Table 1.
Clinical details of 20 patients with SCOS.
| Case | Sex | Age (year) | Presentation (duration) | Location (site in long bone) | Maxium size (cm) | Enneking Staging | ALP | LDH | Treatment | Chemotherapy Protocol | Response to chemotherapy | Margin of resection | Metastasis (interval) | Recurrence (interval) | Follow-up (duration) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 21 | Pain (2 m) | R prox fum (meta) | 7.8 | IIB | nor | nor | NC + WRA + C | DOX | good | wide | n | n | ANED (126 m) |
| 2 | F | 29 | Pain (2 m) | L dist fum (meta) | 2.0 | IIB | nor | nor | NC + WRA + C | THP | good | wide | n | n | ANED (123 m) |
| 3 | F | 39 | Pain (3 m) | L mid tibia (dia) | 3.9 | IIB | nor | nor | NC + EPR + C | DOX | good | radical | lung (95 m) | n | AWD (99 m) |
| 4 | M | 48 | Pain (24 m) | L prox tibia (mata) | 9.9 | IIB | abn | abn | NC + amputation | DOX | poor | wide | n | n | ANED (7 m) |
| 5 | M | 14 | Injury (2d) | R diat fum (meta) | 8.6 | IIB | abn | abn | NC + WRA + C + amputation | THP | poor | wide + intralesional | lung (15 m) | 2 m; 8 m | DOD (16 m) |
| 6 | M | 60 | Swelling (5 m) | R diatal tibia (meta) | 5.2 | IIB | abn | nor | NC + WRA + C + amputation | THP | poor | wide | multiple (18 m) | 10 m | DOD (37 m) |
| 7 | M | 25 | Swelling (5 m) | R prox hum (meta) | 9.4 | IIB | nor | abn | NC + WRA + C | DOX | good | wide | n | n | ANED (85 m) |
| 8 | M | 31 | Mass (4 m) | R pelvis | 7.5 | IIB | abn | nor | NC + Te + C + R | DOX | poor | marginal | lung (3 m) | 3 m | DOD (6 m) |
| 9 | M | 49 | Swelling (1 m) | L dist fum (meta) | 6.4 | III | abn | nor | NC + WRA + C | THP | poor | wide | n | n | ANED (70 m) |
| 10 | F | 15 | Pain (6 m) | R prox tibia (dia) | 4.4 | III | nor | nor | NC + WRA + C | DOX | good | wide | n | n | ANED (52 m) |
| 11 | M | 19 | Mass (1 m) | R pelvis | 8.9 | III | abn | abn | NC + TCAE + EPR + C | VAC/IE, DOX | good | marginal | bone (4 m) | n | DOD (40 m) |
| 12 | M | 17 | Pain (8 m) | L pelvis | 18.3 | III | abn | abn | NC + amputation | DOX | poor | marginal | n | n | DOD (4 m) |
| 13 | F | 20 | Mass (4 m) | L mid-dist fum (dia) | 5.8 | IIB | nor | nor | NC + resection + C | DOX | poor | radical | n | n | ANED (53 m) |
| 14† | M | 18 | Pain (1 m) | L dist fum (dia) | 9.5 | IIB | abn | nor | NC + WRA + C + amputation | THP | poor | wide + marginal | lung (2 m) | 11 m | DOD(12 m) |
| 15 | M | 25 | Pain (4 m) | R pelvis | 14.1 | III | abn | abn | NC | DOX | poor | n | lung (1 m) | n | DOD(7 m) |
| 16‡ | M | 40 | Pain (9 m) | L dist fum (dia) | 18.8 | III | abn | abn | NC + hemipelvectomy + C | THP | poor | intralesional | lung (7 m) | n | DOD (6 m) |
| 17 | M | 8 | Pain (2 m) | R diat fum (dia) | 4.2 | IIB | abn | abn | NC + resection + C | DOX | poor | radical | lung (9 m) | 11 m | DOD (17 m) |
| 18 | M | 19 | Pain (3 m) | R prox tibia (meta) | 8.5 | IIB | nor | nor | NC + resection + C | DOX | poor | radical | lung (1 m) | 15 m | DOD (21 m) |
| 19 | M | 14 | Pain (1 m) | R prox fum (meta) | 11.2 | IIB | nor | nor | NC + WRA + C | DOX | good | wide | n | n | AWD (21 m) |
| 20 | M | 69 | Pain (12 m) | L pelvis | 27.2 | III | abn | abn | Te | n | n | intralesional | n | n | DOD (7 m) |
Note: †family history of cancer; ‡previous history of sarcoma treated by radiation.
Abbreviations: abn, abnormal; ANED, alive with no evidence of disease; AWD, alive with disease; C, adjuvant chemothrapy; dia, diaphysis; dist, distal; DOD, died of disease; EPR, limb-salvage surgery with endoprosthetic replacement of resected bone; fum, fumer; hum, humerus; L, left; meta, metaphysis; mid, middle; m, months; n, none; NC, neo-adjuvant chemotherapy; nor, norMEmal; prox, proximal; r, radiotherapy; R, right; SCOS, small cell osteosarcoma; TCAE, transcutaneous arterial embolization; Te, tumor exclusion; WRA, wide resection and arthroplasty; y, years; DOX, doxorubicin-based osteosarcoma directed chemotherapy protocol; THP, pirarubicin-based osteosarcoma directed chemotherapy protocol; VAC/IE, an Ewing sarcoma directed chemotherapy protocol with vincristine, doxorubicin, cyclophosphamide, dactinomycin, ifosfamide and etoposide.