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The proximal humerus is the fourth most common site for primary bone sarcomas, with up to 15% of osteosarcomas and 10% of Ewing’s sarcomas occurring at this site. Preserving growth and function following limb-salvage surgery in paediatric patients remains a challenge, as the proximal humeral physeal growth is responsible for 80% of the total growth of the humerus, and loss of one of the physes is associated with limb-length discrepancy. This is particularly challenging in small children in whom endoprosthetic replacements are often too large and expandable prostheses have higher rates of complications compared with adults.
Vascularised autografts may provide rapid biological incorporation with the potential for growth and a limb-salvage reconstruction with longevity.
Vascularised fibular epiphyseal transfer preserves function and growth in young children following excision of the proximal humerus for a malignant bone tumour. Function compares favourably to other limb-salvage procedures in children. Failures are caused by avascular necrosis of the graft but the reported survival of the reconstruction is 82% at five years.
Author: Mr Jonathan Stevenson FRCS (Tr & Orth)
Institution: The Royal Orthopaedic Hospital ,Birmingham ,UK.

INDICATIONS
Reconstruction following wide resection of bone tumours of the proximal humerus in adults is reliably addressed with endoprostheses, which are associated with comparable function, fewer complications, and longer durability than allograft-prosthesis composite, osteoarticular allograft, or clavicular pro humero reconstructions. Preserving growth and function following limb-salvage surgery in paediatric patients remains a challenge, as the proximal humeral physeal growth is responsible for 80% of the total growth of the humerus, and loss of one of the physes is associated with limb-length discrepancy. This is particularly challenging in small children in whom endoprosthetic replacements are often too large and expandable prostheses have higher rates of complications compared with adults.
Cadaveric anatomical research during the 1980s identified reliable blood supply to the proximal epiphysis and the proximal two-thirds of the diaphysis of the fibula from the anterior tibial artery. Developed by Marco Innocenti of Florence, this became the preferred pedicle for vascularised transfer of the proximal fibular epiphysis to reconstruct the proximal humerus and distal radius, showing evidence of graft hypertrophy and longitudinal growth at the epiphysis. Vascularised epiphyseal transfers have the advantage of more predictable growth and prevent premature physeal closure when compared with non-vascularised grafts, and rapid autograft incorporation in limbs compromised by chemotherapy or radiotherapy. These rare and demanding operations require careful pre-operative discussion with parents and liaison with plastic surgery colleagues.
SYMPTOMS & EXAMINATION
Patients with primary bone tumours present principally with pain and swelling, with or without limitation of motion (and reduced ability to bear weight in the lower limb).
Biopsy tract, range of motion of the shoulder and elbow, deformity, neurovascular status, skin quality including previous scars and sinuses, deltoid function, regional lymphadenopathy and availability of local soft tissue flaps should all be considered in the clinical examination.
IMAGING
Plain radiographs usually delineate the size and location of the tumour as well as any extra-osseous extension. Radiographs are assessed for the extracellular matrix deposition of the tumour be that osteoid (e.g. osteosarcoma), chondroid (e.g. chondrosarcoma) or fibrous (fibrosarcoma of bone) which hints at the diagnosis. The age and anatomical location (epiphyseal, metaphyseal, diaphyseal) are important clues in narrowing the differential diagnosis.
In the proximal humerus necessary radiographs include measured radiographs of the whole humerus AP and lateral and including the gleno-humeral and elbow joints.
Patients undergo biopsy to confirm the histological diagnosis. In our centre biopsies either occur in the operating theatre using a Jamshidi needle and fluoroscopy or CT guidance typically via a deltopectoral approach. The positioning of the biopsy tract is crucial to ensure that is does not cross more than one anatomical compartment or compromise vital structures as the biopsy tract is excised en-bloc at the time of surgery. Typically the histological biopsy results take 7 to 10 days to be reported and are discussed at the sarcoma multi-disciplinary team (MDT) meeting comprising orthopaedic surgeons, radiologists, pathologists and oncologists.
Prior to surgery patients are ‘staged’, which involves local staging of the affected bone (i.e. MRI scan of the whole humerus including the gleno-humeral and elbow joints) and distal staging (CT chest and whole body bone scintigraphy) to exclude metastatic disease. If metastases are identified at the time of diagnostic staging this may influence the extent of surgical and systemic treatment but may not preclude surgical excision of the primary tumour; all of these details are considered at the sarcoma MDT when deciding upon the optimum treatment by all involved specialists.
ALTERNATIVE OPERATIVE TREATMENT
For primary malignant bone tumours of the proximal humerus, alternatives to vascularised fibula autograft reconstruction include endoprosthetic replacement, allograft or allograft prosthetic composite. Endoprosthetic replacements are the most commonly used limb-salvage reconstruction of the proximal humerus in our centre, however in very young children the residual bone is often too small to accept the stem of a prosthesis. Although ‘growing’ endoprostheses in this location have been used they may require multiple invasive lengthening procedures and the abduction and forward flexion are poor (https://online.boneandjoint.org.uk/doi/pdf/10.1302/0301-620X.81B3.0810495).
Allografts and allograft prosthetic composites reconstruct the proximal humerus with an allograft matched in all dimensions to the planned resection, secured with plates and screws. The latter involves pre-operative insertion of a prosthetic humeral component and then osteosynthesis of the allograft to the residual distal humerus. In some centres, allografts are augmented with vascularised fibula autografts (Capanna technique) to encourage host bone incorporation and minimise fracture and non-union rates.
NON-OPERATIVE MANAGEMENT
Osteosarcomas typically receive pre-operative chemotherapy, then surgical resection and limb-salvage followed by further post-operative chemotherapy. Some tumours, such as Ewing’s sarcoma are relatively sensitive to radiotherapy which may be a non-operative intervention in some appendicular Ewing’s sarcomas.
CONTRAINDICATIONS
If the patient was medically unfit to undergo major surgery or if the staging revealed that the disease had already spread to the lungs with a very poor prognosis then surgery may be contraindicated although palliative surgery to control pain may still be contemplated.

GA + spinal block, urinary catheter, arterial line
Supine with right upper limb and right lower limb isolated using drapes
Tourniquet right proximal thigh
IV Antibiotics (flucloxacillin and gentamicin), ultra clean theatre
Double alcoholic skin preparation, Ioban incisional drapes
Microscope prepared for vascular anastomosis

High dependency level care.
Neurovascular observations of both the right upper and lower limbs.
Antibiotics for 24 hours.
Sling to be maintained without passive movement for six weeks and then passive movement of the neo-glenohumeral joint under physiotherapy guidance from weeks six to eight.
Check radiographs and commencing active motion, further X-rays at three months of the whole humerus.
A hinged knee brace to be applied to the knee to prevent coronal instability for eight weeks.
Wheelchair until brace removed from the knee.
MDT discussion of histology results: Histology has confirmed 95% necrosis and wide margins of growth in 2mm of the neurovascular bundle medially.

There is a paucity of data describing the outcome of this procedure in children for primary bone sarcomas. At a mean follow-up of 5.2 years, Stevenson et al. reported a rate of limb-salvage of 100% and a rate of overall survival, local recurrence and metastasis of 91% (https://online.boneandjoint.org.uk/doi/full/10.1302/0301-620X.100B4.BJJ-2017-0830.R1).
Manfrini M , et al. Evolution of surgical treatment for sarcomas of proximal humerus in children: retrospective review at a single institute over 30 years. J Pediatr Orthop 2011;31:56–64.
The mean functional MSTS score of 77% reported by Stevenson is identical to that reported by Manfrini et al. in eleven children with the same reconstruction, and slightly inferior to the 80% in their three allograft-prosthetic composite patients
Emori M, et al . Vascularised fibular grafts for reconstruction of extremity bone defects after resection of bone and soft-tissue tumours: a single institutional study of 49 patients. Bone Joint J 2017;99-B:1237–1243.
The mean growth at the transplanted proximal fibular epiphysis was 4.6 mm per annum in the grafts that did not develop avascular necrosis. 18% did develop avascular necrosis of the graft, which was in keeping with previous studies by Manfrini et al. and Emori et al.
Reference
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