Pediatr Blood Cancer 2011;56:477–481 BRIEF REPORT Therapy and Outcome of Primitive Neuroectodermal Tumor of the Jaw Sameer Bakhshi, MD, 1, * Subha Pathania, MD, 1 B.K. Mohanti, MD, 2 Sanjay Thulkar, MD, 3 and Alok Thakar, MD 4 Data pertaining to outcomes in jaw primitive neuroectodermal tumor (PNET) are lacking. Eleven cases of jaw PNET (five maxil- lary and six mandibular) were treated at our cancer center with the same chemotherapeutic agents from June 2003 to January 2009. Four patients underwent surgery after neoadjuvant chemotherapy and all received local radiotherapy. At median follow-up of 56 months (range: 19–77 months), 7/11 patients are in sustained remission. There was no difference in outcome with respect to site of tumor, and whether surgery was performed or not. These results support the use of chemoradiation as initial modality of treatment rather than going for extensive and mutilating surgery. Pediatr Blood Cancer 2011;56:477–481. © 2010 Wiley-Liss, Inc. Key words: Ewing sarcoma; mandible; maxilla; primitive neuroectodermal tumor; therapy INTRODUCTION Primitive neuroectodermal tumor (PNET) or Ewing sarcoma is the second most frequent primary malignant bone cancer. The most frequently affected sites are the long bones of the lower extremities and pelvis. Head and neck region accounts for 1–4% of all cases of PNET [1]. The most commonly affected bones in head and neck region are jaw and skull bones [2]. However, data pertaining to these sites with regard to outcomes using a uniform treatment modality are lacking. METHODS This is a retrospective study of primary jaw PNET treated at our center from June 2003 to January 2009. The patients’ demographic profile, clinical data, metastatic workup, treatment, and survival were studied. Complete remission (CR), partial response (PR), and stable disease (SD) were defined as per RECIST criteria [3]. CLINICAL PROFILE During the study period, there were 11 cases of primary jaw PNET (six maxillary and five mandibular) with a median age of 16 years (range 4–19 years) and male/female ratio of 9:2 (Table I). All patients presented with jaw swelling; one patient also had fever as a presenting manifestation (case #10). Diagnostic work-up including bone marrow biopsy, bone scan, and computed tomography (CT) scan of chest did not reveal metastatic disease in any patient. CT scan of face showed variably enhancing mass lesion with hetero- geneous attenuation in all cases. There was bone destruction in all cases with tumor extension beyond the jaw in 8/11 cases. Periosteal calcification was noticed in one case only. All patients underwent biopsy (two trucut and nine incisional) and histopathology revealed a small round malignant tumor which were positive for MIC-2 anti- gen. However, we did not have the means to study the reciprocal chromosomal translocation for this disease. THERAPY AND OUTCOME Chemotherapy was given using vincristine, doxorubicin, acti- nomycin D, cyclophosphamide, ifosfamide, and etoposide; three patients (cases 1, 5, and 9) received these drugs as per the institu- tional protocol of St. Jude Children’s Research Hospital [4] while the other patients received alternating cycles of the above drugs [5]. Repeat evaluation after 5–6 cycles of neoadjuvant chemother- apy revealed partial remission in all cases. All patients except case 11 had residual soft tissue component on imaging after neoadju- vant chemotherapy. In only one of the patients (case 9), a biopsy of the soft tissue component was performed which showed fibro- collagenous tissue and so no surgery was performed on him. In 5/9 remaining cases, the soft tissue component extended into sur- rounding structures such as inferior orbital wall and infratemporal fossa which would have resulted in mutilating surgery if a complete resection was attempted. Thus, a definitive surgery was performed in only four patients. One patient underwent partial maxillectomy; one total maxillectomy; one marginal mandiblectomy; and one total mandiblectomy. Out of these four patients, histopathological exam- ination showed evidence of active tumor in all and in one of these patients (case 6), the tumor was involving the posterior margin as well. Radiotherapy was given in all patients irrespective of surgery at a dose of 45–55 Gy (median 50 Gy) in 20–30 fractions (median 25 fractions) 5 days a week, over 5–6 weeks. Following radiother- apy and/or surgery, further chemotherapy was given in all cases as per protocol for 48 weeks. One of the patients (case 8) devel- oped a chronic discharging sinus at the surgical site due to radiation necrosis of the mandible and required excision of the affected bone fragment. Seven of 11 cases are in CR at a median follow-up of 56 months (range: 19–77) since diagnosis. The 3-year event- free survival (EFS) is 59 ± 15% at a median follow-up of 24.6 months (Fig. 1). The 3-year EFS of patients with mandibular PNET 1 Department of Medical Oncology, All India Institute of Medical Sci- ences, New Delhi, India; 2 Department of Radiotherapy, Dr B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sci- ences, New Delhi, India; 3 Department of Radiology, Dr B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India; 4 Department of Otorhinolaryngiology, Dr B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India Conflict of interest: Nothing to declare. *Correspondence to: Sameer Bakhshi, Associate Professor of Pediatric Oncology, Department of Medical Oncology, Dr B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India. E-mail: sambakh@hotmail.com Received 15 February 2010; Accepted 5 April 2010 © 2010 Wiley-Liss, Inc. DOI 10.1002/pbc.22615 Published online 11 November 2010 in Wiley Online Library (wileyonlinelibrary.com).