Table 1.
Clinical, histopathologic and immunohistochemical features of pediatric chordomas
| Case no | 1 | 2 | 3 | 4 | 5 | 6 | 7 | Recc | 8 | 9 | Recc |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Age(years)/Sex | 7/M | 8/F | 4/M | 10/M | 12/M | 6/M | 18/M | 2/F | 6/M | ||
| Clinical presentation | Neck pain | Headache, gait instability | Neck pain | Neck pain | Diminution of vision | Neck pain, weakness limbs | Headache, proptosis, loss of vision | Neck swelling | Nasal obstruction, proptosis | ||
| Duration (months) | 8-9 | 6 | 18 | 4 | 5 | 3 | 8 | 8 | 3 | 4 | 6 |
| Site | Clivus | Clivus | Clivus | Clivus | Clivus | Clivus | Clivus | Neck | Rt nasal | ||
| Architecture | Solid | Lobular | Solid | Lobular | Lobular | Solid | Solid | Solid | Solid | Lobular | Lobular |
| Cellularity | C | N | C | N | N | C | C | C | C | N | N |
| Stroma | S | A | S | A | A | S | S | S | S | A | A |
| Mitoses/10 hpf | 3–4 | 0–1 | 4 | 2 | 0–1 | 2–3 | 9 | 9–10 | 2 | 0–1 | 0–1 |
| Necrosis (%) | +(40) | − | +(30) | +(40) | − | +(30) | − | +(30) | − | − | − |
| Vascular invasion | − | − | − | − | − | + | − | − | − | − | − |
| Cytologic atypia | +(vn) | − | +(vn) | +(sh) | +(sh) | +(vn) | +(vn) | +(vn) | +(vn) | − | − |
| Category | PD | Conv | Atyp | Conv | Conv | Atyp | Atyp | Atyp | Atyp | Conv | Conv |
| P53 | + | − | + | − | + | + | + | + | + | + | + |
| INI1 IHC | − | − | − | − | + | − | − | − | − | + | + |
| INI1 FISH | loss | No tissue | No tissue | loss | Not done | Loss | loss | No tissue | Not done | ||
| INI1 Sequencing | No mut | No tissue | No tissue | No mut | Not done | No mut | No mut | No tissue | Not done | ||
| MIB-1 LI (%) | 5 | 2 | 6 | 3 | 2 | 7–8 | 12–13 | 15 | 4 | 3 | 2 |
| EGFR | + | + | + | + | + | + | + | + | + | + | + |
| Follow up | SD for 2mths and lost thereafter | SD 75 mths | NA | Expired | SD 57 mths | Expired | Recurrence at 3 mths, SD for 3 mths and lost thereafter | Expired | Recurrence at 4 mths & SD 16 mths | ||
Abbreviations: A, abundant; Atyp, atypical; C, hypercellular; Conv, conventional, F; female; IHC, immunohistochemistry; Lt, left; M, male; mths, months; mut, mutation; N, normocellular; NA, not available; PD, poorly differentiated; Recc, recurrent; Rt, right; S, scant; SD, stable disease; sh, smudgy hyperchromatic; vn, vesicular chromatin with nucleoli.