to this by presenting this rare clinical case.

Original languageHungarian
Pages (from-to)133-136
Number of pages4
JournalLege Artis Medicinae
Volume18
Issue number2
Publication statusPublished - febr. 2008

Fingerprint

Leiomyosarcoma
Inferior Vena Cava
Sarcoma
Blood Vessels
Neoplasms
Tunica Intima
Budd-Chiari Syndrome
Liver Failure
Rare Diseases
Lower Extremity
Autopsy
Veins
Edema
Thrombosis
Survival

Keywords

  • Budd-Chiari syndrome
  • Inferior vena cava
  • Leiomyosarcoma

ASJC Scopus subject areas

  • Medicine(all)

Cite this

István, P., Attila, K., Magdolna, K., Ákos, G., Gabriella, G., Szalay, F., & Speer, G. (2008). A vena cava inferior leiomyosarcomája. Lege Artis Medicinae, 18(2), 133-136.

A vena cava inferior leiomyosarcomája. / István, Pinczés; Attila, Kovács; Magdolna, Kardos; Ákos, Gara; Gabriella, Gyory; Szalay, F.; Speer, G.

In: Lege Artis Medicinae, Vol. 18, No. 2, 02.2008, p. 133-136.

Research output: Article

István, P, Attila, K, Magdolna, K, Ákos, G, Gabriella, G, Szalay, F & Speer, G 2008, 'A vena cava inferior leiomyosarcomája', Lege Artis Medicinae, vol. 18, no. 2, pp. 133-136.
István P, Attila K, Magdolna K, Ákos G, Gabriella G, Szalay F et al. A vena cava inferior leiomyosarcomája. Lege Artis Medicinae. 2008 febr.;18(2):133-136.
István, Pinczés ; Attila, Kovács ; Magdolna, Kardos ; Ákos, Gara ; Gabriella, Gyory ; Szalay, F. ; Speer, G. / A vena cava inferior leiomyosarcomája. In: Lege Artis Medicinae. 2008 ; Vol. 18, No. 2. pp. 133-136.
@article{da34a9f571be4c3f8082773910d961ef,
title = "A vena cava inferior leiomyosarcom{\'a}ja",
abstract = "INTRODUCTION - Primary neoplasm of the great blood vessels is rarely reported. The tumour most commonly presents in the inferior vena cava. In general, the prognosis is very poor, since intimal sarcomas metastasise early, and mural sarcomas grow silently and remain hidden for a long time. Also, because this type of tumour is so uncommon, it is often not considered for diagnosis. CASE REPORT - We report on the case of a 77-year-old woman with progressive symmetric oedema of the lower limbs. The investigations revealed a tumour thrombus that obliterated the inferior vena cava in its entire length. No surgery was possible because of the advanced stage of the disease. Later, Budd-Chiari syndrome developed and the patient died of progressive hepatic failure. The tumour was initially thought to be that of extravascular origin breaking into the vein. However, autopsy revealed a primary tumour of vascular origin, which histologically proved to be a leiomyosarcoma. CONCLUSION - Today, the recognition of the insidiously growing vascular mural sarcomas is assisted by an entire diagnostic arsenal. It is important, however, that the possibility of this rare disease is considered in time to permit surgical removal, the only treatment that can prolong survival. The authors would like to contribute to this by presenting this rare clinical case.",
keywords = "Budd-Chiari syndrome, Inferior vena cava, Leiomyosarcoma",
author = "Pincz{\'e}s Istv{\'a}n and Kov{\'a}cs Attila and Kardos Magdolna and Gara {\'A}kos and Gyory Gabriella and F. Szalay and G. Speer",
year = "2008",
month = "2",
language = "Hungarian",
volume = "18",
pages = "133--136",
journal = "Lege Artis Medicinae",
issn = "0866-4811",
publisher = "Literatura Medica Publishing House",
number = "2",

}

TY - JOUR

T1 - A vena cava inferior leiomyosarcomája

AU - István, Pinczés

AU - Attila, Kovács

AU - Magdolna, Kardos

AU - Ákos, Gara

AU - Gabriella, Gyory

AU - Szalay, F.

AU - Speer, G.

PY - 2008/2

Y1 - 2008/2

N2 - INTRODUCTION - Primary neoplasm of the great blood vessels is rarely reported. The tumour most commonly presents in the inferior vena cava. In general, the prognosis is very poor, since intimal sarcomas metastasise early, and mural sarcomas grow silently and remain hidden for a long time. Also, because this type of tumour is so uncommon, it is often not considered for diagnosis. CASE REPORT - We report on the case of a 77-year-old woman with progressive symmetric oedema of the lower limbs. The investigations revealed a tumour thrombus that obliterated the inferior vena cava in its entire length. No surgery was possible because of the advanced stage of the disease. Later, Budd-Chiari syndrome developed and the patient died of progressive hepatic failure. The tumour was initially thought to be that of extravascular origin breaking into the vein. However, autopsy revealed a primary tumour of vascular origin, which histologically proved to be a leiomyosarcoma. CONCLUSION - Today, the recognition of the insidiously growing vascular mural sarcomas is assisted by an entire diagnostic arsenal. It is important, however, that the possibility of this rare disease is considered in time to permit surgical removal, the only treatment that can prolong survival. The authors would like to contribute to this by presenting this rare clinical case.

AB - INTRODUCTION - Primary neoplasm of the great blood vessels is rarely reported. The tumour most commonly presents in the inferior vena cava. In general, the prognosis is very poor, since intimal sarcomas metastasise early, and mural sarcomas grow silently and remain hidden for a long time. Also, because this type of tumour is so uncommon, it is often not considered for diagnosis. CASE REPORT - We report on the case of a 77-year-old woman with progressive symmetric oedema of the lower limbs. The investigations revealed a tumour thrombus that obliterated the inferior vena cava in its entire length. No surgery was possible because of the advanced stage of the disease. Later, Budd-Chiari syndrome developed and the patient died of progressive hepatic failure. The tumour was initially thought to be that of extravascular origin breaking into the vein. However, autopsy revealed a primary tumour of vascular origin, which histologically proved to be a leiomyosarcoma. CONCLUSION - Today, the recognition of the insidiously growing vascular mural sarcomas is assisted by an entire diagnostic arsenal. It is important, however, that the possibility of this rare disease is considered in time to permit surgical removal, the only treatment that can prolong survival. The authors would like to contribute to this by presenting this rare clinical case.

KW - Budd-Chiari syndrome

KW - Inferior vena cava

KW - Leiomyosarcoma

UR - http://www.scopus.com/inward/record.url?scp=39849104023&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=39849104023&partnerID=8YFLogxK

M3 - Article

AN - SCOPUS:39849104023

VL - 18

SP - 133

EP - 136

JO - Lege Artis Medicinae

JF - Lege Artis Medicinae

SN - 0866-4811

IS - 2

ER -