Transcript A RARE MEDIASTINUM TUMOR: THE PRIMARY LEIOMYOSARCOMA

A RARE MEDIASTINUM
TUMOR: THE PRIMARY
LEIOMYOSARCOMA
S.BELABBES,S.BELLASRI,S.CHAOUIR,T.AMIL,H.EN-NOUALI
Department of Radiology, Military Teaching
Hospital Mohammed V of Rabat. Morocco
CH17
INTRODUCTION
• Primary leiomyosarcoma of the thorax is a rare
malignant mesenchymatous tumor.
• It représents 1, 4% of all soft tissue sarcomas and 11% of
mediastinal primary sarcomas
• Location by decreasing frequency: lung, mediastinum
and pleura.
INTRODUCTION
• In most cases, leiomyosarcoma of the mediastinum grow at
the expense of the esophagus or great vessels
• Exceptionally, they originate from the soft tissues of the
mediastinum
• The posterior mediastinum is the preferential site of
mediastinal leiomyosarcomas.
• The imagery is used to specify the characteristics of the lesion,
its seat and extension
CLINICAL CASE
• A 35 years-old female presented with a gene respiratory
lasting for a year and left shoulder pain radiating to the
ipsilateral upper limb, without coughing or dyspnea or
dysphagia.
• Clinical examination including pleuropulmonary is
unremarkable
IMAGING DATA
chest plain radiography
shows a médiastinopulmonary left opacity with
net outer limit and lobed
IMAGING DATA
CT scan shows an
heterogeneous soft tissue mass
of middle and posterior
mediastinum, well limited,
multilobed with intimate contact
with the esophagus, the left
subclavian artery, the aortic
arch and left pulmonary pedicle
IMAGING DATA
The magnetic resonance imaging
performed to better study
vascular relationships of the
tumor , confirms the presence of a
border security with the vascular
structures of the mediastinum.
The patient was operated and the anatomopathologic examination
found a primary leiomyosarcoma
DISCUSSION
• Leiomyosarcoma is a rare mesenchymal tumor localized
preferentially to the uterus and digestive tract
• In the mediastinum: leiomyosarcoma most often
develops from smooth muscle tissue of the wall of the
esophagus, vena cava, aorta, pulmonary artery or the
trachea
• Very rarely, no origin can be individualized and
leiomyosarcoma arises from the soft tissue of the visceral
mediastinum
DISCUSSION
• The primary leiomyosarcoma of visceral mediastinum
occurs most often in young adults.
• It can remain asymptomatic for a long time.
• It can reach a large size, and is manifested by signs of
compression of adjacent organs depending on its
location.
DISCUSSION
• In Imaging, it appears as a well circumscribed tissue
mass which displaces or invades adjacent structures
with areas of central hypodensities corresponding to
areas of necrosis or hemorrhage
• The diagnosis of certitude is histological
DISCUSSION
• Surgical resection offers the only chance of cure
• The place of radiotherapy and chemotherapy is very
limited without any consensus on their use.
• survival rate at five years: from 15 to 20% after complete
surgical resection.
• Recurrences are possible and occur most frequently
during the first two or three years after operation.
CONCLUSION
• Primary leiomyosarcoma of visceral mediastinum is an
extremely rare malignancy
• it must be, however, be included in the differential
diagnosis of mediastinal tumors in young adults
• The imagery is used to specify the characteristics of the
lesion, its seat and relationship to surrounding
structures especially vascular.