Efficacy of Proton Beam Therapy in the Treatment of Ewing's Sarcoma of the Paranasal Sinuses and Anterior Skull Base

ABSTRACT

Ewing's sarcoma involving the sinonasal cavity and anterior skull base is very rare. The purpose of this article is to present our experience with two such cases, which were both treated with combined chemotherapy and proton beam radiation therapy. The patients were selected from a retrospective medical record analysis that was conducted of all patients who were diagnosed with Ewing's sarcoma of the sinonasal cavity at the Massachusetts Eye & Ear Infirmary/Massachusetts General Hospital Cranial Base Center from 2004 to 2008. One of the patients underwent pretreatment endoscopic subtotal resection to facilitate proton beam radiation therapy. Response to treatment was assessed, post-treatment endoscopic biopsies were performed to assure eradication of disease, and treatment complications were recorded. Both patients completed chemotherapy and proton beam radiation therapy without complication. One patient completed treatment in December 2006 and remains disease free. The second patient completed treatment in March 2007 and remains disease free. Both patients developed headaches accompanied by frontal sinus opacification after treatment that required endoscopic drainage. After endoscopic drainage, the opacification and symptoms resolved.

KEYWORDS

Ewing's sarcoma - anterior skull base - proton beam - endoscopic surgery

REFERENCES

• 1 Dahlin D C, Coventry M B, Scanlon P W. Ewing's sarcoma: a critical analysis of 165 cases.  J Bone Joint Surg Am. 1961;  43-A 185-192
• 2 Spjut H J, Dorfman H D, Fechner R E, Ackerman L V. Tumors of bone and cartilage. In: Atlas of Tumor Pathology. 2nd series, fasc 5. Washington, DC; Armed Forces Institute of Pathology 1971: 317
• 3 Siegal G P, Olivier W R, Reinus W R. Primary Ewing's sarcoma involving the bones of the head and neck.  Cancer. 1987;  60 2829-2840
• 4 Fernandez C H, Lindberg R D, Sutox W W, Samuels M L. Localized Ewing's Sarcoma – treatment and results.  Cancer. 1974;  34(1) 143-148
• 5 Strong L C, Herson J, Osborne B M, Sutow W W. Risk of radiation-related subsequent malignant tumors in survivors of Ewing's sarcoma.  J Natl Cancer Inst. 1979;  62(6) 1401-1406
• 6 Hossfeld D K, Seeber S, Siemers E, Schmidt C G, Scherer E. Early results of combined modality therapy of patients with Ewing's sarcoma.  Recent Results Cancer Res. 1982;  80 124-127
• 7 Pontius K I, Sebek B A. Extraskeletal Ewing's sarcoma arising in the nasal fossa. Light- and electron-microscopic observations.  Am J Clin Pathol. 1981;  75(3) 410-415
• 8 Woodruff G, Thorner P, Skarf B. Primary Ewing's sarcoma of the orbit presenting with visual loss.  Br J Ophthalmol. 1988;  72(10) 786-792
• 9 Lane S, Ironside J W. Extra-skeletal Ewing's sarcoma of the nasal fossa.  J Laryngol Otol. 1990;  104(7) 570-573
• 10 Csokonai L V, Liktor B, Arato G, Helffrich F. Ewing's sarcoma in the nasal cavity.  Otolaryngol Head Neck Surg. 2001;  125(6) 665-667
• 11 Aferzon M, Wood W E, Powell J R. Ewing's sarcoma of the ethmoid sinus.  Otolaryngol Head Neck Surg. 2003;  128(6) 897-901
• 12 Windfuhr J P. Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management.  Ann Otol Rhinol Laryngol. 2004;  113(7) 533-543
• 13 Howarth K L, Khodaei I, Karkanevatos A, Clarke R W. A sinonasal primary Ewing's sarcoma.  Int J Pediatr Otorhinolaryngol. 2004;  68(2) 221-224
• 14 Coskun B U, Cinar U, Savk H, Basak T, Dadas B. Isolated maxillary sinus Ewing's sarcoma.  Rhinology. 2005;  43(3) 225-228
• 15 Kawabata M, Yoshifuku K, Sagara Y, Kurono Y. Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus.  Rhinology. 2008;  46(1) 75-78
• 16 Jaffe N, Traggis D, Salian S, Cassady J R. Improved outlook for Ewing's sarcoma with combination chemotherapy (vincristine, actinomycin D and cyclophosphamide) and radiation therapy.  Cancer. 1976;  38 1925-1930
• 17 Rosen G, Caparros B, Nirenberg A et al.. Ewing's sarcoma: ten-year experience with adjuvant chemotherapy.  Cancer. 1981;  47 2204-2213
• 18 Jurgens H, Exner U, Gadner H et al.. Multidisciplinary treatment of primary Ewing's sarcoma of bone. A 6-year experience of a European Cooperative Trial.  Cancer. 1988;  61 23-32
• 19 Bacci G, Toni A, Avella M et al.. Long-term results in 144 localized Ewing's sarcoma patients treated with combined therapy.  Cancer. 1989;  63 1477-1486
• 20 Burgert E O, Nesbit M E, Garnsey L A et al.. Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: Intergroup Study IESS-II.  J Clin Oncol. 1990;  8 1514-1524
• 21 Nesbit M E, Gehan E A, Burgert E O et al.. Multimodal therapy for the management of primary nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the first Intergroup Study.  J Clin Oncol. 1990;  8 1664-1674
• 22 Kinsella T J, Miser J S, Waller B et al.. Long-term follow-up of Ewing's sarcoma of bone treated with combined modality therapy.  Int J Radiat Oncol Biol Phys. 1991;  20 389-395
• 23 Dunst J, Sauer R, Burgers M V et al.. Radiation therapy as local treatment in Ewing's sarcoma. Results of the cooperative Ewing's sarcoma studies CESS 81 and CESS 86.  Cancer. 1991;  67 2818-2825
• 24 Hayes F A, Thompson E J, Meyer W H et al.. Therapy for localized Ewing's sarcoma of bone.  J Clin Oncol. 1989;  7 208-213
• 25 Bacci G, Campanacci M, Pagani P A. Adjuvant chemotherapy in the treatment of clinically localised Ewing's sarcoma.  J Bone Joint Surg Br. 1978;  60-B 567-574
• 26 Oberlin O, Habrand J L, Zucker J M et al.. No benefit of Ifosfamide in Ewing's sarcoma: a nonrandomized study of the French Society of Pediatric Oncology.  J Clin Oncol. 1992;  10 1407-1412
• 27 Patel S, DeLaney T F. Advanced technology radiation therapy for bone sarcomas.  Cancer Control. 2008;  15(1) 21-37
• 28 Ewing J. Diffuse endothelioma of bone.  Proc N Y Pathol Soc. 1921;  21 17-24
• 29 Batsakis J G, Mackay B, El-Naggar A K. Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report.  Ann Otol Rhinol Laryngol. 1996;  105 838-843
• 30 Wood R E, Nortje C J, Hesseling P et al.. Ewing's tumor of the jaw.  Oral Surg Oral Med Oral Pathol. 1990;  69 120-127
• 31 Yalcin S, Turoglu H T, Ozdamar S, Sadikoglu Y, Gurbuzer B, Yenici O. Ewing's tumor of the mandible.  Oral Surg Oral Med Oral Pathol. 1993;  76(3) 362-367
• 32 Harman M, Kiroglu F, Kosem M et al.. Primary Ewing's sarcoma of the paranasal sinus with intracranial extension: Imaging features.  Dentomaxillofacial Radiol. 2003;  32(5) 343-346
• 33 Bridge R S, Rajaram V, Dehner L P, Pfeifer J D, Perry A. Molecular diagnosis of Ewing sarcoma/primitive neuroectodermal tumor in routinely processed tissue: a comparison of two FISH strategies and RT-PCR in malignant round cell tumors.  Mod Pathol. 2006;  19 1-8
• 34 Fiorillo A, Tranfa F, Canale G et al.. Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy.  Cancer Lett. 1996;  103(2) 177-182
• 35 Burgert Jr E O, Nesbit Jr M E, Gehan E A et al.. Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: Intergroup Study IESS-II.  J Clin Oncol. 1990;  8 1514-1524
• 36 Bacci G, Picci P, Ferrari S et al.. Neoadjuvant chemotherapy for Ewing's sarcoma of bone: No benefit observed after adding ifosfamide and etoposide to vincristine, actinomycin, cyclophosphamide, and doxorubicin in the maintenance phase—results of two sequential studies.  Cancer. 1998;  82(6) 1174-1183