mesenchymales 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: