For many surgeons amputation is the usual treatment in sarcoma of the foot. The aim of our study was to report the functional and oncologic results of treatment in 54 sarcomas of the foot to assess if conservative treatment was acceptable

We retrospectively reviewed the records of 54 patients with sarcomas of the foot, aged 6 to 50 (mean 17), 30 females and 26 males. At time of referral, 18 had a local recurrence of a previous inadequate treatment. There were 27 soft tissue sarcomas (STS: 10 synovial sarcomas, 6 rhadomyosarcomas, 1 liposarcomas and 10 others) and 27 bone tumours (16 Ewing's, 8 chondrosarcomas, 3 osteosarcomas). Toes tumours were excluded, 18 tumours involved the metatarsal, 12 the plantar soft tissues, 11 the calcaneum, 3 the talus, 2 the midtarsal bones.

Surgery consisted in 19 resection without reconstruction, 21 resections with bone reconstruction, 9 partial amputations of the foot, and 6 trans tibial amputations.

In 34 cases surgical margins were adequate (R0), in 13 patients resection was inadequate (9 R1 and 4 R2). In 7 cases the margins were not assessed.

After a 5.5 years average follow-up (3m to 17y), 31 patients had no evidence of disease, 8 were in second remission, 4 had an evolutive disease and 11 were deceased. The mean MSTS score was 26/30 (31 cases).

In conclusion, a conservative treatment is feasible in metatarsal bones with skin coverage by flap if necessary. In STS adequate margins are difficult to achieve with a high rate of local recurrence. In calcaneus and talus, a conservative treatment is possible in tumours limited to bone after good response to chemotherapy. In other cases conservative treatment is debatable because amputation gives excellent functional results.

Objective: To determine patient characteristics and outcomes for extremity rhabdomyosarcoma (RMS) utilizing an international cohort of prospectively treated patients.

Methods: Data were collected from 566 patients (1984 through 2003) treated on cooperative protocols : US IRS III, IV Pilot, IV studies – SIOP 84, 89, 95 studies – Italian ICG 79, 88, 96 studies – German CWS81, 86, 91, 96 studies.

Results: 29 % of the patients were < 3 year old, 36 % were 3 to 10 year old and 35 % > 10 year old. 350 (63%) patients had alveolar RMS and 116 (22%) had regional nodes.

The overall survival and EFS were 65% and 51% at 5 years respectively and 59% and 48% at 10 years respectively.

By univariate analysis, EFS was influenced by age below 3 years but not by age over 10 years (EFS were 61%, 49% and 46% for patients below 3 years, from 3 to 10 and 10 years or more respectively). It was also influenced by tumor invasiveness, tumor size, lymph node involvement, histology, completeness of surgery at diagnosis and cooperative groups. In multivariate analysis of EFS, size, lymph nodes, quality of surgery, cooperative groups had independent impact. Age and histology had no more impact.

OS (univariate analysis) was influenced by age below 3 years but not by age over 10 years (OS were 77%, 61% and 58% for patients below 3 years, from 3 to 10 and 10 years or more respectively). In multivariate analysis, age, lymph nodes, tumour invasiveness, quality of surgery at diagnosis had independent impact. Histology, tumour size and cooperative groups had no more impact.

Conclusion: This analysis shows that significant cut-point for age is 3 years, that histology per se has no impact on OS and EFS. It also underscores the impact of initial surgery on outcome.

The Euro-E.W.I.N.G. 99 trial aimed to improve the dismal prognosis of patients with primary disseminated multifocal Ewing tumors (PDM-ET) with a dose-intense treatment concept.

From 1999 to 2005, 281 patients with PDM-ET were enrolled onto the EURO-E.W.I.N.G. 99 trial. Median age was 16.2 years (0.4–49). Recommended treatment consisted of 6 VIDE, one VAI cycle, local treatment (surgery and/or radiotherapy), and high-dose busulfan-melphalan followed by autologuous stem cell transplantation (HDT/SCT).

After a median follow up of 3.8 years, event-free survival (EFS) and overall survival (OS) at 3 years for all 281 patients were 27%±3% and 34%±4%. Six VIDE cycles were completed by 250 patients (89%); 169 (60%) received HDT/SCT. Forty-six children less than 14 years and HDT/SCT achieved a 3-year EFS of 45%. Cox regression analyses demonstrated increased risk at diagnosis for patients over 14 years (HR 1.6), a primary tumor volume > 200ml (HR 1.8), more than one bone metastatic site (hazard ratio: HR 2.0, bone marrow metastases (HR 1.6) and additional lung metastases (HR 1.5). An “up front” risk score based on these HR factors identified three groups with EFS rates of 50% for score ≤3 (82 patients), 25% for score > 3 to < 5 (102 patients), and 10% for score ≥5 (70 patients), p< 0.0001.

PDM-ET patients may survive with intensive multimodal therapy. Age, tumor volume, and extent of meta-static spread are relevant risk factors. A score based on these factors identifies PDM-ET patients may facilitate risk adapted treatment approaches.

Purpose of the study: We wanted to assess long-term outcome after treatment for Ewing tumor of the pelvis.

Material and method: We reviewed 62 patients aged 5 to 28 years treated from 1983 through 1993. There were 35 males and 27 males. Sixteen patients had pulmonary metastases at diagnosis. Patients were given chemotherapy using three protocols (Ew 84, Ew 88, Ew 93) proposed by the French Society of Pediatric Oncology. Fourteen patients were give high-dose chemotherapy with a bone marrow graft. The local treatment was not randomized. Radiotherapy was used alone in 25 patients and 15 underwent surgery and radiotherapy. Eighteen underwent surgery without complementary radiotherapy. For patients were not given local treatment. Outcome at last follow-up was assessed retrospectively.

Results: Mean follow-up was 6.6 years (3 months to 18 years); 29 patients were in remission, 6 had progressive disease, and 27 had died. Two patients who had bone marrow grafts developed a second tumor in the radiated territory. The overall chances of survival were 55±6% at five years and 53±7% at ten years. There was no significant difference by type of chemotherapy. In the group of operated patients, the five year survival was 68% versus 43% in the group of non-operated patients (p=0.007). In patients with initial metastases, chances of survival at ten years were 19.7±10% versus 65.9±7% in patients without metastasis. Only two patients who presented metastases initially were in remission at last follow-up. Five patients developed local recurrence after surgery and none had been radiated despite incomplete response to chemotherapy or presence of contaminated resections.

Discussion: Rigorous comparison between operated and non-operated patients is hindered due to the different indications. Results of treatment of Ewing tumors of the pelvis without metastasis are comparable to those obtained for tumors in other localizations. The fact that a second tumor can develop in the radiated territory is a particularly important factor in patients given high-dose chemotherapy with a bone marrow graft.

Conclusion: Surgical treatment appears to improve local control of Ewing tumors of the pelvis. If initial metastasis is not present, the prognosis appears to be similar to other localizations. Radiotherapy remains and indispensable adjuvant in the event of surgical resection or incomplete response to chemotherapy.