retroperitoneales Leiomyosarkom
Retroperitoneal leiomyosarcoma, leiomyosarcoma arising within the retroperitoneum, is one of the commonest primary retroperitoneal neoplasms. The retroperitoneum is considered the most common extrauterine site for leiomyosarcoma.
Epidemiology
They are more common in women.
Clinical presentation
Symptoms are often non-specific with abdominal pain being one of the most frequent complaints. The tumors can reach large sizes at the time of detection.
Pathology
Etiology
They arise from smooth muscle within arteries, veins, or bowel.
Location
The most frequent site is the inferior vena cava (50%), which is discussed separately: inferior vena cava leiomyosarcoma.
They can be:
- completely extra-vascular: 62%
- completely intra-vascular: 5%
- have extra- and intra-luminal components: 33%
Metastases to the liver and lung are common when the tumor is intravascular.
Radiographic features
Like other sarcomas, imaging findings are non-specific. Retroperitoneal leiomyosarcomas tend to develop massive cystic components which undergo degeneration. Unlike other sarcomas, fat and calcification are not typically present.
Contiguous involvement of a vessel may clear and would favor this over other types of sarcoma.
MRI
- T1: intermediate to low signal intensity
- T2: intermediate to high signal intensity
Differential diagnosis
- undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma MFH)
- liposarcoma
- metastases
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Assoziationen und Differentialdiagnosen zu retroperitoneales Leiomyosarkom:
