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  • br Authors disclosure Mark Clemons received honoraria

    2019-06-11


    Authors\' disclosure Mark Clemons received honoraria for talks from Novartis and Amgen, research funding from Novartis.
    Case In September 2007 a 9½-year-old boy was diagnosed with a Ewing sarcoma (EWS-FL1 translocation positive) of the right scapula. MRI showed a tumor of 8.0×2.9 ×6.2cm located in the right scapula with dorsal displacement of the infraspinatus and teres minor muscles (Fig. 1). The tumor extended in to the glenoid, but the shoulder joint was not contaminated. Staging, including bone scan (Fig. 2) and CT-imaging, did not show metastatic lesions. The child was treated with chemotherapy according to the EURO-EWING 99 protocol [1]. After 6 VIDE courses soft tissue involvement was absent on MRI. Intraoperative extra-corporal irradiation (IEI) was chosen since functional results with scapula prostheses are poor [2]. After the resection of the scapula pathologic samples were taken from the specimen: vital tumor amc 7 were seen on the surface of the dorsal part of the periost. The resected scapula was irradiated with a tumoricidal dose of 120Gy by means of 6MV photons (Fig. 3), creating dead autologue bone graft of the correct dimensions for re-implantation and reconstruction [3]. Postoperative examination of shoulder function gives anteflexion of over 160°, abduction of over 160° (Fig. 4), exorotation to 60°, endorotation to the left scapula as shown in Fig. 4. Sensibility and strength are intact. At five year follow-up there has been subtotal resorption of the scapula (Fig. 5), leaving the glenohumeral joint intact. Remaining shoulder function gives anteflexion of over 120°, abduction of 100°, exorotation to 60°, endorotation to the left scapula as shown in Fig. 6. Sensibility and strength are intact.
    Discussion Malignant tumor of the scapula is a rare condition. Several surgical treatment types are available, such as total scapulectomy, constrained prosthesis and scapular allograft. All of these surgical treatment options result in very little or no shoulder function [2,4–7]. Resection, extracorporeal irradiation and re-implantation (IEI) is described in literature as a treatment option for bony malignancy [3,8–15]. It has been used in incidental cases, both in Ewing Sarcoma and other bone tumors [3,9,11–15]. As far as we know IEI had never been described in a child with a scapular Ewing sarcoma. Types of cancer affecting the scapula described in literature are chondro sarcoma, synovial sarcoma, Ewing sarcoma (ES) and metastasis [4–7,16,17]. Modern treatment of ES of bone in children and adults consists of chemotherapy and local treatment by surgery or radiotherapy or a combination of both. If possible, surgery with appropriate margins alone is the preferred local treatment.
    Contributions of authors
    Re-irradiation of symptomatic recurrent painful bone metastases Radiotherapy to painful bony metastases is undoubtedly useful in pain relief. It was demonstrated on a recent updated systemic review [1] that the effectiveness in pain relief is comparable for single-fraction (SF) and multi-fraction (MF) radiotherapy. It was found that 11–42% and 0–24% of patients given SF and MF treatment respectively would eventually require retreatment [2]. The re-irradiation rate is significantly higher in patients with single fraction radiotherapy (2.6 fold, 20% versus 9% in the SF arm versus the MF arm respectively) [1]. On the other hand, with increased effectiveness of systemic cancer treatment, patients nowadays have longer life expectancy. Thus, bone re-irradiation is increasingly considered. A recent systematic review and meta-analysis by Huisman et al. [3] included 2694 patients treated with re-irradiation from 4Gy up to 26Gy, 2–8Gy per fraction daily. It showed an overall pain response of 58%. Toxicities are the major concern for re-irradiation. Huisman et al. [3] reviewed the toxicities from various studies, and reported that toxicity data was only available in three studies from Jeremic et al. in 1999 [4]and 2002 [5], and van der Linden et al. in 2004 [6] respectively. Jeremic et al. 1999 [4] reported that there was no serious acute toxicity (≥RTOG grade 3). Pathological fractures were reported in 3/135 (2%) patients and spinal cord compression in 3/135 (2%) patients.. The main side effect was mainly gastrointestinal (grade 1 or 2 nausea and vomiting) in 18% (25/135). The same group in 2002 [5] reported there was no acute or late high-grade toxicity (>3), no pathological fractures or spinal cord compression. Van den Linden et al. [6] reported retreatment toxicities in both single-fraction (SF) versus multi-fraction (MF) treatment groups with Rotterdam Symptom Checklist. Most SF and MF patients reported no or only mild nausea and vomiting. Nausea score 4 (very bad) was reported in 12% of MF patients vs. 6% of SF patients (p=0.39). Vomiting score 4 (very bad) was reported in 1MF patient and 2 SF patients (p=0.49). Severe tiredness was reported in 18% of SF patients and 27% of MF patients (p=0.41). Overall, it seems that re-irradiation is a tolerable treatment.