To explore the possibility that environmental and/or genetic factors may yield clues to the etiology of osteogenic sarcoma, an epidemiologic retrospective study of all cases of osteogenic sarcoma in a country with clear geographic and racial variables (Malaysia) was conducted covering a 4-year period. Sixty-eight cases were identified (+85% of the predicted total). The incidence (cases per 100,000 population per year) was 0.11 in Malay, 0.23 in the Chinese, and 0.23 in the Indian. The urban versus rural incidence in the Malay was 0.22 versus 0.09, and in the Chinese 0.31 versus 0.18. The Indian population was too small when dividied into urban and rural segments to be significant.
To our knowledge extraosseous osteosarcoma has not been reported in the Malaysian literature. We report a case of extraosseous osteosarcoma which was treated by intra-arterial chemotherapy, embolization, wide resection of tumour and salvage of limb with good functional result.
Osteosarcoma is the most common primary malignant tumour of bone and it usually metastasises to the lung. In the nasal cavity metastatic disease is extremely rare. We describe a case of osteosarcoma presenting with recurrent epistaxis, and proptosis due to secondaries in the nasal cavity. To our knowledge such a case has not been reported previously in the available English literature.
We report a case of a sixteen-month old boy with osteosarcoma of the left humerus. To the best of our knowledge this is the youngest case reported in Malaysia. This case illustrates that osteosarcoma although rare does occur in a very young child. The child died six weeks after presentation due to disseminated disease.
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.
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.
Osteosarcomas are highly malignant neoplasms of bone that are challenging to diagnose. These neoplasms often show atypical behavior. In the initial phase they may present as nondescript bony swellings with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease. Similarly, the histological growth pattern of this neoplasm can be quite diverse, presenting with areas that mimic benign myofibroblastic tumors, giant cell granulomatous conditions and partial encapsulation. The final diagnosis of an osteosarcoma is often reached after thorough sampling and examination of multiple biopsy specimens. All these clinical features and histological diagnostic difficulties were encountered in a case of osteosarcoma affecting the right mandible of a 62-year-old Chinese woman described here. The diagnostic lessons accrued from this case are discussed.
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.
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.
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.
Osteopoikilosis is a rare bone dysplasia which is inherited as an autosomal dominant trait with a prevalence of less than 0.1 per million.1 It is characterised by dense ovoid or circular spots in cancellous bone which may appear at birth or during skeletal growth. It is usually found in the metaphyseal and epiphyseal regions of long bones, the carpals and tarsals, the end of large turbular bones and around the acetabula. It is clinically asymptomatic and occasionally associated with hereditary multiple exostosis and dermatofibrosis lenticularis disseminata. It is not associated with spontaneous fractures and treatment is unnecessary. However a case of osteosarcoma developing in a man with osteopoikilosis has been reported. The first case of osteopoikilosis was reported in Malaysia four years ago in a 25 years old lady who is also of Indian descent. It would be interesting to know if these two patients are related. Since the bone lesions could easily be mistaken for metastatic disease, it is important that family physicians be aware of the benign nature of this condition.
Given that p53 is a tumor suppressor that plays a central role in the cellular response to DNA damage and that more than 50% of all cancers have mutated p53, the wider utility of photodynamic therapy (PDT) in the treatment of cancer will depend on an understanding of whether p53 status modulates response to PDT. In this study, we investigated the photosensitivity of isogenic cell lines that differ only in their p53 status to PDT using hypericin as the photosensitizer.
Radiation-induced sarcomas are well-known though uncommon potential late sequelae of radiation therapy. We report irradiation-induced osteosarcoma involving the maxilla following treatment of nasopharyngeal carcinoma in a 44-year-old man who presented with painful cheek swelling. Radiographs and computed tomography showed a large destructive lesion of the left maxilla. Diagnosis of osteosarcoma was confirmed by excision biopsy.
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.
Osteosarcoma is a rare tumour in the sinonasal region. Early diagnosis is essential for adequate management and better prognosis and this requires a meticulous histopathological examination. Reported is a case of osteosarcoma misdiagnosed as chondrosarcoma and treated by surgery followed by radiotherapy. However, appropriate diagnosis and pre-operative chemotherapy would have been significant in the prognosis. The pitfall of accurate diagnosis and the subsequent treatment is discussed in order to find the ways to maximize five years survival which is not more than 25% in this type of lesions.
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.
Biopsy is a crucial step in the management of musculoskeletal sarcoma. The surgical approach to the biopsy site is important, as the tract must be removed en bloc with the tumour during limb sparing surgery so as to reduce the risk of local recurrence. The biopsy tracts of 26 osteosarcoma patients were evaluated histologically for tumour infiltration. Horizontal sections of 1 mm thickness with 2 cm radius from were evaluated from each excised biopsy tract. Five out of 26 cases (19.2%) showed positive tumour infiltration. One case (3.85%) had tumour infiltration in the pseudocapsule, two cases (7.69%) had tumour infiltration into the muscle and two other cases (7.69%) had tumour infiltration into the subcutaneous tissue. None had skin infiltration. We conclude that resection of the entire biopsy tract is indeed mandatory for surgical treatment of osteosarcoma.
A 35 kDa glycoprotein whose abundance was previously demonstrated to be enhanced in sera of patients with endometrial adenocarcinoma (n = 12), was isolated from pooled sera of three of the cancer patients using champedak galactose-binding lectin affinity chromatography in the present study. Subjecting it to 2-DE and MS/MS, the glycoprotein was identified as the O-glycosylated fragment of inter-alpha-trypsin inhibitor heavy chain H4 (ITIH4). When compared to control sera (n = 17), expression of the 35 kDa ITIH4 cleavage fragment was demonstrated to be significantly enhanced in sera of patients with breast carcinoma (n = 10), epithelial ovarian carcinoma (n = 10), and germ cell ovarian carcinoma (n = 10) but not in patients with nasopharyngeal carcinoma (n = 13) and osteosarcoma (n = 7). The lectin-based electrophoretic bioanalytical method adopted in the present study may be used to assess the physiological relevance of ITIH4 fragmentation and its correlation with different malignancies, their stages and progression.