mesenchymales Hamartom der Brustwand

mesenchymal hamartoma of the chest wall
thoracic mesenchymoma
mesenchymal hamartomas of the chest wall
Mesenchymal hamartomas of chest wall
Chest wall mesenchymal hamartoma
Mesenchymoma of chest wall
Chrondromatous hamartoma of chest wall
chondromesenchymal hamartoma of the chest wall
chondromesenchymales Hamartom der Brustwand

A mesenchymal hamartoma of the chest wall is a very rare benign chest wall tumor. It is sometimes classified as a non-neoplastic developmental anomaly rather than a tumor.
Epidemiology
They typically present in neonates or infants .
Clinical presentation
Many present with a chest wall mass . Some may present with respiratory distress or a chest wall deformity .
Pathology
Macroscopically the tumors are generally well delineated, lobulated, and tan to reddish in color .
Microscopically, they tend to show showed alternating areas of cartilaginous islands and primitive appearing mesenchymal proliferation. There may be also areas of bone formation and blood-filled cystic spaces (secondary aneurysmal bone cyst formation).
Location
Many lesions arise from the ribs. Some can have multifocal disease.
Radiographic features
General
Radiologically they are well-circumscribed masses with solid and cystic components. They may have multiple fluid-fluid levels, in association with single or multiple rib destruction or change.
Plain radiograph
Plain radiographs may show a well-defined, partly calcified mass involving one or more ribs.
MRI
MRI usually shows heterogeneous signal intensities of the mass on both T1- and T2-weighted images. Hemorrhagic cavities (secondary aneurysmal bone cyst regions) are readily detectable in MRI .
Treatment and prognosis
Rapid growth may occur but usually is self-limited. While the general rule is that they are they are non-neoplastic, occasional cases of malignant transformation have been reported . En bloc resection is curative, but the large residual chest wall defect can frequently result in scoliosis .
See also
Siehe auch:
