Laporan Kasus

Elbow Reconstruction for Distal Humerus Osteosarcoma by Using Extracorporeal Irradiation Autograft

Yanuarso, A Fauzi K, Errol Untung Hutagalung

Division of Orthopaedic and Traumatology, Faculty of Medicine University of Indonesia

Abstract: Malignant bone tumor around elbow joint is a rare case. Reconstruction with resection and extracorporeal irradiation is one type of limb salvage procedures. Management of patients with malignant bone tumor of the elbow joints with limb salvage surgery has become the choice of care. A limb-sparing approach is applicable to virtually any bone lession, whether low grade or high grade. Two cases with osteosarcoma in the distal humerus were reported. One case was diagnosed as conventional osteosarcoma and the other as recurrence parosteal osteosarcoma. The diagnosis was established in clinicopathological conference that consist of orthopaedic surgeon, radiologist, pathologist, and hemato-oncologist. Using the extracorporeal irradiation of osteoarticular autogaft limb salvage procedures of distal humerus osteosarcoma were performed. Four weeks after operation the patients regained range of motion from 40 to 95 degree and pain-free elbow function. Evidence of local recurrences or distance metastases, healing process and function of elbow joint were still evaluated. Key words: limb salvage, distal humerus primary malignant bone tumor.

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Elbow Reconstruction for Distal Humerus Osteosarcoma

Rekontruksi Siku pada Osteosarkoma Humerus Distal Extracorporeal Irradiation Graft Yanuarso, A Fauzi K, Errol Untung Hutagalung
Divisi Ortopedi dan Traumatologi, Fakultas Kedokteran Universitas Indonesia

Abstrak: Tumor ganas tulang di sekitar sendi siku adalah kasus yang jarang ditemui. Rekontruksi dengan cara rejeksi dengan penyinaran ekstrakorporal adalah salah satu prosedur untuk menyelamatkan lengan. Penatalaksanaan pasien dengan tumor tulang ganas pada sendi siku dengan metode ini telah menjadi metode terpilih. Dilaporkan dua kasus osteosarkoma pada humerus distal, kasus pertama didiagnosis sebagai osteosarkoma konvensional dan kasus kedua sebagai osteosarkoma parosteal rekurens. Diagnosis ditegakkan melalui konferensi klinik patologi. Terdiri atas bedah ortopedi, radiology, patologi, dan hematolo-onkologi. Menggunakan iradiasi ekstrakorporal dari autograft osteoalrtikular dilakukan prosedur penyelamatan lengan dengan osteosarkoma humerus distal. Empat minggu setelah pembedahan pasien mendapatkan kembali range of motion dari 40 menjadi 95 derajat disertai hilangnya nyeri pada siku. Rekurens, metastasis jauh, proses penyembuhan, dan fungsi sendi siku masih dalam evaluasi. Kata kunci: penyelamatan lengan, humerus distal, tumor ganas tulang primer

Introduction Malignant bone tumors are rare lesions and form only about 1.3% of the cancer cases at Cipto Mangunkusumo Hospital and 1-2% at Mayo Clinic. Osteosarcoma is the 2nd most common malignant bone tumors after multiple myeloma (about 19.19%).1 These tumors have long been known to be very aggressive in their natural history and therefore for a very long time amputation was considered to be the only way to achieve local control of the tumor in the limb. Even after amputation, only 10-20% survived, the rest succumbed to systemic disease.2 The disease is common in first and second decade of life, male than female and localized at around knee, proximal humerus and distal radius. Symptoms complained by patient wase intermittent pain at first and swelling. Limb salvage surgery includes all of the surgical procedures designed to accomplish removal of a malignant tumor and reconstruction of the limb with an acceptable oncologic, functional, and cosmetic result. Tumor recurrence, metastases, and a generally dismal prognosis were powerful deterrent to progress in treatment. Limb salvage surgery has replaced amputation as the treatment of choice for sarcomas of the extremities. This dramatic change came about as the result of two important developments: effective chemotherapy and precision imaging techniques.3

Case Presentation FIRST CASE A 22 year-old female presented with history of lump that got bigger in the left distal humerus for six months. Pain and loss of function were present as well. There was no history of trauma. On physical findings there was a mass with 26 cm in circumferential length (20 cm in the health tissue), firm, tender, fixed with ill-defined margin (fig.1 a-b).

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Fig 1 a-b. Tumor at the Left Elbow

Laboratory findings showed haemoglobine and white blood cells 12.8 g/dl and 8.100/l respectively, and slightly increased eritrocyte sedimentation rate 29 mm/h, serum alka-

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Elbow Reconstruction for Distal Humerus Osteosarcoma line phosphatase 161 u/l (40 150) and lactate dehidrogenase 303 u/m (230 460). Radiologic examination of left elbow AP and lateral (January 2005) showed blastic and lytic lesions in the left distal humerus, with a periosteal reaction and soft tissue swelling (fig. 2 a-b). After induction chemotherapy for three cycles, tumor became more sclerotic and solid (fig. 2 c-d). CT scan showed lytic-sclerotic lesion with irregular margin at the distal humerus, thickening of cortex and periosteal new bone formation and good medullary cavity (fig. 3 a-d). Thoracic X-Ray showed no evidence of metastatic feature and bone scintigraphy showed increased uptake only at the left elbow and neither showed no evidence of metastatic disease (fig. 4 and 5). According to the Enneking surgical staging system, the case was classified as stage IIB.

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Fig. 2 a-b. Elbow AP and Lateral X Rays: Before Induction Chemotherapy Revealed Blastic and Lytic Lesions with Periosteal Reaction.

Fig. 4. Thoracic X Ray

Fig. 5. Bone Scintigraphy

The patient was admitted and underwent a fine needle biopsy and discussed in the Clinico-Pathological-Conference (CPC). A Fine needle biopsy result was positif sarcoma as osteosarcoma (fig. 6).

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(d) Fig. 6. Cytology Examination Showed Spindle Cells, Pleomor phic with Osteoid Positif

Fig 2 c-d. Elbow AP and Lateral X rays: After Induction CheMotherapy Revealed More Sclerotic and More Solid

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Fig. 3 a-d. CT scan Showed Lytic-sclerotic Lesion with Irregular Margin, Thickening of Cortex and Periosteal New Bone Formation and Good Medullary Cavity. Maj Kedokt Indon, Volum: 57, Nomor: 3, Maret 2007 97

Elbow Reconstruction for Distal Humerus Osteosarcoma From CPC the patient was diagnosed as conventional osteosarcoma and planned to undergo induction chemotherapy. Induction chemotherapy consisted of three cycles cysplatinum and doxorubicin. After three cycles induction chemotherapy, clinical and radiological showed a significant reduction of the mass and more sclerotic and clearer demarcation of tumor, thus confirming the efficiency of the chemotherapy. Therefore, the patient was scheduled for wide excision of the tumor and limb salvage surgery using an extracorporeal irradiation of distal humerus.The patient underwent two stage operations. First operation was resection of the half distal humerus and excision of the tumor mass (fig. 7 a-c). The second was reconstruction of the half distal humerus and elbow by using extracorporeal irradiation autograft (fig. 7 d-g). In the first stage the posterior approach of the humerus was performed. Skin incision was drawn along the shaft of the humerus and extended to proximal ulna. Osteotomy of olecranon was performed. Radial and ulnar nerves were conserved. Muscle groups were conserved except for the part of the triceps and brachialis, which were attached to the tumoral process. Common flexor and extensor origins were detached and marked. After resection of the shaft of the humerus, the tumor mass was sent to the Anatomy Pathological Department, while the half distal humerus was sent to BATAN for irradiation with dose of at least 30,000 rads.4 In the second stage on the following day, the extracorporeal irradiation of half distal humerus was harvested and fixed into the proximal shaft with plate and screw. The olecranon fixed with the tension band wire and the common flexor and extensor origins were sutered again to the original sites. The radiohumeral joint was fixed with K wire for temporary immobilization with the back slab (fig. 8 a-c).

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(c) Fig. 8 a-c. Postoperative X Rays: Humeral Shaft Fixed with Plate and Screw and Olecranon Fixed with TBW

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Fig. 7 a. Posterior Approach Humerus with Osteotomy of Olecranon. The Distal Shaft of Humerus Had Been Resected. Fig 7 b-c. Distal Humerus After the Tumor Excised, Which Raedy for Irradiation. Fig. d-g Showed the Second Stage Operation i.e. Reconstruction.

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Elbow Reconstruction for Distal Humerus Osteosarcoma Histological examination of the surgical specimen revealed much residual viable tumor cells and the tumor was classified as unresponder to induction chemotherapeutic agents (Huvos 1) (fig. 9 a-c). After surgery, the patient was planned to receive adjuvant chemotherapy consisting of other agents. u/l (40 150) and lactate dehidrogenase 165 u/m (230 460). Radiologic findings from left elbow AP and lateral views (April, 2005) showed heavily mineralized mass attached by broad base to the posterior aspect of left distal humerus and soft tissue swelling (fig. 11 a-b).

(a) Fig. 9 a-c.

(b) (c) Histopathology from Tumor Specimen that Excised in the First stage (After Induction Chemo Therapy). Histological Examination of the Surgical Specimen Revealed Much Residual Viable Tumor Cells and the Tumor was Classified as Unresponder (Huvos 1) (c) (a) (b)

Second Case A woman, 30 year-old, presented with recurrent lump that was getting bigger at the left distal humerus since two years. The lump arised at the posterior distal portion of left shaft humerus since nine years ago (1997), and had been excised on April 2004 at a private hospital. Four months after the first surgery the lump arised again at the same site. There were painless and loss of function at the region of disease due to the mass (Fig. 10 a-c).

Fig. 11 a-b. Elbow AP and Lateral X Rays: Heavily Mineralized Mass at Posterior Aspect of Left Distal Humerus

Thoracic X-Ray showed no evidence of metastatic feature and bone scintigraphy showed inceased uptake only at the left elbow and neither showed no evidence of metastatic disease (fig. 12 and 13). According to the Enneking surgical staging system, the case was classified as stage IIB.

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(c) Fig. 12. Thoracic X Ray Fig. 13. Bone Scan

Fig. 10. Clinical Picture Showed Tumor at the Elbow

Laboratory findings defined hiemoglobin 12.85 g/dl and white blood cells 5,600/l, slightly increased eritrocyte sedimentation rate (30 mm/h), serum alkaline phosphatase 192

The patient was admitted and with reviewed slide from the first operation, discussed in the Clinico Pathological Conference (CPC). From this forum, the patient was diag-

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Elbow Reconstruction for Distal Humerus Osteosarcoma nosed recurrent of parosteal osteosarcoma (fig. 14), and planned to limb-salvage procedure using extra corporeal irradiation autograft. In the first stage the posterior approach of the humerus was performed. After resection of the shaft of the humerus, the soft tissues and the bulk of tumour were removed from the bone and tumour and soft tissues sent to The Pathological Anatomy Department. The ressected humerus was sent to BATAN for irradiation with a dose of at least 30.000 rads.4 In the second stage on the following day, the extracorporeal irradiation of half distal humerus was harvested and fixed into the proximal shaft with plate and screw. The olecranon fixed with the tension band wire and the common flexor and extensor origins were sutred again to the original sites. The radiohumeral joint was fixed with the K wire for temporary and immobilized with the back slab (fig. 16 a-b).

Fig. 14. Review Slide from First Operation Showed Spindle Cells, Minimal Cytologic Atypia and Rare Mitotic Figurec, Osteoid Positif

The patient underwent two stage operations and extracorporeal irradiation between those operation. First operation was resection of the half distal humerus, and excision of the tumor mass (fig. 15 a-d). The second was reconstruction of the half distal humerus and elbow by using extracorporeal irradiation autograft (fig. 15 e-f).

Fig. 16. a-b. Postoperative X Rays: Humeral Shaft Fixed With Plate and Screw and Olecranon Fixed With TBW

Fig. 15. (a-d).

Mass that Had Been Excised. The Distal Shaft of Humerus Had Been Resected. (e-f) Second Stage Operation i.e. Reconstruction

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Elbow Reconstruction for Distal Humerus Osteosarcoma Discussion Conventional osteosarcoma is a primary intramedullary high grade malignant tumour in which the neoplastic cells produce osteoid, even if only in small amounts. It is the most common, non-haemopoietic, primary malignant tumor of the bone with estimated incidence of 4-5 per million population. Conventional osteosarcoma is largely a disease of the young. It occurs most frequently in the second decade with 60% of patients under 25 years of age. Although 30% of osteosarcoma occur in patients over 40 years age, the predisposing condition should always be considered in older patiens (e.g. Paget disease of bone, post-radiation sarcoma). It affects males more frequently than females with ratio of 3:2. Conventional osteosarcoma shows a profound propensity for involvement of the long bones; in particular the distal femur, proximal tibia and proximal humerus. The distal femoral metaphysic is the most common site. Osteosarcoma arising in bones distal to the wrists and ankles is extremely unusual. The elbow is an uncommon site for primary bone tumors or metastatic disease. Osteosarcoma at the distal humerus is very rare. This is the first case found in Cipto Mangunkusumo Hospital Jakarta since thirty years ago. Parosteal osteosarcoma is considered separately from other osteosarcomas because it is distinctly less malignant and, therefore, its clinical behavior is vastly different. This tumor is a rare neoplasm, less than 1% of malignant tumor and just over 4% of all osteosarcoma at Mayo Clinic.1 Approximately 64% were female, two-thirds of all patients were in the third and fourth decades of life. This tumor involves femur, humerus or tibia. By far the most common site is posterior distal portion of shaft femur.1,5 The second case, a recurrent parosteal osteosarcoma at posterior distal portion of shaft humerus, is a rare case. Chemotherapy and irradiation have no place in the treatment of parosteal osteosarcoma; irradiation can turn the lession more malignant. In this case limb salvage with extracorporeal irradiation was performed.4,6 The patients should undergo MRI examination, but due to financial problem (government/JPS supports vonusvion CT), it could not be done. Work up and treatment of the patients were decided in the Clinico-Pathological Conference (CPC) that consist of orthopaedic surgeon, radiologist, pathologist and hemato-oncologist Before the 1970s, the management routinely consisted of transbone amputations or disarticulation, with dismal survival rates 10% to 20%. The development of more effective chemotherapeutic agents and imaging CT and MRI allows precise visualization and anatomic location of tumor and surrounding structures, fostering better patient selection for spesific treatment of limb salvage procedure.7 The current goal of limb salvage in the upper extremity is to achieve local control of the tumor and preserve maximal function. Complex limb salvage in the upper extremity has become a viable option with the advent of more effective chemotherapy and sophisticated imaging techniques to accurately delineate the intramedullary and soft tissue extent of tumor. In both cases, there were no major neurovascular structures encased by tumor, no extensive muscle involvement that can hinder limb salvage.7,8 A combination of prosthetic materials or segmental allografts or both can be used to reconstruct large bone defects after tumor resection. Prosthetic reconstruction of the elbow for degenerative joint have been studied extensively, but there are few published series reviewing prosthetic or allograft reconstructions of the humerus and elbow after tumor resection. Resection of an upper extremity tumor may require resection of the articular portion of the distal humerus or proximal ulna. In addition, there are vital neurovascular structures in this location that could lead to loss of function of the elbow, wrist, and hand after resection. Reconstruction of the defect using contemporary surgical techniques can provide patients with the necessary function to do routine activities of daily living. At the other site, limb salvage is more acceptable rather than limb ablation. Almost all patients in Cipto Mangunkusumo Hospital have financial problem, so they can not provide prosthetic or allograft. Autoclave or extracorporeal irradiation autograft for limb salvage procedur was usually done if the lesion presents with blastic appearance. Specifically for these, extracorporeal irradiation autograft was used. In the first case the patient was given neoadjuvant chemotherapeutic for the following reason 2,9: It controls micrometastases and improves survival Response of chemotherapy that can be evaluated after surgery, is an important prognostic factor It decreases tumor size and vascularity Pseudocapsule become thick and composed of mature fibrous tissue.

Local recurrence rates are related to the histologic features of the tumor. The incidence of metastasis also varies from 20% to 50% depending on the type of tumor. Evaluation of induction chemotherapeautic agents to the tumor consist of unresponder group (HUVOS I). In the second case, the patient was followed up for development of elbow joint function with physiotherapist without other management. Other problem is the sterilization in all of process of limb salvage procedure. There are many steps and times in this procedure that create chances to infection that can dissolve the goal. The oncologic and functional outcome of this patient is still documented after major tumor resection involving the distal humerus to determine whether aggressive limb salvage as an alternative to amputation is warranted in patients

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Elbow Reconstruction for Distal Humerus Osteosarcoma with primary tumor at the elbow. Conclusion Management of malignant bone tumors of extremities present many challenge. Advances of imaging, chemotherapy and reconstructive surgery for most patients with malignat bone tumors can offer limb sparing surgery. Functional outcome and patient satisfaction appear to be at least good, and probably better after reconstruction than after ablation. The outcome should continually improve as advance in combination of chemotherapy, surgical technique, bone allograft or autograft and postoperative management. References
1. Unni KK. Osteosarcoma & Parosteal osteosarcoma. In editor Dahlins Bone Tumors General Aspects and Data on 11,087 Cases. Philadelphia: Lippincot-Raven; 1996.p.143-95. Agarwal MG, Puri A. Limb salvage for malignant primary bone tumors: Current status with a review of the literature. Indian J of Surg 2003;65:354-60. McDonald DJ. Limb-salvage surgery for treatment of sarcomas of the extremities. Am J Roentgenol 1994;163:509-13. 4. Uyttendaele D, De Schryver A, Claessens H, Roels H, Berkvens P, Mondelaers W. Limb conservation in primary bone tumors by resection, extracorporeal irradiation and re-implantation. J Bone Joint Surg [Br] 1988;70-B:348-53. Unni KK, Inwards CY, Bridge JA, Kindblom LG, Wold LE. Fourth series Fascicle 2 Tumors of the bones and joints. Washington DC: ARP Press, 2005.p.170-7. Steenbrugge F, Poffyn B, Uyttendaele D, Verdonk R, Verstraete K. Parosteal osteosarcoma: A case report. Acta Orthopdica Belgica 2001;67:387-94. DiCaprio MR, Friedlaender GE. Malignant bone tumors: Limb sparing versus amputation. J Am Acad Orthop Surg 2003;11:2537. Abudu A, Carter SR, Grimer RJ. The outcome and functional results of diaphyseal endoprostheses after tumor excision. J Bone Joint Surg [Br] 1996;78-B:652-7. Bielack SS, Kempf-Bielack B, Delling G, Exner U, Flege S, Helmke K, et al. Prognostic factors in high-grade osteosarcoma of extremities or trunk: an analysis of 1,702 patients treated on neoadjuvant Cooperative Osteosarcoma Study Group Protocols. J Clin Oncol 2002;20:776-90.

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