Table 1.
Patient and tumor characteristics, treatment, and outcome.
| Pat. no. | Age | Sex | Tumor site | Tumor size (ml) | Primary mets. | AP | LDH | Pre-op chemotherapy | Surgical remission | Type of surgery | Tumor response | Postoperative chemotherapy | Event | Further therapy | EFS (years) | OAS (years) | Status |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 10 | F | Tarsus (calcaneus) | 40.56 max.; dimension: 6.5 cm | None | N | N | A, M, I, P | CR | Amputation lower leg below knee | Poor | A, M, I, E | SMD (Ewing sarcoma) | 8.9 | 8.9 | DOC-SMD | |
| 2 | 12 | F | Tarsus (os cuneiforme lateral) | 5.5 max.; dimension 2.5 cm | None | N | N | A, M, I, P | CR | Amputation forefoot in Chopart joint | Good | A, I, P | None | 13 | 13 | LFU-CR1 | |
| 3 | 12 | M | Tarsus (talus: position in the extensor aspect) | 12.08 max.; dimension 3 cm | None | — | — | A, M, I, P | CR | Resection of talus, half os naviculare, and distal part of lateral calcaneus incl. sinus tarsi; reimplantation of proximal talus: tumor in 1 cm distance | Poor | A, M, I, P | None | 11 | 11 | LFU-CR1 | |
| 4 | 13 | M | Tarsus (talus medial part) | 31.2 max.; dimension 5 cm | 2–5 lung | E | E | A, M, I, P | No | En bloc resection distal part of tibia, fibula and talus | Poor | A, P, E, HD-I | No CR progressive lung mets., local rec. | n.f.s. | 0 | 0.7 | DOD-primary disease |
| 5 | 13 | M | Tarsus (calcaneus) | 36.11 max.; dimension 6.5 cm | None | E | N | A, M, I, P | CR | Amputation of foot: exarticulation upper ankle joint | Poor | A, M, I, P | None | 7.6 | 7.6 | LFU-CR1 | |
| 6 | 13 | M | Tarsus (calcaneus) | 54 max.; dimension 8 cm | None | E | N | A, M, P | CR | Amputation lower leg below knee | Poor | A, M, P | None | 0.86 | 0.86 | CR1 | |
| 7 | 23 | M | Tarsus (calcaneus) | — | None | N | N | A, M, P | CR | Amputation lower leg below knee | Poor | A, M, P | None | 0.9 | 0.9 | LFU-CR1 | |
| 8 | 25 | F | Tarsus (calcaneus) | — | None | N | N | COSS 96, n.f.s | CR | Amputation lower leg below knee | — | n.f.s. | None | 4.8 | 4.8 | LFU-CR1 | |
| 9 | 25 | F | Tarsus (calcaneus) | — | None | N | N | A, M, I, P | CR | First operation: excochleation of cyst; second operation: amputation of lower leg below knee | Poor | A, M, I, P | Lymph node metastases ipsilateral groin | Second- and third-line chemotherapy | 0.8 | 1.4 | DOD-Rec1 |
| 10 | 29 | F | Tarsus (calcaneus) | — | None | E | N | A, M, P | No | Amputation lower leg n.f.s | Poor | A, M, I, P, E | No CR: pulmonary filiae during preoperative chemo, mets. HWK4 and BWK 3 | Corporectomy of HWK4 and BWK3, mets. Os pubis, fourth rib | 0 | 2.1 | DOD-primary disease |
| 11 | 32 | M | Tarsus (os naviculare + cuneiforme III) | — | Lymph node; left groin | N | E | A, M, I, P | CR | Amputation: foot through calcaneus; resection of groin metastases | Poor | A, M, I, P | Rec. lower leg and lung mets. | Resection of distal fibula | 2.2 | 4.2 | DOD-Rec2 |
| 12 | 33 | F | Tarsus (os naviculare) | — | None | E | E | A, M, I, P | CR | Amputation of forefoot | Good | A, I, P, E, M | Lung mets. | High-dose chemo rejected | 4.9 | 5.0 | LFU-Rec1 |
| 13 | 38 | M | Tarsus (calcaneus) | 32 (5 × 5 × 2.5 cm) | None | N | N | A, M, I, P | CR | First operation: intralesional excochleation of a “cyst,” second operation: amputation lower leg below knee | N.A. (primary OP) | A, M, I, P | Lung mets. 3x | 2 × metastasectomies | 2.2 | 4.1 | DOD-Rec3 |
| 14 | 38 | F | Tarsus (calcaneus) | 51 max.; dimension 7 cm | None | N | — | A, M, I, P | CR | Amputation lower leg below knee | Poor | A, M, I, P | None | 1.1 | 1.1 | LFU-CR1 | |
| 15 | 56 | M | Tarsus (talus) | — | None | N | N | A, P, I | CR | Amputation distal lower leg | Good | A, P, I | None | 2.0 | 2.0 | LFU-CR1 | |
| 16 | 58 | M | Tarsus (calcaneus) | — | None | N | N | — | CR | First operation: resection of calcaneal cyst: second operation: amputation lower leg below knee | N.A. (primary OP) | A, P, I | None | 0.4 | 0.4 | LFU-CR1 | |
| 17 | 11 | F | Metatarsale IV: proximal right part | — | None | N | N | A, P, M | CR | Resection ray IV, metatarsale III and V reconstruction of axis III with fibula | Poor | A, P, M, E, I | None | 4.0 | 4.0 | LFU-CR1 | |
| 18 | 39 | F | Metatarsale IV: mediocranial | 3.31 max.; dimension 3.5 cm | None | — | — | — | CR | Partial resection of ray III–V through cuboid and cuneiforme III | N.A. (primary OP) | A, I, P, M | Lung mets. | Rec. I–III: 3 × wedge resection of affected pulmonary site | 5.3 | 18.45 | CR3 |
| 19 | 44 | M | Metatarsale II, os cuneiforme II and III | — | None | N | N | A, I, P | CR | Amputation forefoot in Chopart joint | Poor | A, P, I | None | 1.6 | 1.6 | CR1 | |
| 20 | 44 | M | Metatarsale V | — | Lung | N | N | A, P, I | CR | First operation: amputation atypical in Chopart joint; second operation: wedge resection left lower lobe | Good | I, P, M, A | Lung mets. bilateral | No further therapy: patient will | 1.5 | 4.72 | DUC-Rec1 |
| 21 | 45 | M | Metatarsale I | — | None | N | N | A, P, I | CR | Amputation ray I and II and os naviculare | Poor | A, P, I | None | 2.0 | 2.0 | LFU-CR1 | |
| 22 | 45 | F | Phalanx I: proximal part | — | None | — | — | None; patient disbelieved diagnosis | CR | Amputation ray I and II right | N.A. (primary OP) | None | Rec. 1: lung mets., Rec. 2: lung + popliteal fossa, and Rec. 3: lymph node mets.: lung, mediastinal, and abdominal | 2x wedge resection, amputation right leg | 1.5 | 6.6 | DOD-Rec3 |
| 23 | 57 | M | Phalanx distalis I | 31 max.; dimension 5 cm | None | N | N | — | CR | Amputation phalanx I | N.A. (primary OP) | A, P, I | None | 1.6 | 1.6 | LFU-CR1 |
Pat. = patient; No. = number; status = status at the last available follow-up; F = female; M = male; N = normal; E = elevated; N = normal; A = doxorubicin; M = methotrexate; I = ifosfamide; HD = high dose; P = cisplatin; E = etoposide; CR = complete surgical remission (primary tumor and metastases); good/poor tumor response = </≥10% viable tumor following pre-op. chemotherapy; SMD = secondary malignant disease; n.f.s. = not further specified; mets. = metastases; OP = operation; N.A. = not applicable; DOC = death of other cause; LFU = lost to follow-up; DOD = death of disease; Rec. = recurrence.