Table 2 Clinical features of FET-TFCP2 rhabdomyosarcomas
Case # | Clinical symptoms | Location | Tumor size (mm) | Locoregional extension | Mets | First-line therapy | Evolution | Second-line therapy | Evolution | Third-line therapy | Evolution | Survival |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
TFCP2-1 | Headache and left exophthalmos | Sphenoid bone (left greater wing) | 90 | Dura, left orbit, and infratemporal fossa | NxM0 | 4 Cycles of etoposide, ifosfamide neoadjuvant (100%) | Progression | Resection of left orbit, sphenoid, ethmoid, and temporal bones with patch of dura (R1 micro) | Local relapse at 2 mo post surgery, metastasis of right femoral bone | 1 Cycle (cyclophosphamide, doxorubicin) and cerebral radiotherapy | Respiratory failure due to pleural effusion | DOD (15 mo) |
TFCP2-2 | Right hip pain | Sacrum | 100 | Right sacro-iliac joint and medullary involvement | NxM1 (lung, mediastinal lymphadenopathies) | 2 Cycles of IVADO (100%) | Locoregional progression and pleural effusion | 1 Cycle IVE (100%) | Local progression | 1 Cycle VAI (100%) | – | DOD (4 mo) |
TFCP2-3 | Peritoneal effusion | Peritoneum | Multiple nodules | Pleural effusion (no mass on CT nor PET-CT) | NxM0 | 1 Cycle of carboplatin, paclitaxel (100%) | Local progression and fistule post-celioscopy | 1 Cycle of adriamycin, holoxan (100%) | Lymphangitic carcinomatosis | – | – | DOD (2 mo) |
TFCP2-4 | Nodule of gingiva and hard palate of 3 cm | Mass of hard palate and upper lip | Multiple confluent nodules | Hard palate, gingiva | NxM1 (vertebra, ribs, pelvis) | 3 Cycles of VAI (100%) | Local progression | 1 Cycle of etoposide-carboplatin (100%) | Local progression | 1 Cycle of actinomycin-cyclophosphamide | Local progression and bacterial infection | DOD (8 mo) |
TFCP2-5 | Soft tissue mass and left exophthalmos | Orbito-temporo-sphenoid mass | 150 × 145 | Left orbit, sphenoid and temporal bones, dura, left infratemporal fossa, soft tissue, and dermis of left temporal region | NxM0 | 1 Cycle doxorubicin–ifosfamide (100%) | Local progression | 3 Cycles of VAC (100%) | Local progression | Radiation therapy and pazopanib | Local progression | DOD (6 mo) |
TFCP2-6 | Inguinal mass | Inguinal | 65 × 40 | NA | NxMx | Resection (hernia surgery, R1) | Local relapse at 4 mo | Palliative care | Local progression | – | – | DOD (6 mo) |
TFCP2-7 | Right thigh pain | Right femur (bifocal: neck and inter-trochanteric area) | 51 | – | NxM1 (lungs and bones:L5 vertebra, left ischio-pubic branch, ribs) | 2 Cycles of VDC-IE (100%) | Local progression (left iliac bone, sacrum) with soft tissue extension in pelvis and right hip | Pazopanib | Local progression | – | – | DOD (8 mo) |
TFCP2-8 | Cervical pain | Cervico-occipital junction | 53 × 37 × 44 | Lysis of left occipital condyle and vertebra C1, and left clivus; extension into left jugular, hypoglossar foramens, and paravertebral soft tissue | NxM0 | 2 Cycles of VDC-IE (100%) and adjuvant RT (60 Gray) | Local progression with extension to the odontoid process and into the surrounding soft tissue measuring 20 × 30 × 34 mm | Crizotinib 500 mg/d for 1 month switched to alectinib 1200 mg/d | Stable disease for 9 mo since initiation of anti-ALK therapy (32 × 29 × 42 mm) | – | – | AWD (15 mo) |
TFCP2-9 | Headache, clinical hypothesis of meningioma | Left occipital bone | 118 | Extracranial component (65 × 17 mm): soft tissue of scalp; intracranial component (53 × 16 mm): meninges, left superior sagittal sinus | NxM0 | Fragmented resection (R1 micro) | Local relapse at 1 mo post surgery | Adjuvant CT: 3 cycles of AI | Local progression and metastasis (lung, mediastinum) | – | Progression with pleural effusion and infiltration of the superior sagittal sinus | DOD (6 mo) |
TFCP2-10 | toothache | Mandible (symphysis and from teeth 37 to 44) | 45 × 32 | Vestibular gingiva, mylohyoid, and hyoglossus muscles | N0M0 | Partial mandibulectomy (R0) | – | Adjuvant CT: 5 cycles: doxorubicin, ifosfamide (100%) | Local relapse at 12 mo involving mandible and soft tissue, lung met | Adjuvant CT: 3 cycles of gemcitabine-docetaxel (100%) | – | AWD (14 mo) |
TFCP2-11 | – | Mandible | 16 × 15 × 13 | Surrounding soft tissue (muscle) | N0M0 | Surgery (R0) | – | Adjuvant CT and RT | No recurrence or metastatic disease | – | – | ANED (21 mo) |
TFCP2-12 | Local painful swelling fo 4 months | Mandible | 55 | Surrounding soft tissue (muscle) | N0M0 | Neoadjuvant CT | – | Surgery (R0), RT, adjuvant CT | No recurrence or metastatic disease | – | – | ANED (21 mo) |
TFCP2-13 | – | Maxilla | 60 | Surrounding soft tissue (muscle) | N0M0 | CT | – | Radiotherapy | Progression | – | – | DOD (unknown) |
TFCP2-14 | Local painful swelling for 1 month | Mandible | 34 | Surrounding soft tissue (muscle) | N0M0 | Surgery (R0) | – | CT | No recurrence or metastatic disease | – | – | ANED (20 mo) |
