Leiomyosarcoma
Leiomyosarcoma
Updated: 08/07/2020
© Jun Wang, MD, PhD
General features
- Malignant tumor of smooth muscle
- Commonly in uterus, skin, subcutis, deep soft tissue and GI
- Third most common retroperitoneal sarcoma after liposarcoma and undifferentiated pleomorphic sarcoma
- Aggressive clinical behavior
- Most common metastasis site: lung
Clinical presentations
- Mass
- Symptoms depending on location
Key morphological features
- Large tumor
- Hypercellular, hyperchromic spindle cells
- Pleomorphic cells, usually marked cytological atypia
- Necrosis, active mitosis, atypical mitosis
Markers
- Positive for desmin, smooth muscle actin
Genetic abnormalities
- Complex, commonly p53 mutation and p16 overexpression
Treatment
- Excision
- Chemotherapy
- Radiation
Poor prognosis indicators
- Retroperitoneal, mesenteric or other deep location
- > 5 cm, except uterus
- > 65 years
- C-myc expression
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