TABLE.
Clinicopathologic Features and CTNNB1 Status in Patients With NMC-DTF
| CTNNB1 status | |||||||
|---|---|---|---|---|---|---|---|
| Patient | Age/sex | Initial clinical presentation: (NMC or NMC-DTF-related) | Location of NMC and location and size of NMC-DTF (cm) | NMC | NMC-DTF | Treatment chronology | Clinical outcome (follow-up duration, yr) |
| 1 | 15/F | Leg length discrepancy, foot drop (NMC) | Right sciatic nerve/right popliteal fossa (6.5 × 5.4 × 6.5) | NA | c.134 C > T p.S45F | Treatment 1: cryotherapy (X 4) | Local progression |
| Treatment 2: vinblastine and methotrexate | Local progression | ||||||
| Treatment 3: sorafenib (with dose escalation) + cryotherapy | Local progression (3 yr) | ||||||
| 2 | 34/F | Lower extremity pain, posterior thigh mass (NMC-DTF) | Right sciatic nerve/right posterior thigh (4.7 × 4.8 × 16.2) and calf (metachronous NMC-DTF, 7.0 × 3.6 × 18.0) | NA | c.133 T > C p.S45P | Treatment 1: surgical resection | Recurrence at 8 mo |
| Treatment 2: tamoxifen (6 mo) | Disease stable | ||||||
| Treatment 3: tamoxifen and NSAID | Disease stable | ||||||
| Treatment 4: radiation therapy | Primary disease stable. Second, distal NMC-DTF in the calf | ||||||
| Treatment 5 (metachronous NMC-DTF) capecitabine × 10 mo Radiation therapy (tomotherapy) | Disease stable (6 yr) | ||||||
| 3 | 20/M | Enlarging popliteal mass (NMC-DTF) | Left sciatic nerve/left popliteal fossa (7.6 × 8.1 × 13) | NA | c.134 C > T p.S45F | Treatment 1: surgical resection | Local progression at 9 mo |
| Treatment 2: radiation therapy | Disease stable (1 yr) | ||||||
| 4 | 51/M | During w/u for hip replacement (congenital dysplasia), old foot drop (NMC) | Left sciatic nerve/left sciatic notch (9.5 × 6.4 × 5.8) | c.134 C > T p.S45F | c.134 C > T p.S45F | Observation | Local progression (2 yr) |
| 5 | 18/M | Ankle fracture, foot drop, pain (NMC) | Right sciatic nerve/right posterior mid-thigh (6.0 × 9.0 × 12.0) | c.134 C > T p.S45F | c.134 C > T p.S45F | Treatment 1: surgical resection | Recurrence at 6 mo |
| Treatment 2: radiation therapy (28 fractions of 5600 cGy) | Disease stable (8 yr) | ||||||
| 6 | 14/M | Gait disturbance, leg length discrepancy, muscle atrophy (NMC) | Right sciatic nerve/right posterior mid-thigh (4.6 × 4.3 × 7.1) | c.134 C > T p.S45F | c.134 C > T p.S45F | Treatment 1: tamoxifen + sulindac × 3 mo | Local progression |
| Treatment 2: doxorubicin (8 cycles/8 mo) | Disease stable (6 yr) | ||||||