Radiation Therapy as an Alternative Treatment for Desmoid Fibromatosis.

AIM: To determine the outcome after radiation therapy for desmoid fibromatosis. MATERIALS AND METHODS: A retrospective review of 50 patients treated between 1988 and 2016 in a specialised bone and soft tissue tumour clinic. RESULTS: The median age at the time of radiation therapy was 36.8 years (range 15.1-69.0) and the median follow-up time was 51 months. Forty-three patients underwent radiation therapy as the definitive treatment with a median dose of 56 Gy (range 30-58.8 Gy). The median dose for the seven patients treated with postoperative radiation therapy was 50.4 Gy (range 48-56 Gy). Eleven patients (22%) developed progressive disease after radiation therapy at a median time of 41 months (range 12-113 months). The recurrences were within the radiation therapy field in four patients and outside the field in seven patients. One patient developed a radiation-induced malignancy 20 years after treatment. CONCLUSIONS: Radiation therapy is an alternative treatment in the management of desmoid fibromatosis. It should be considered in patients for whom surgical resection is not feasible, or as adjuvant therapy after surgery with involved margins where any further recurrences would cause significant morbidity.

Clinical, functional and radiological outcomes of extracorporeal irradiation in limb salvage surgery for bone tumours.

AIMS: We present a retrospective review of patients treated with extracorporeally irradiated allografts for primary and secondary bone tumours with the mid- and long-term survivorship and the functional and radiographic outcomes. PATIENTS AND METHODS: A total of 113 of 116 (97.4%) patients who were treated with extracorporeally irradiated allografts between 1996 and 2014 were followed up. Forms of treatment included reconstructions, prostheses and composite reconstructions, both with and without vascularised grafts. Survivorship was determined by the Kaplan-Meier method. Clinical outcomes were assessed using the Musculoskeletal Tumor Society (MSTS) scoring system, the Toronto Extremity Salvage Score (TESS) and Quality of Life-C30 (QLQ-30) measures. Radiographic outcomes were assessed using the International Society of Limb Salvage (ISOLS) radiographic scoring system. RESULTS: There were 61 (54%) men with a mean age of 22 years (6 to 70) and 52 (46%) women with a mean age of 26 years (3 to 85). There were 23 deaths. The five-year patient survivorship was 82.3% and the ten-year patient survivorship was 79.6%. The mean follow-up of the 90 surviving patients was 80.3 months (2 to 207). At the last follow-up, 105 allografts (92.9%) were still in place or had been at the time of death; eight (7%) had failed due to infection, local recurrence or fracture. Outcome scores were comparable with or superior to those in previous studies. The mean outcome scores were: MSTS 79% (sd 8); TESS 83% (sd 19); QLQ 82% (sd 16); ISOLS 80.5% (sd 19). Pearson correlation analysis showed a strong relationship between the MSTS and ISOLS scores (r = 0.71, p < 0.001). CONCLUSION: This study shows that extracorporeal irradiation is a versatile reconstructive technique for dealing with large defects after the resection of bone tumours with good functional and radiographic outcomes. Functional outcomes as measured by MSTS, TESS and QLQ-30 were strongly correlated to radiographic outcomes. Cite this article: Bone Joint J 2017;99-B:1681-8.

Posterior high sacral segmental disconnection prior to anterior en bloc exenteration for recurrent rectal cancer.

This article describes a novel technique for en bloc resection of locally recurrent rectal cancer that invades the high sacral bone (above S3). The involved segment of the sacrum is mobilised with osteotomes during an initial posterior approach before an anterior abdominal phase where the segment of sacral bone is delivered with the specimen. This allows en bloc resection of the involved sacrum while preserving uninvolved distal and contralateral sacral bone and nerve roots. The goal is to obtain a clear bony margin and offer a chance of cure while improving functional outcomes by maintaining pelvic stability and minimising neurological deficit.

Limb preservation surgery with extracorporeal irradiation in the management of malignant bone tumor: the oncological outcomes of 101 patients.

BACKGROUND: En bloc resection, extracorporeal irradiation (ECI) and reimplantation have been used selectively at our centers as part of limb preservation surgery of malignant bone tumors since 1996. We report the long-term oncological outcomes. PATIENTS AND METHODS: One hundred one patients were treated with ECI at two Australian centers between 1996 and 2011. A single dose of 50 Gy was delivered to the resected bone segments. The irradiated bones were reimplanted immediately as a biological graft. Patients were treated with chemotherapy as per standard protocol. The three main histological diagnoses were Ewing's sarcoma (35), osteosarcoma (37) and chondrosarcoma (20). There were nine patients with a range of different histologies. RESULTS: There was one local recurrence (2.86%) in Ewing's sarcoma and the 5-year cumulative overall survival was 81.9%. There was no local recurrence in osteosarcoma and five distant recurrences. The 5-year cumulative overall survival was 85.7%. The local recurrence rate was 20% (4 of 20) in chondrosarcoma, and the 5-year cumulative overall survival was 80.8%. Limb preservation was achieved in 97 patients. For the 64 patients with disease in the pelvis or lower limb, 53 (82.3%) could walk without aids at the time of last follow-up. CONCLUSIONS: This large series of ECI shows an excellent long-term local control. It is a good alternative reconstruction method in selected patients. The overall survival is comparable to other published series.

Extracorporeal irradiation for pelvic reconstruction in Ewing's sarcoma.

We review the treatment of pelvic Ewing's sarcoma by the implantation of extracorporeally-irradiated (ECI) autografts and compare the outcome with that of other reported methods. We treated 13 patients with ECI autografts between 1994 and 2004. There were seven males and six females with a median age of 15.7 years (interquartile range (IQR) 12.2 to 21.7). At a median follow-up of five years (IQR 1.8 to 7.4), the disease-free survival was 69% overall, and 75% if one patient with local recurrence after initial treatment elsewhere was excluded. Four patients died from distant metastases at a mean of 17 months (13 to 23). There were three complications which required operative intervention; one was a deep infection which required removal of the graft. The functional results gave a mean Musculoskeletal Tumor Society score of 85% (60% to 97%), a mean Toronto extremity salvage score of 86% (69% to 100%) and a mean Harris hip score of 92 (67 to 100). We conclude that ECI grafting is a suitable form of treatment for localised and resectable pelvic Ewing's sarcoma.

Intercalary femoral reconstruction with extracorporeal irradiated autogenous bone graft in limb-salvage surgery.

Between 1996 and 2003, 16 patients (nine female, seven male) were treated for a primary bone sarcoma of the femur by wide local excision of the tumour, extracorporeal irradiation and re-implantation. An additional vascularised fibular graft was used in 13 patients (81%). All patients were free from disease when reviewed at a minimum of two years postoperatively (mean 49.7 months (24 to 96). There were no cases of infection. Primary union was achieved after a median of nine months (interquartile range 7 to 11). Five host-donor junctions (16%) united only after a second procedure. Primary union recurred faster at metaphyseal junctions (94% (15) at a median of 7.5 months (interquartile range 4 to 12)) than at diaphyseal junctions (75% (12) at a median of 11.1 months (interquartile range 5 to 18)). Post-operatively, the median Musculoskeletal Tumour Society score was 85% (interquartile range 75 to 96) and the median Toronto Extremity Salvage score 94% (interquartile range 82 to 99). The Mankin score gave a good or excellent result in 14 patients (88%). The range of movement of the knee was significantly worse when the extracorporeally irradiated autografts were fixed by plates rather than by nails (p = 0.035). A total of 16 (62%) of the junctions of the vascularised fibular grafts underwent hypertrophy, indicating union and loading. Extracorporeal irradiation autografting with supplementary vascularised fibular grafting is a promising biological alternative for intercalary reconstruction after wide resection of malignant bone tumours of the femur.

Surgical biopsy with intra-operative frozen section. An accurate and cost-effective method for diagnosis of musculoskeletal sarcomas.

The most appropriate protocol for the biopsy of musculoskeletal tumours is controversial, with some authors advocating CT-guided core biopsy. At our hospital the initial biopsies of most musculoskeletal tumours has been by operative core biopsy with evaluation by frozen section which determines whether diagnostic tissue has been obtained and, if possible, gives the definitive diagnosis. In order to determine the accuracy and cost-effectiveness of this protocol we have undertaken a retrospective audit of biopsies of musculoskeletal tumours performed over a period of two years. A total of 104 patients had biopsies according to this regime. All gave the diagnosis apart from one minor error which did not alter the management of the patient. There was no requirement for re-biopsy. This protocol was more labour-intensive and 38% more costly than CT-guided core biopsy (AU$1804 vs AU$1308). However, the accuracy and avoidance of the anxiety associated with repeat biopsy outweighed these disadvantages.

Myopericytoma: a unifying term for a spectrum of tumours that show overlapping features with myofibroma. A review of 14 cases.

BACKGROUND: Myopericytoma (MPC) is a recently proposed term to describe a group of tumours that originate from perivascular myoid cells and show a range of histological growth patterns. Only a small number of series describing MPC have been reported. MPC is frequently misdiagnosed as a sarcoma. AIMS: To document the clinical and histopathological findings of a series of MPCs, to describe the range of growth patterns and morphological spectrum, and to compare MPC with myofibroma (MF). PATIENTS/METHODS: Fourteen patients with features of MPC and/or MF were identified from the archival files of the department of anatomical pathology, Royal Prince Alfred Hospital, Sydney, Australia. RESULTS: There were six female and eight male patients. The mean and median patient ages were 37 and 35.5 years, respectively. The tumours were located in the skin, subcutis, or superficial soft tissues of the distal extremities (13 patients) or the head and neck region (one patient), and showed a spectrum of morphological appearances. They were divided into two groups based upon the predominant growth pattern corresponding to MPC (seven cases) and MF (seven cases). The feature most suggestive of MPC was the presence of a concentric perivascular arrangement of plump spindle shaped cells. The presence of a zonation/biphasic appearance was most characteristic of MF. CONCLUSIONS: MPC exhibits a spectrum of growth patterns that overlap with MF. Tumours can be designated as MPC or MF depending on the predominant growth pattern.

En-bloc resection, extracorporeal irradiation, and re-implantation in limb salvage for bony malignancies.

We treated 50 patients with bony malignancy by en-bloc resection, extracorporeal irradiation with 50 Gy and re-implantation of the bone segment. The mean survivor follow-up was 38 months (12 to 92) when 42 patients were alive and without disease. There were four recurrences. The functional results were good according to the Mankin score (17 excellent, 13 good, nine fair, three failures), the Musculoskeletal Tumour Society score (mean 77) and the Toronto Extremity Salvage score (mean 81). There was solid union, but bone resorption was seen in some cases. The dose of radiation was lethal to all cells and produced a dead autograft of perfect fit. Extracorporeal irradiation is a useful technique for limb salvage when there is reasonable residual bone stock. It allows effective re-attachment of tendons and produces a lasting biological reconstruction. There should be no risk of local recurrence or of radiotherapy-induced malignancy in the replanted bone.

Epiphyseal osteoblastoma-like osteosarcoma.

Osteoblastoma-like osteosarcoma is a rare variant of osteosarcoma occurring in this instance in a highly unusual location: the lateral femoral condyle of a 13-year-old girl. The radiological features were non-aggressive and, although slightly unusual, were most suggestive of chondroblastoma.

Extracorporeal irradiation for malignant bone tumors.

PURPOSE: Extracorporeal irradiation (ECI) has been used selectively in the management of primary malignant bone tumors since 1996. We report our techniques for ECI and the short-term oncologic and orthopedic outcomes. METHODS AND MATERIALS: Sixteen patients with primary malignant bone tumors were treated with ECI from 1996 to 2000. The median age was 14 years. The histologic diagnoses were Ewing's sarcoma (11), osteosarcoma (4) and chondrosarcoma (1). The treated sites were femur (7), tibia (4), humerus (2), ilium (2), and sacrum (1). Following induction chemotherapy in Ewing's sarcomas and osteosarcoma, en bloc resection of the tumor and tumor-bearing bone was performed. A single dose of 50 Gy was delivered to the bone extracorporeally using either a linear accelerator (9 cases) or a blood product irradiator (7 cases). The orthopedic outcome was recorded using a standard functional scale. RESULTS: At a median follow-up of 19.5 months, there were no cases of local recurrence or graft failure. One patient required amputation due to chronic osteomyelitis. For the 10 patients with follow-up greater than 18 months, the functional outcomes were graded good to excellent. CONCLUSION: The short-term oncologic and orthopedic results are encouraging and suggest that ECI provides a good alternative for reconstruction in limb conservative surgery in selected patients. This technique should only be used in a multidisciplinary setting, where careful follow-up is available to assess the long-term outcomes.

Telangiectatic dedifferentiation of a parosteal osteosarcoma.

A unique case of parosteal osteosarcoma (POS) of the proximal femur, with areas of telangiectatic dedifferentiation, in a 28-year-old woman is reported. The patient had a 7-week history of pain and swelling in her right thigh. A biopsy diagnosis of POS was established. The patient was treated with two cycles of intraarterial chemotherapy, followed by limb salvage surgery. Histological examination of the resected specimen showed POS with areas of dedifferentiation composed of highgrade telangiectatic osteosarcoma with associated secondary aneurysmal bone cyst change.

Osteosarcoma and pulmonary metastases: 15-year experience from a single institution.

BACKGROUND: The outcome of management in patients with osteosarcoma and pulmonary metastases at a Sydney teaching hospital was reviewed. METHODS: A retrospective review was undertaken of all patients diagnosed with osteosarcoma and treated by the Bone and Soft Tissue Unit and the Medical Oncology Department, Royal Prince Alfred Hospital between 1979 and January 1995. Information was collected on demographics, tumour site, tumour histology, primary management including surgery and adjuvant therapy, and the subsequent development and management of pulmonary metastases. RESULTS: A total of 56 patients with localized osteosarcoma was seen. Overall survival and survival following pulmonary metastases was assessed. There were 33 (59%) males and 23 (41%) females, with a median age of 27 years. Survival at 5 years, for patients with non-axial osteosarcoma was 60% (95% CI, 44-77%). Pulmonary metastases without other metastatic disease being apparent, developed in 22 patients, of whom 12 underwent surgical resection. The median disease-free interval of these latter patients was 20 months (95% CI, 8-32 months). Median survival among patients not undergoing surgical resection was 5 months from detection of metastases. Patients undergoing resection of pulmonary metastases had a median survival of 17 months following detection of pulmonary metastases (95% CI, 7-27 months). Actuarial 5-year survival was 16% (95% CI, 0-42%). CONCLUSIONS: A small proportion of patients with resectable pulmonary metastases from osteosarcoma achieve long-term disease-free survival following surgical resection. It is not possible to accurately identify these patients prospectively.

Competency-based learning in traumatology.

BACKGROUND: A multidisciplinary, competency-based trauma teaching program was introduced for final year medical students (n = 67) at Royal Prince Alfred Hospital (RPAH) in 1994 to complement the surgical clerkship. METHODS: The method involved small groups rotating through a series of teaching stations each structured to address a predetermined competency. Four 3-hour sessions were held on the subject areas of resuscitation, plastic, orthopedic, and neurotrauma. Performance in the trauma section of a summative Objective Structured Clinical Examination (OSCE), 6 months after the teaching, was compared with that of a control group (n = 127) from other campuses where trauma was taught by a series of discipline-based lectures. Three trauma OSCE stations were designed to test psychomotor skills while five addressed aspects of the cognitive domain. Checklists were used to ensure standardization of scoring in a range of questions asked for skills tested at each station. RESULTS: The marks of the RPAH students (mean 78% +/- SD 9%) were significantly higher (P < 0.0005) than the controls (mean 70% +/- SD 9%) in the 8 trauma questions. There was no significant difference (P = 0.8) in marks obtained by the study group (mean 61% +/- SD 8%) and controls (mean 63% +/- SD 7%) in 22 questions sampling a wide spectrum of nontrauma subject areas. The study group performed significantly better in one of the three skills stations and three of the five problem-solving stations when compared with the control group. CONCLUSION: The innovation has the potential to fulfill a need for an integrated trauma program in the undergraduate core curriculum.

Nonunion of a pathologic juvenile scaphoid fracture after osteomyelitis.

A patient with osteomyelitis of the scaphoid in childhood resulting in a pathologic fracture with nonunion is presented. This ununited fracture of the scaphoid was successfully grafted eight years later with a satisfactory outcome.

Intraluminal allograft restoration of the upper femur in failed total hip arthroplasty.

Deep-frozen cancellous allografts have been used to restore the proximal femur of 23 hips following failed total hip arthroplasty. The canal is cleared and reaming is performed under direct vision through a cortical window. A modified Huckstep prosthesis permits early weight-bearing on the distal femur regardless of the state of proximal bone, but retains the option for later restoration of proximal loading once grafts appear mature. This transfer has been performed on four occasions. All 23 patients have had relief of pain with early ambulation. The follow-up periods ranged from six to 30 months. With the exception of one deep infection, the grafts have not undergone resorption in this period of follow-up study. Consolidation appears slower when the defect has followed multiple operations than following an initial revision for prosthetic loosening. The basic bone bank facilities are required for the supply of frozen allografts.