MyMedR

Fulltext Modular endoprosthetic replacement after total femur resection for malignant bone tumor

Faisham WI, Zulmi W, Halim AS

Med J Malaysia, 2005 Jul;60 Suppl C:45-8.

Total femur endoprothesis is an alternative replacement for massive malignant bone tumor with intramedullary extension or skip lesion. Four patients underwent total femoral resection and replacement with megaprosthesis: three had primary malignant bone tumor and one had salvage procedure for aseptic loosening of the distal femoral replacement. Tumor-free margins were achieved in all patients with two patients required vascularized latissimus dorsi free flap cover for reconstruction of soft tissue defects. The average follow-up was 24 months (range 16 - 60 months). All four patients were still alive with three of them being disease-free and one survived even with the presence of lung metastasis. The functional results obtained were either excellent or good in all patients in accordance to the Musculoskeletal Tumors Society grading system.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Growing endoprosthesis in management of bone tumours in Malaysia

Saravanan S, Vivek AS

Med J Malaysia, 2007 Dec;62(5):418-9.

PMID: 18705481 MyJurnal

This is to report on the use of growing endoprosthesis, also known as lengthening prosthesis in the management of four patients in the paedriatic age group in the Orthopaedic Oncolgy Unit at University Malaya Medical Centre. These are custom made prosthesis, designed and made in India based on measured roentrograms. The ages of these patients vary from 6 to 13 years old. These are cases of Osteosarcoma and Ewing's sarcoma around the knee. This is the first time these custom made prosthesis have ever been used in Malaysia. We feel that this is a feasible option for limb salvage in the treatment of primary bone tumours in growing children.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Moulded cement-prosthesis for osteosarcoma of the proximal humerus

Pan KL, Ong GB, Potukuchi AP

Med J Malaysia, 2006 Dec;61 Suppl B:55-7.

PMID: 17600994

We report a case of an 11-year-old boy with osteosarcoma of the proximal humerus treated with wide excision and reconstruction with a cement spacer-prosthesis. After seven years of follow-up, the patient is now almost a young adult. We present his current physical and functional status, which seems to defray the initial doubts regarding long-term problems when we chose this method of reconstruction.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Resection arthrodesis for primary bone tumour about the knee

Kwan MK, Ng ES, Penafort R, Saw A, Sengupta S

Med J Malaysia, 2005 Jul;60 Suppl C:66-71.

PMID: 16381287 MyJurnal

Bone defect following en bloc resection of primary bone tumor around the knee can be reconstructed by allograft or prothesis or combination of both. Resection-arthrodesis is an alternative option for young vigorous patients facing circumstances of financial constrain or limited supply of allograft. This study was undertaken to determine the outcome and complications associated with resection-arthrodesis of 22 primary bone tumors (13 giant cell tumors and 9 osteosarcomas) around the knee treated between 1990 and 2003 at the University Malaya Medical Center. The mean follow-up was 6 years (range 1-13 years). hree patients with osteosarcoma died of lung metastasis, 3 required above knee amputation and 2 defaulted follow-up. Local complications of the procedure include infection in 8 cases (36.4%), non-union 7 (31.8%) and mal-union. Of 14 patients who returned for final evaluation, 79.8% had satisfactory outcomes according to the Musculoskeletal Tumor Society grading system. In conclusion, resection-arthrodesis of the knee is a viable treatment option for selected patients with primary bone tumor around the knee, and good functional outcome can be expected in the presence of short-term local complications.

Matched MeSH terms: Osteosarcoma/surgery*

Implantation metastasis in a 13-year-old girl: a case report

Singh C, Ibrahim S, Pang KS, Shanti S

J Orthop Surg (Hong Kong), 2003 Jun;11(1):94-6.

PMID: 12810979

We report a case of a 13-year-old girl with an osteosarcoma of the right humerus, which had been diagnosed as an aneurysmal bone cyst at our institution. She underwent curettage and bone grafting of the lesion, which resulted in implantation metastasis of the tumour to the ilium. She died 15 months after presentation owing to pulmonary metastases. This report highlights the possibility of metastasis occurring by direct implantation to a graft donor site. We strongly recommend that a biopsy be performed in cases of presumed benign lesions before proceeding with the definitive surgery.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Limb salvage in osteosarcoma using autoclaved tumor-bearing bone

Pan KL, Chan WH, Ong GB, Premsenthil S, Zulkarnaen M, Norlida D, et al.

World J Surg Oncol, 2012;10:105.

PMID: 22681750 DOI: 10.1186/1477-7819-10-105

Tumor prostheses currently give the best short- and medium-term results for limb-salvage reconstruction procedures in the treatment of bone tumors. However, in developing countries, the cost of a tumor prosthesis is beyond the reach of much of the population. We report the use of autoclaved tumor-bearing bone in 10 patients, as an affordable alternative to the use of prostheses.

Matched MeSH terms: Osteosarcoma/surgery*

Endoprosthetic reconstruction for giant cell tumors of the distal tibia: a short term review

Ajit Singh V, Nasirudin N, Bernatt M

Asia Pac J Clin Oncol, 2013 Jun;9(2):182-9.

PMID: 22897856 DOI: 10.1111/j.1743-7563.2012.01553.x

Custom-made endoprosthetic reconstruction for distal tibia tumors is a viable option of treatment in carefully selected patients. It maintains satisfactory function and provides good pain relief. We report four cases of giant cell tumors of the distal tibia successfully treated by endoprosthetic reconstruction. This is a feasible option in cases of this nature and offers a better function than the other available options.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Osteosarcoma: the outcome of limb salvage surgery

Faisham WI, Zulmi W, Halim AS, Biswal BM, Mutum SS

Med J Malaysia, 2004 Dec;59 Suppl F:24-34.

PMID: 15941157

We reviewed the surgical and oncological management 23 consecutive patients with osteosarcoma of the long bones to determine the outcome of limb salvage technique performed in our centre. All patients received neoadjuvant chemotherapy. There were 15 males and 8 females with a mean age at diagnosis of 19 years (9 to 36). The median follow-up was 30 months (10 to 60). Fifteen had lesion around the knee joint followed by three in the proximal humerus, two in distal humerus, two in the pelvis, and one in the distal tibia. Six patients presented with lung metastases at diagnosis. We performed limb salvage surgery to control local disease in 16 patients and amputation in 7. The resection margins of the primary lesion were adequate and free of tumour cells in all patients. Local recurrence developed in 1 patient of limb salvage group. The overall median survival was 22 months and actuarial survival was 52% at 3 years. Eleven patients died of pulmonary metastases within 2 years of follow-up. Median survival of the limb salvage surgery group was 30 months compared to 6 months in the amputation group. As per our experience, limb salvage technique is a feasible option in extremity osteosarcoma without compromising survival.

Matched MeSH terms: Osteosarcoma/surgery*

Fulltext Survival analysis of osteosarcoma patients: A 15-year experience

Yasin NF, Abdul Rashid ML, Ajit Singh V

J Orthop Surg (Hong Kong), 2020 2 23;28(1):2309499019896662.

PMID: 32077796 DOI: 10.1177/2309499019896662

INTRODUCTION: Management of osteosarcoma has evolved considerably for the past two decades and there have been changes of practices especially pertaining to chemotherapy regime. This is a review of our cases in the past 15 years.

METHOD: This is a retrospective survival analysis study of 128 patients treated at University Malaya Medical Centre (UMMC) from 1997 to 2011.

RESULTS: There were 80 (62.5%) male and 48 (37.5%) female patients with the median age being 15 (5-59). Majority had osteosarcoma of extremities (94.5%). More than 60% patients developed metastasis throughout the course of treatment with 39% presenting with lung metastasis. Osteoblastic osteosarcoma was the commonest subtype (65.6%). Of the 109 patients treated surgically, 84 patients (65.6%) underwent limb salvage surgery while the rest underwent amputation. Seventy-one per cent of patients completed treatment with local recurrence rate of 22.7%. The 5-year and 10-year survival rates were 56.31% (95% CI: 46.20, 65.24) and 22.33% (95% CI: 14.86, 30.76), respectively. The 5-year event-free survival was 52.94% (95% CI: 41.83, 62.87). In multivariate analysis, the independent prognostic factors were presence of metastasis and completion of treatment for both 5-year and 10-year overall survival. Good histological response was only significant for multivariate analysis at 5 years. Patients with metastasis had a hazard ratio of 20.4 at 5 years and 3.26 at 10 years.

CONCLUSION: Overall survival rate for osteosarcoma patients at our centre was comparably higher than other centres in the region. Two independent risk factors for survival are metastatic status and completion of treatment. A standardized chemotherapy regime is essential for long-term survival.

Matched MeSH terms: Osteosarcoma/surgery

Fulltext Evaluation of the Receptor Activator of Nuclear Factor Kappa B Ligand (RANKL) Expression in Osteosarcoma and Its Association with the Clinicopathological Data

Chua HH, Tuan Sharif SE, Wan Ismail WFN, Zawawi MSF, Abdullah S

Asian Pac J Cancer Prev, 2021 Mar 01;22(3):741-747.

PMID: 33773537 DOI: 10.31557/APJCP.2021.22.3.741

BACKGROUND: The receptor activator of nuclear factor kappa B ligand (RANKL) is one of the key regulators of bone remodelling in bone oncology, including osteosarcoma. We assessed RANKL immunohistochemical expression in osteosarcoma, its association, and disease-free survival with the patients' clinicopathological characteristics.

METHODS: One hundred osteosarcoma cases from 2003 to 2018 in Hospital Universiti Sains Malaysia were retrieved. The tissue samples were stained for RANKL, and the association with the clinicopathological characteristics was evaluated. Staining was interpreted in a semiquantitative scoring system and classified into negative and positive expressions. Results: Eighty-two cases had a positive expression of RANKL in which 56 and 26 patients were classified as low expression and high expression, respectively. The positive expression of RANKL did not show a significant association with clinicopathological characteristics. However, Kaplan Meier survival analysis showed a significant improvement in the disease-free survival patients who underwent limb salvage surgery (LSS) than amputated patients (p-value 0.002), whereas poorer survival was observed among conventional osteosarcomas compared to non-conventional osteosarcoma (p-value 0.01).

CONCLUSION: Limb salvage surgery had proven to improve osteosarcoma patients' survival compared to amputation, which could improve overall quality of life in osteosarcoma patients. However, our data did not show a significant association between positive RANKL immunohistochemistry with any of the clinicopathological characteristics and patients' final survival. Further studies may be acquired to understand the suitability of using RANKL immunohistochemistry as prognostication in the management of osteosarcoma patients.

Matched MeSH terms: Osteosarcoma/surgery

Fulltext Major surgery in an osteosarcoma patient refusing blood transfusion: case report

Dhanoa A, Singh VA, Shanmugam R, Rajendram R

World J Surg Oncol, 2010;8:96.

PMID: 21059231 DOI: 10.1186/1477-7819-8-96

We describe an unusual case of osteosarcoma in a Jehovah's Witness patient who underwent chemotherapy and major surgery without the need for blood transfusion. This 16-year-old girl presented with osteosarcoma of the right proximal tibia requiring proximal tibia resection, followed by endoprosthesis replacement. She was successfully treated with neoadjuvant chemotherapy and surgery with the support of haematinics, granulocyte colony-stimulating factor, recombinant erythropoietin and intraoperative normovolaemic haemodilution. This case illustrates the importance of maintaining effective, open communication and exploring acceptable therapeutic alternative in the management of these patients, whilst still respecting their beliefs.

Matched MeSH terms: Osteosarcoma/surgery*

An unusual mutation in RECQ4 gene leading to Rothmund-Thomson syndrome

Balraj P, Concannon P, Jamal R, Beghini A, Hoe TS, Khoo AS, et al.

Mutat Res, 2002 Oct 31;508(1-2):99-105.

PMID: 12379465

Rothmund-Thomson syndrome (OMIM #268400) is a severe autosomal recessive genodermatosis: characterised by growth retardation, hyperpigmentation and frequently accompanied by congenital bone defects, brittle hair and hypogonadism. Mutations in helicase RECQ4 gene are responsible for a subset of cases of RTS. Only six mutations have been reported, thus, far and each affecting the coding sequence or the splice junctions. We report the first homozygous mutation in RECQ4 helicase: 2746-2756-delTGGGCTGAGGC in IVS8 responsible for the severe phenotype associated with RTS in a Malaysian pedigree. We report also a 5321 G-->A transition in exon 17 and the updated list of the RECQ4 gene mutations.

Matched MeSH terms: Osteosarcoma/surgery

Outcome of expandable endoprosthesis: A single centre retrospective review

Ajit Singh V, Earnest Kunasingh D, Haseeb A, Yasin NF

J Orthop Surg (Hong Kong), 2019 5 30;27(2):2309499019850313.

PMID: 31138060 DOI: 10.1177/2309499019850313

PURPOSE: Expandable endoprosthesis allows limb salvage in children with an option to leading a better life. However, the revision rate and implant-related complications impose as a limitation in the skeletal immature. This study investigates the functional outcomes and complications related to expandable endoprosthesis in our centre.

MATERIALS AND METHODS: Twenty surviving patients with expandable endoprosthesis from 2006 till 2015 were scored using Musculoskeletal Tumour Society (MSTS) outcomes instrument and reviewed retrospectively for range of motion of respected joints, limb length discrepancy, number of surgeries performed, complications and oncological outcomes. Patients with less than 2 years of follow-up were excluded from this study.

RESULTS: Forty-five percentage patients reached skeletal maturity with initial growing endoprosthesis and 25% of patients were revised to adult modular prosthesis. One hundred fifty-seven surgeries were performed over the 9-year period. The average MSTS score was 90.83%. The mortality rate was 10% within 5 years due to advanced disease. Infection and implant failure rate was 15% each. The event-free survival was 50% and overall survival rate was 90%.

CONCLUSION: There is no single best option for reconstruction in skeletally immature. This study demonstrates a favourable functional and survival outcome of paediatric patients with expandable endoprosthesis. The excellent MSTS functional scores reflect that patients were satisfied and adjusted well to activities of daily living following surgery despite the complications.

Matched MeSH terms: Osteosarcoma/surgery*

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