Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: recent experience at the istituto rizzoli.

Abstract

PURPOSE:

The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported.

PATIENTS AND METHODS:

A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfamide, and etoposide) was administered to all patients. Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11.

RESULTS:

At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity. The 5-year event-free survival and overall survival were 54.2% and 62.7%, respectively. To assess the prognostic significance of neural differentiation in the family of Ewing's sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing's sarcoma (TES) who were treated according to the same protocol. Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemotherapy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P <.012; 5-year overall survival, 62.7% v 78.3%, P <.002).

CONCLUSION:

The authors conclude that studies into new adjuvant therapy for Ewing's sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor.

• Authors: G Bacci, S Ferrari, F Bertoni, D Donati, P Bacchini, A Longhi, A Brach Del Prever, C Forni, S Rimondini
• Journal: Journal of clinical oncology : official journal of the American Society of Clinical Oncology (J Clin Oncol) Vol. 18 Issue 4 Pg. 885-92 (Feb 2000) ISSN: 0732-183X [Print] United States
• PMID: 10673532 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antibiotics, Antineoplastic
• Antineoplastic Agents, Alkylating
• Antineoplastic Agents, Phytogenic
• Dactinomycin
• Vincristine
• Doxorubicin
• Cyclophosphamide

Topics

• Adolescent
• Adult
• Antibiotics, Antineoplastic (administration & dosage, adverse effects)
• Antineoplastic Agents, Alkylating (administration & dosage, adverse effects)
• Antineoplastic Agents, Phytogenic (administration & dosage, adverse effects)
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Bone Neoplasms (drug therapy, radiotherapy, surgery)
• Chi-Square Distribution
• Cyclophosphamide (administration & dosage, adverse effects)
• Dactinomycin (administration & dosage, adverse effects)
• Disease-Free Survival
• Doxorubicin (administration & dosage, adverse effects)
• Female
• Follow-Up Studies
• Humans
• Male
• Neoadjuvant Therapy
• Neoplasm Recurrence, Local (pathology)
• Neuroectodermal Tumors, Primitive, Peripheral (drug therapy, radiotherapy, surgery)
• Prognosis
• Radiotherapy, Adjuvant
• Sarcoma, Ewing (drug therapy, radiotherapy, surgery)
• Survival Rate
• Treatment Outcome
• Vincristine (administration & dosage, adverse effects)