Japanese Journal of Clinical Oncology 32:430-434 (2002)
© 2002 Foundation for Promotion of Cancer Research
Hemiarthroplasty of the Elbow with a Vascularized Fibular Graft after Excision of Ewing’s Sarcoma of the Proximal Ulna: a Case Report
Divisions of 1 Orthopedic Surgery, 2 Head and Neck Surgery and 3 Surgical Pathology, Chiba Cancer Center Hospital, Chiba, Japan
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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We report an 8-year-old girl with a Ewing’s sarcoma in the right proximal ulna. The patient presented with pain in her right elbow, and plain radiographs showed destructive changes with a periosteal reaction in the proximal third of the ulna. A biopsy confirmed the diagnosis of Ewing’s sarcoma. For preoperative chemotherapy, the patient received two courses of vincristine, doxorubicin and cyclophosphamide alternating with high-dose ifosfamide. MRI and angiography demonstrated that the chemotherapy was effective and therefore the patient underwent wide local excision of the tumor and reconstruction of the elbow joint using a vascularized fibular graft. Pathological examination of the resected specimen showed no evidence of viable tumor cells. After surgery, the patient received three courses of chemotherapy consisting of the same agents as those for the preoperative chemotherapy. Four years after surgery, there is no evidence of local recurrence or distant metastasis. Furthermore, the grafted bone is extending favorably and the patient has excellent function with active movement of the elbow.
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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Ewing’s sarcoma of the bone is the second most common malignant primary bone tumor occurring in children (1,2). More than half of all Ewing’s sarcomas arise in the lower extremities and pelvic girdle. Statistically, it rarely arises in the ulna (3,4). Until the 1970s, radiotherapy was the usual local treatment for Ewing’s sarcoma (5,6). However, with recent advances in chemotherapy, the survival of patients with Ewing’s sarcoma has markedly improved and the standard treatment has changed to chemotherapy and surgery. In addition, because of advances in diagnostic imaging and surgical techniques, limb-sparing surgery is now acceptable (7).
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CASE REPORT |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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An 8-year-old girl presented with a 1-week history of pain in the right elbow. Plain radiographs showed destructive changes with a periosteal reaction in the proximal third of the ulna (Fig. 1). MRI showed a tumor filling the marrow cavity and a large soft-tissue mass surrounding the involved bone. The patient was admitted on the same day and underwent a needle biopsy. Frozen section examination at the time of biopsy showed findings suggestive of Ewing’s sarcoma. On the following day, induction chemotherapy was started. Later, hematoxylin and eosin-stained slides of the paraffin-embedded biopsy specimen showed proliferation of small round cells with round-to-oval nuclei (Fig. 2). A periodic acid–Schiff (PAS) preparation revealed intracellular glycogen and immunostaining of the cells showed membranous immunoreactivity only for the MIC-2 gene product. These findings confirmed the diagnosis of Ewing’s sarcoma. Chest CT showed no evidence of metastatic disease and bone scintigraphy showed uptake only at the right ulna. According to the Enneking surgical staging system (8), the case was classified as stage IIB.
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Induction chemotherapy consisted of two courses: a course of 2 mg/m2 vincristine, 60 mg/m2 doxorubicin and 1800 mg/m2 cyclophosphamide alternating with 16 g/m2 ifosfamide (Fig. 3). After two courses of induction chemotherapy, MRI showed a significant reduction of the large soft-tissue mass surrounding the ulna (Fig. 4), thus confirming the efficiency of the chemotherapy. Therefore, the patient was scheduled for wide excision of the tumor and limb-sparing surgery using a vascularized fibular graft.
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The skin incision was drawn along the shaft of the ulna including the site of biopsy. Muscle groups were conserved except for the anconeus, supinator and flexor digitorum profundus, which were attached to the tumoral process. The median, radial and ulnar nerves were conserved. After excision of 13 cm of the proximal ulna including the ulnar collateral ligament, the vascularized osteocutaneous fibular graft including the fibular head was harvested from the patient’s right leg and fixed into the recipient site with a plate and screws. To stabilize the fibular head, the tendon of the biceps femoris muscle of the fibular graft was sutured to the annular ligament of the radius. Also, the fibular collateral ligament of the fibular graft was sutured to the tendon of the triceps muscle. For vascularization, the donor anterior tibial artery was anastomosed to the ulnar artery and the donor anterior tibial vein was anastomosed to the basilic vein. Fixation with a gypsum cast was applied for 6 weeks.
Histological examination of the surgical specimen revealed no residual viable tumor cells and the pathological findings confirmed wide, clean excisional margins. After surgery, the patient received three courses of chemotherapy consisting of the same agents as those used for the preoperative protocol (Fig. 3). The dose of each agent was reduced owing to delayed recovery of the neutrophil count. Three months after surgery, complete bony union was achieved and at 4 years there was no evidence of local recurrence or distant metastasis. X-ray examination has shown no collapse of the fibular head (Fig. 5). Moreover, because the grafted bone has extended (2.4 cm), the upper extremity is growing without malalignment or deformity. At present, the patient has excellent function with active movement of the elbow from 5 to 150°, unrestricted pronation and supination and full hand function (Fig. 6). According to the modified system of Enneking et al. (9) for functional evaluation of extremity reconstruction after tumor excision, the rating is 100% (30/30). Regarding the condition of the knee joint of the donor side, there is no varus instability and she can walk without any problems. Furthermore, the patient is able to enjoy sports (basketball and swimming).
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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To improve the long-term survival of patients with Ewing’s sarcoma, many investigators have tried new treatment strategies (3,4,10–14). With advances in chemotherapy over the past 10 years, disease-free survival rates have improved considerably; in several institutional studies, 5-year survival rates of over 60% have been reported (3,10,11). In general, current standard chemotherapy includes vincristine, doxorubicin and cyclophosphamide (VDoxC) alternating with ifosfamide (IFO) and etoposide (1,14). Some investigators recommend intensive chemotherapy that incorporates high-dose IFO with Mesna for Ewing’s sarcoma (3,12). We have also reported the effectiveness of intensive chemotherapy incorporating high-dose IFO (12–18 g/m2) for Ewing’s sarcoma (15). With our present regimen, patients with Ewing’s sarcoma receive alternating cycles of 2 mg/m2 vincristine, 60 mg/m2 doxorubicin and 1800 mg/m2 cyclophosphamide (VDoxC) and 16 g/m2 IFO (Fig. 3). For cases without metastasis at diagnosis, the 3-year disease-free rate has reached 66%. In the present case, preoperative chemotherapy reduced the incidence of disseminated lesions and there was no evidence of viable tumor cells in the surgical specimen.
Until the 1970s, radiotherapy was the usual local treatment for Ewing’s sarcoma (5,6). Even now, radiotherapy is performed for local control in cases where wide excision is difficult. On the other hand, a retrospective study has noted that patients who receive 6000 cGy or more have a 20% incidence of second malignancy (1,16). Because of this risk of second malignancy and skin disturbance, radiotherapy is not now commonly performed. Recently, better local control has been achieved by intensive chemotherapy. Therefore, the use of limb-sparing surgery after wide excision of the tumor has increased over the past 10 years (7).
Regarding surgical options for limb-sparing, Windhager et al. recommend resection–replantation, in which the tumor-bearing area is resected as a ‘cylindrical segment’ and the distal arm is replanted with shortening (17). While wide excisional margin was achieved in the method, the cosmetic deformity seems to be severe. Sperling et al. reported 13 patients who underwent total elbow arthroplasty after excision of tumors at the distal humerus (18). On the other hand, there has been no report of total elbow arthroplasty after excision of tumors at the proximal ulna. The use of vascularized fibular grafts has been described for the treatment of bone loss after excision of tumors (19,20). However, to our knowledge, there is only one report of hemiarthroplasty of the elbow joint using a vascularized fibular graft after excision of a bone tumor. Gianoutsos et al. reported vascularized fibular grafting for reconstruction after excision of adamantinoma of the proximal ulna (21). Therefore, this is the second report about hemiarthroplasty of the elbow joint using a vascularized fibular graft after the excision of a malignant tumor of the proximal ulna.
After excision of a bone tumor in the diaphyseal area, vascularized fibular grafting has commonly been performed for reconstruction (19). In the present case, since the tumor occurred in the metaphyseal area, the proximal ulna had to be excised along with the surface of the elbow joint. Therefore, a vascularized fibular graft including the fibular head was used for reconstruction. Usui et al. reported that the use of a free vascularized fibular graft including the head carried a risk of fibular head collapse and considered that the anterior tibial artery was preferable to the peroneal artery for supplying the fibular head (22). Therefore, we selected the anterior tibial artery for vascularization in this case and, up to the time of writing, no fibular head collapse has occurred (Fig. 5). Moreover, because the grafted bone is still extending, the upper extremity is growing without malalignment or deformity, thus confirming that the epiphyseal and metaphyseal blood supply to the grafted fibular head is preserved. In addition, preservation of the brachio-radial joint has provided excellent elbow function.
In summary, since intensive chemotherapy reduced the disseminated lesion in this case of Ewing’s sarcoma of the proximal ulna, we were able to perform limb-sparing surgery after wide excision. Our medium-term results suggest that vascularized fibular grafting including the fibular head is useful for reconstruction after excision of a malignant tumor in the proximal ulna.
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FOOTNOTES |
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+ For reprints and all correspondence: Takeshi Ishii, Division of Orthopedic Surgery, Chiba Cancer Center Hospital, 666–2 Nitona-cho, Chuo-ku, Chiba, 260-8717, Japan
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REFERENCES |
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1 Supplementary information at http://www.cancer.gov/cancer_information/cancer_type/ewings_family_of_tumors/.
2 Rosen G, Caparros B, Nirenberg A, Marcove RC, Huvos AG, Kosloff C, et al. Ewing’s sarcoma: ten-year experience with adjuvant chemotherapy. Cancer 1981;47:2204–13.[Web of Science][Medline]
3 Paulussen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, et al. Localized Ewing tumor of bone: final results of the cooperative Ewing’s Sarcoma Study CESS 86. J Clin Oncol 2001;19:1818–29.
4 Wunder JS, Paulian G, Huvos AG, Heller G, Meyers PA, Healey JH. The histological response to chemotherapy as a predictor of the oncological outcome of operative treatment of Ewing sarcoma. J Bone Joint Surg Am 1998;80:1020–33.
5 Razek A, Perez CA, Tefft M, Nesbit M, Vietti T, Burgert EO Jr, et al. Intergroup Ewing’s Sarcoma Study: local control related to radiation dose, volume and site of primary lesion in Ewing’s sarcoma. Cancer 1980;46:516–21.[Web of Science][Medline]
6 Lewis RJ, Marcove RC, Rosen G. Ewing’s sarcoma – functional effects of radiation therapy. J Bone Joint Surg Am 1977;59:325–31.
7 Sluga M, Windhager R, Lang S, Heinzl H, Krepler P, Mittermayer F, et al. The role of surgery and resection margins in the treatment of Ewing’s sarcoma. Clin Orthop 2001;392:394–9.
8 Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop 1986;204:9–24.
9 Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard D. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop 1993;286:241–6.
10 Cotterill SJ, Ahrens S, Paulussen M, Jurgens HF, Voute PA, Gadner H, et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol 2000;18:3108–14.
11 Craft A, Cotterill S, Malcolm A, Spooner D, Grimer R, Souhami R, et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: The Second United Kingdom Children's Cancer Study Group and the Medical Research Council Ewing’s Tumor Study. J Clin Oncol 1998;16:3628–33.[Abstract]
12 Rosito P, Mancini AF, Rondelli R, Abate ME, Pession A, Bedei L, et al. Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone: a preliminary report of 6 years of experience. Cancer 1999;86:421–8.[Web of Science][Medline]
13 Sluga M, Windhager R, Lang S, Heinzl H, Krepler P, Mittermayer F, et al. A long-term review of the treatment of patients with Ewing’s sarcoma in one institution. Eur J Surg Oncol 2001;27:569–73.[Web of Science][Medline]
14 Ferrari S, Mercuri M, Rosito P, Mancini A, Barbieri E, Longhi A, et al. Ifosfamide and actinomycin-D, added in the induction phase to vincristine, cyclophosphamide and doxorubicin, improve histologic response and prognosis in patients with non metastatic Ewing’s sarcoma of the extremity. J Chemother 1998;10:484–91.[Web of Science][Medline]
15 Yokoyama R, Ishii T, Araki N, Yoshikawa H, Takayama J, Uchida A. Current status of treatment for Ewing’s sarcoma/primitive neuroectodarmal tumor in Japan. Syouni-gan 2000;37:497–501 (in Japanese).
16 Kuttesch JF Jr, Wexler LH, Marcus RB, Fairclough D, Weaver-McClure L, White M, et al. Second malignancies after Ewing’s sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol 1996;14:2818–25.
17 Windhager R, Millesi H, Kotz R. Resection–replantation for primary malignant tumours of the arm. An alternative to fore-quarter amputation. J Bone Joint Surg Br 1995;77:176–84.
18 Sperling JW, Pritchard DJ, Morrey BF. Total elbow arthroplasty after resection of tumors at the elbow. Clin Orthop 1999;367:256–61.
19 Minami A, Kutsumi K, Takeda N, Kaneda K. Vascularized fibular graft for bone reconstruction of the extremities after tumor resection in limb-saving procedures. Microsurgery 1995;16:56–64.[Web of Science][Medline]
20 Amr SM, El-Mofty AO, Amin SN, Morsy AM, El-Malt OM, Abdel-Aal HA. Reconstruction after resection of tumors around the knee: role of the free vascularized fibular graft. Microsurgery 2000;20:233–51.[Web of Science][Medline]
21 Gianoutsos MP, Marsden FW, McCarthy SW, Lee KK. Ulnar adamantinoma: en bloc excision and fibular osteoseptocutaneous free flap reconstruction. J Hand Surg [Am] 1994;19:495–9.[Medline]
22 Usui M, Murakami T, Naito T, Wada T, Takahashi T, Ishii S. Some Problems in wrist reconstruction after tumor resection with vascularized fibular-head graft. J Reconstr Microsurg 1996;12:81–8.[Web of Science][Medline]
Received March 15, 2002; accepted June 28, 2002
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