DEFINITION AND PATHOGENESIS
- A primary or secondary (metastatic) usually moderate grade tumor arising from smooth muscle origin
- Relatively rare, accounting for ~7% of all soft-tissue sarcomas
- 9-80 years of age (mean = 52)
- M:F = 1-2.4:1
- 90% with pain averaging 11 months in duration (± pathologic fracture)
- May have a history of a 'benign' uterine fibroid (malignant degeneration in up to 1%) resected previously---review the pathology slides (2-4 years previously)
- Has been reported in a pt with a hx of retinoblastoma
- Has been reported associated (probably incidentally) with the femoral interference screw after ACL reconstruction
- (Biopsy of the lesion through the screw hole after screw removal)
- GI sxs
- Massive hematemesis reported in a gastric location
- Diaphyseal or metaphyseal
- Average lesion is 7cm in size
- Bones involved: distal femur, tibia, jaw, proximal humerus, fibula, pelvis, clavicle, ribs
- Purely lytic lesion with geographic to moth eaten margins, without sclerotic margins
- 10-15% with soft tissue mass
- ± Codman's triangle
- T1-weighted images isointense with bone
- T2-weighted images heterogeneous
- May be nearly isointense to fat on T2, hypointense to fluid
- ± soft tissue mass (esp pelvic lesions)
- Pink to yellow, firm, ± hemorrhage/necrosis
- May arise directly from blood vessels in the extremities
- 11/16 from the femoral vein
- 3/16 from the popliteal vein
- 2/16 from the posterior tibial vein
- 3-33 cm (mean 10.4 cm)
HISTOLOGIC AND MOLECULAR FEATURES
- Both primary and metastatic lesions have the same histologic appearance
- Low power: eosinophilic cell bundles with smooth muscle appearance, tending to cross at right angles
- Thin-walled vessels between thick bundles of spindle cells
- Intracytoplasmic filaments seen at high power H&E stain, on EM, with immunostaining for actomyosin, with polarizing lenses, and PTAH staining (intracellular myofilaments)
- + reticulin staining in lower grade lesions
- + to desmin, laminin, vimentin, actin, and ?-SMA staining to myoglobin staining (for rhabdomyosarcoma)
- + PAS with/without diastase
- Trichrome staining: red cytoplasm
- Saffron- or phloxine-tartrazine hematoxyin: muscle pink, collagen yellow
- ± artifactual intracytoplasmic pseudovacuaoles caused by fixation
- Cigar-shaped nuclei
- ± hyalinized areas
- Mitotic activity
- 1-3/hpf = grade I
- 3-20/hpf = grade 2
- >20/hpf = grade 3
- >20 with nuclear pleomorphism = grade 4
- Spindle cells with basal lamina and thin filaments with dense bodies indicating smooth muscle differentiation
- Similar 32K BAC array and gene expression profiling to undifferentiated pleomorphic sarcoma
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Metastatic leiomyosarcoma
- Has been reported with extramedullary spinal cord compression from a missed dx of uterine leiomyosarcoma
DISEASE COURSE AND TREATMENT
- Women require work up for a primary uterine leiomyosarcoma (review of previous slides, pelvic exam, CT of the pelvis, hysterectomy if warranted)
- All pts warrant evaluation for a silent primary in the retroperitoneum or GI tract
- Inferior vena cava lesions txd with neoadjuvant external-beam XRT (45-50 Gy) and then en bloc resection
- No reconstruction of the inferior vena cava was performed
- Lung and skeletal metastases with 50% 5 year survival warrants wide resection in solitary lesions with adjunctive chemotherapy
- Overall survival at 5 yrs of pts whose tumors arise directly from blood vessels is 25%
- Predictors of metastases
- Other areas of metastases
PRIMARY DURAL LEIOMYOSARCOMA
- Reported without uterine primary
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