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Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_30 | Pages 34 - 34
1 Aug 2013
Bell S Mohammed F Mullen M Mahendra A
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Primary bone tumours of the clavicle are rare. Currently the existing literature is limited to a single case series and case reports or cases. Information regarding the patient's demographics and tumour types is therefore limited. The aim of this study was to investigate the and also suggest a management protocol for suspected primary bone tumours of the clavicle. We retrospectively reviewed the Scottish Bone Tumour Register from January 1971 to January 2012 and included all primary bone tumours of the clavicle. We identified only sixteen primary bone tumours over forty one year's highlighting the rarity of these tumours. There were ten benign and six malignant tumours with a mean age of 32 years (Range 4 to 66). The average presentation to orthopaedics after onset of symptoms was two months with five patients presenting following a pathological fracture. Malignant tumour types identified were consistent with previous literature with two cases of Ewing's sarcoma and osteosarcoma and a single case of osteosarcoma post radiotherapy and a single case of chondrosarcoma. Benign tumours were treated effectively with intralesional procedures. Malignant tumours were treated with wide local excision and subtotal or total clavicle excision. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the clavicle. This is the largest series of primary bone tumours of the clavicle in the literature


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXVIII | Pages 21 - 21
1 Jun 2012
Bell S Young P Mahendra A
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Primary bone tumours of the talus are rare. Currently the existing literature is limited to a single case series and case reports or cases described in series of foot tumours. Information regarding the patient's demographics and tumour types is therefore limited. The aim of this study was to investigate these questions and also suggest a management protocol for suspected primary bone tumours of the talus. We retrospectively reviewed the Scottish Bone Tumour Register from January 1954 to May 2010 and included all primary bone tumours of the talus. We identified only twenty three bone tumours over fifty six years highlighting the rarity of these tumours. There were twenty benign and three malignant tumours with a mean age of twenty eight years. A delay in presentation was common with a mean time from onset of symptoms to diagnosis of ten months. Tumour types identified were consistent with previous literature. We identified cases of desmoplastic fibroma and intraosseous lipodystrophy described for the first time. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the talus. This is the largest series of primary bone tumours of the talus in the literature


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_2 | Pages 2 - 2
1 Mar 2021
McAleese T Clesham K Moloney D Hughes A Faheem N Merghani K
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Abstract. Background. Schwannomas are slow-growing, benign tumours normally originating from the Schwann cells of the nerve sheath. Intraosseous schwannoma accounts for 0.175% of primary bone tumours and extremely rare especially outside the axial skeleton. Monoclonal gammopathy has been associated with soft tissue schwannomas but never with the intraosseous variety. Presenting problem. A 55-year-old woman with a background of monoclonal gammopathy of undetermined significance (MGUS) presented with a 2-year history of right thigh pain. CT scan showed a well defined, lytic lesion with a thin peripheral rim of sclerosis in the midshaft of the femur. MRI displayed a hyperintense, well marginated and homogenous lesion. Definitive diagnosis was made based on the classical histopathological appearance of schwannoma. Clinical management. We managed our patient with local curettage and prophylactic cephalomedullary nailing on the basis of a high mirel score. Discussion. Intraosseous schwannomas are poorly understood but most commonly reported in middle-aged women. Radiologically, their differential diagnosis includes malignant bone tumours, solitary bone cysts, aneurysmal bone cysts and giant cell tumours. As a result, they are usually diagnosed incidentally on histology. Although malignant transformation is possible in soft tissue schwannomas, all intraosseous schwannomas reported to date have been benign. This case demonstrates the importance of suspecting intraosseous schwannoma as a differential diagnosis for lytic bone lesions to avoid the overtreatment of patients. We also highlight monoclonal gammopathy of undetermined significance as a potential risk factor for a poorly understood disease and make recommendations about the appropriate management of these lesions. Declaration of Interest. (b) declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported:I declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_7 | Pages 18 - 18
1 Apr 2014
Halai M Gupta S Spence S Wallace D Rymaszewski L Mahendra A
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Primary bony tumours of the elbow account for approximately 1% of all osseous tumours. The delayed diagnosis is commonly reported in the literature as a result of lack of clinician familiarity. We present the largest series of primary bone tumours of the elbow in the English literature. We sought to identify characteristics specific to primary elbow tumours and compare these to the current literature. We discuss cases of misdiagnosis and reasons for any delay in diagnosis. The authors also recommend a collaborative protocol for the diagnosis and management of these rare tumours. A prospectively collected national database of all bone tumours is maintained by an independent clerk. The registry and case notes were retrospectively reviewed from January 1954 until June 2013. Eighty cases of primary osseous elbow tumours were studied. Tumours were classified as benign or malignant and then graded according to the Enneking spectrum. There were no benign latent cases in this series. All cases in this series required surgical intervention. These cases presented with persistent rest pain, with or without swelling. The distal humerus was responsible for the majority and most aggressive of cases. The multidisciplinary approach at a specialist centre is integral to management. Misdiagnosis was evident in 12.5 % of all cases. Malignant tumours carried a 5-year mortality of 61%. Benign tumours exhibited a 19% recurrence rate and in particular, giant cell tumour was very aggressive. The evolution in treatment modalities has clearly benefited patients. Clinicians should be aware that elbow tumours can be initially misdiagnosed as soft tissue injuries or cysts. The suspicion of a tumour should be raised in the patient with unremitting, unexplained non-mechanical bony elbow pain. We suggest an investigatory and treatment protocol to avoid a delay to diagnosis. With high rates of local recurrence, we recommend regular postoperative reviews


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_11 | Pages 105 - 105
1 Jul 2014
Bonuccelli G Avnet S Fotia C Salerno M Grisendi G Granchi D Dominici M Baldini N
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Summary. Reciprocal metabolic reprogramming of MSCs and osteosarcoma cells influences tumor-stroma cross talk. Drugs targeting Warburg metabolism may define innovative therapeutic approaches in osteosarcoma. Introduction. Osteosarcoma (OS) is a malignant primary bone tumour of mesenchymal origin, in which cells with stem-like characteristics (CSCs) have been described. Recent studies have demonstrated a mutual interaction between stroma and tumor cells in exploiting a role in the pathogenesis and progression of cancer, and also in the enhancing stemness phenotype. Here we take in consideration the complex juxtacrine and paracrine intercellular cross talk played by mesenchymal stromal cells (MSCs) with adherent osteosarcoma cells and OS cells with stem-like characteristics (CSCs). Methods. MSCs were isolated from human adipose tissue and expanded. To evaluate the interaction between the stroma and the cancer cell compartment, we used two different osteosarcoma cancer cell lines (Saos-2 and HOS) and co-cultured them with MSCs. The different cell populations were sorted to study the reciprocal interaction including metabolic reprogramming. CSCs were obtained from SAOS-2 and HOS cell lines using the sphere formation assay and characterised for their self-renewal, mesenchymal stem cell properties and expression of pluripotency markers. CSCs sensitivity to paracrine factors produced by human MSCs was analysed in a model of co-culture system. Mitochondrial activity in the co-culture systems was also evaluated. Results. Our results revealed that upon intercellular contact, MSCs undergo Warburg metabolism and mitochondrial oxidative stress. In particular, the cell contact activated the stromal component, triggering autophagy and increased expression of monocarboxylate transporter-4 (MCT4) responsible for the extrusion of lactate. Conversely, osteosarcoma cancer cells, upon contact with MSCs, increased their aerobic metabolism and their development. In addition, using a co-culture method without cell contact, we observed that MSCs grown in serum-starvation conditions promoted proliferation of the CSCs obtained from OS cells, probably by secreting one or several soluble factors. By Real Time PCR we evaluated the gene expression of stemness markers like Oct4, Nanog and SOX2, which appeared to have increased in CSCs. Conclusions. The final goal is to have a better understanding of the role of the stroma on tumor cell metabolism. Apparently, our data show that MSCs may exploit a role in the modulation of tumor metabolic activity and stemness/differentiation in osteosarcoma. Importantly, these findings suggest an adaptation of cancer cells to bioenergetic changes “engaging” stromal cells as their survival strategy. Understanding the interactions in the tumor microenvironment should present new opportunities for the design of cancer therapeutics


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 11 | Pages 1528 - 1532
1 Nov 2008
Verdegaal SHM Corver WE Hogendoorn PCW Taminiau AHM

Surgery is considered to be the most effective treatment for cartilaginous tumours. In recent years, a trend has emerged for patients with low-grade tumours to be treated less invasively using curettage followed by various forms of adjuvant therapy. We investigated the potential for phenol to be used as an adjuvant. Using a human chondrosarcoma-derived cartilage-producing cell line OUMS-27 as an in vitro model we studied the cytotoxic effect of phenol and ethanol. Since ethanol is the standard substance used to rinse phenol out of a bone cavity, we included an assessment of ethanol to see whether this was an important secondary factor with respect to cell death. The latter was assessed by flow cytometry.

A cytotoxic effect was found for concentrations of phenol of 1.5% and of ethanol of 42.5%. These results may provide a clinical rationale for the use of both phenol and ethanol as adjuvant therapy after intralesional curettage in low-grade central chondrosarcoma and justify further investigation.


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 9 | Pages 1249 - 1255
1 Sep 2008
Nishida H Tsuchiya H Tomita K

We evaluated the possible induction of a systemic immune response to increase anti-tumour activity by the re-implantation of destructive tumour tissue treated by liquid nitrogen in a murine osteosarcoma (LM8) model. The tumours were randomised to treatment by excision alone or by cryotreatment after excision. Tissue from the tumour was frozen in liquid nitrogen, thawed in distilled water and then re-implanted in the same animal. In addition, some mice received an immunological response modifier of OK-432 after treatment. We measured the levels of interferon-gamma and interleukin-12 cytokines and the cytotoxicity activity of splenocytes against murine LM8 osteosarcoma cells. The number of lung and the size of abdominal metastases were also measured.

Re-implantation of tumour tissue after cryotreatment activated immune responses and inhibited metastatic tumour growth. OK-432 synergistically enhanced the anti-tumour effect. Our results suggest that the treatment of malignant bone tumours by reconstruction using autografts containing tumours which have been treated by liquid nitrogen may be of clinical value.