A Humane Option or a Step Too Far?
Eric L Masterson BSc MCh FRCS(Orth)
Fellow in Musculoskeletal Oncology
University Musculoskeletal Oncology Unit
Telephone (416) 586-8607
The first hemicorporectomy was performed by Kennedy in 1960 (2). The procedure was completed in a single stage in a 74 year old man with locally invasive rectal cancer. The patient died of pulmonary oedema 10 days post-operatively. The following year, Aust performed the first successful operation on a 29 year old paraplegic with malignancy arising in a decubitus ulcer (3). The procedure was performed in two stages and the patient survived for 19 years, before finally succumbing to pulmonary oedema.
The third reported case was described by Yancey et al in 1963 (4). Their patient had locally invasive cervical cancer and died of pulmonary oedema 4 days post-operatively.
Since then, the largest series of cases have been the series of 10 cases from Memorial Hospital in New York reported by Miller, and the 6 cases reported by Aust (5,6). The total number of hemicorporectomies which have been reported in the literature to date is 44 (7).
Indications, results and ethical considerations
To perform the procedure on a paraplegic patient with intractable ulceration and osteomyelitis about the pelvis with or without malignant change in the ulcers or sinus tracts would seem reasonable in occasional cases. Eighteen such procedures have been described with 6 deaths. Bowel and bladder function are conveniently diverted, and chronic foul smelling discharge and recurrent systemic infection from the pelvis are eliminated.
The issue of whethe or not to offer the procedure as a last resort to a patient with locally advanced pelvic malignancy is much more difficult to address. Of the 24 hemicorporectomies performed up to 1988 for malignancy, 16 have died (8). Seven deaths were due to recurrent or metastatic disease. Eight procedures have been performed for primary bone tumours. These included 5 pelvic chondrosarcomas, 1 pelvic osteosarcoma and 1 giant cell tumour of the sacrum. Of these, only 3 have become long term survivors (2 pelvic chondrosarcomas and one giant cell tumour of the sacrum).
Many surgeons are not prepared to offer their patient a hemicorporectomy under any condition, arguing that the procedure pushes the frontiers of surgery beyond what can be considered reasonable. There is little doubt that given the high mortality following this procedure, especially when performed for visceral malignancy, the indications for its use should be very restrictive. In this regard, it should be noted that 8 of the 44 reported cases to date died within one month of surgery, a peri-operative mortality rate of 18.2% (7).
The patients must have a strong desire to live and must be in good overall physical condition in order to have a reasonable prospect of surviving the procedure. If performed for malignancy, careful pre-operative staging is essential, although this cannot outrule the presence of microscopic metastases. The available survival figures for the procedure must be put to the patient in an objective way so that he or she is equipped with the necessary information to make the most daunting decision of their lives. Given the high mortality from recurrent or metastatic disease following hemicorporectomy for visceral or bone malignancy, it is likely that many patients will choose not to proceed with the operation for this particular indication. Psychological councelling should be regarded as an essential component of the pre-operative work-up. There is a strong case for extending this councelling to the patient’s family and to the hospital personnel who must care for the patient (9).
At the second stage the common iliac vessels, the vena cava, lumbar spine, dura and cauda equina are transected and the anterior and posterior skin flaps are approximated.
Hemicorporectomy results in a reduction of body weight by about 50%. The reduction in total vascular area reduces the ability to compensate for changes in circulating blood volume. Small losses may produce shock and intravenous transfusions can easily produce pulmonary oedema. The loss of normal abdominal wall function results in a reduction of lung vital capacity and functional residual capacity and a ventilation-perfusion mismatch with tidal volume diverted more to the lung apices.
Upper limb strengthening, vocational training, psychological councelling, sex hormone replacement, education and dietary advice to avoid obesity are all further aspects of what is a lengthy process of readjustment. Patients must, in addition, learn the importance of lifelong careful fluid balance, given the reduced ability of the autonomic system to autoregulate circulating blood volume.
Physical examination was unremarkable apart from a slight reduction in hip movements and a leg length discrepancy of 1.5 centimetres. Plain radiographs of the pelvis revealed an extensive tumour with chondroid features involving the peri-acetabular bone (Figure 1). In addition, there was evidence of multiple chondroid lesions throughout the pelvis and in both femora.
A diagnosis of grade II/IV chondrosarcoma with a backround of Ollier’s disease was confirmed by biopsy. Extensive intrapelvic involvement was confirmed with cross-sectional imaging and precluded limb salvage (Figure 2). The patient underwent a posterior flap hemipelvectomy (Figure 3).
Pathological assessment of the resection specimen revealed that the margins were negative for the index tumour but that there were multiple enchondromata with in situ chondrosarcoma at the bony resection margins and in the resected femur.
This young lady has made an excellent recovery from her amputation but is at very high risk of developing a second chondrosarcoma, this time in the remaining hemipelvis. This may well necessitate resection of the remaining hemipelvis, with or without the sacrum. The net result will be effectively a hemicorporectomy and seating difficulties will probably make faecal and urinary diversion necessary.