Oncontrol biopsy needle

https://www.youtube.com/watch?v=QVVxmExqaj4DSPs

 

The Oncontrol bone biopsy needle is a powered bone biopsy system used for bone marrow aspiration, but which can be used for biopsy of other bones. The driver runs at a single speed. Care should be taken to ensure the needle does not heat up when accessing hard bone.

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Dedifferentiated liposarcoma

A dedifferentiated liposarcoma is a soft tissue tumour which shows progression either in the primary tumour or after local recurrence to a sarcoma of variable histological grade, often not lipogenic. Dedifferentiated liposarcoma occurs in up to 10% of well-differentiated liposarcomas, although the risk is higher in the retroperitoneum and lower in the extremities, likely reflecting the longer time taken to detect tumours in the former location.

Dedifferentiated liposarcomas are most common in the retroperitoneum, but can occur in the spermatic cord or rarely head and neck and trunk. They are extremely rare in subcutaneous tissues. These tumours usually present as a painless mass, with both lipomatous and non-lipomatous elements on MRI scanning. Some present with an increase in size of a long-standing tumour, suggestive of dedifferentiation.

Dedifferentiated areas are variable histologically, but can resemble undifferentiated pleomorphic sarcoma or myxofibrosarcoma. Tumours most often contain ring or giant marker chromosomes.

After treatment, local recurrence can occur in up to 40% of cases, particularly in the retroperitoneum. Tumours exhibit a less aggressive clinical course than other types of high grade pleomorphic sarcoma, with metastases in 15-20% of cases and a overall mortality of 28-30% at 5 years.

 

1. WHO Classification of Tumours of Soft Tissue and Bone. 4th Edition, 2013.

Atypical lipomatous tumour

An atypical lipomatous tumour is a locally aggressive mesenchymal neoplasm in which the adipocytes show significant variation in cell size and have some nuclear atypia. These tumours are at risk of local recurrence. In the extremities or trunk the term atypical lipomatous tumour is justified as complete excision is usually curative and local recurrence manageable. In the retroperitoneum or mediastinum however, tumours with the same biological composition are difficult to completely remove and can recur with disastrous consequences for the patient. In these locations, the term “well-differentiated liposarcoma” may therefore be more appropriate.

Atypical lipomatous tumours account for 40-45% of all liposarcomas and occur predominantly in middle aged adults, peaking in the sixth decade. They most frequently occur in the deep soft tissues of the limbs, especially the thigh, but may also occur in the retroperitoneum or paratesticular area. Tumours often grow slowly, are painless and can achieve significant sizes.

Anatomical location is the most important prognostic factor. Lesions in surgically resectable sites tend not to recur after complete excision. In more difficult anatomic sites, incomplete excision is associated with local recurrence, which in turn can lead to loss of local control, or be associated with dedifferentiation and death. The risk of dedifferentiation is said to be over 20% in the retroperitoneum, but <2% in the limbs.

1. WHO Classification of Tumours of Soft Tissue and Bone. 4th Edition, 2013.

Lipoma

Lipomas are benign soft tissue tumours composed of mature adipocytes. Lipomas are the most common mesenchymal tumour in adults and are most common between the ages of 40 and 60 years. 5% of patients have multiple lipomas.

Lipomas typically occur in the subcutaneous soft tissues, but can also present in deeper locations, for example within or between muscles, or on the surface of bones.

Lipomas usually present as a painless soft tissue mass. On histological examination they can contain bone (osteolipoma), cartilage (chondrolipoma) or fibrous tissue (fibrolipoma). Deep intramuscular lipomas often encase muscle fibres – this can be seen on MRI scanning and these tumours have a higher rate of local recurrence.

Angiolipoma is a lipoma variant containing small capillary walled vessels. 5% of cases are inherited as an autosomal dominant. These usually present as tender subcutaneous masses. Angiolipomas are always benign and show no tendency to recur.

A large lipoma with stranding of muscle fibres

A large intramuscular lipoma of thigh with stranding caused by muscle fibres

1. WHO Classification of Tumours of Soft Tissue and Bone, 4th Edition.

 

Information for patients:

A good summary from NHS Choices.

Guide to trainees for the orthopaedic oncology rotation

We have compiled the following unofficial guide to trainees coming to the North of England Bone and Soft Tissue Tumour Service. It contains key topics in the curriculum to which you may get exposure in this attachment, competence levels for surgical procedures and other useful information.

Orthopaedic Oncology Syllabus

Classic papers in Orthopaedic Oncology

We recently scratched our heads and put a list of classic orthopaedic oncology papers together for this book:

http://www.amazon.co.uk/Classic-Papers-Orthopaedics-Paul-Banaszkiewicz/dp/1447154509

We were looking for papers that made an impact on the specialty or inform daily practice, as well as those which are cited widely. The list we came up with was:

 

CADE, S. (1955). Osteogenic sarcoma; a study based on 133 patients. Journal of the Royal College of Surgeons of Edinburgh, 1(2), 79–111. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/13307660
Codman, E. A. (2009). The classic: the registry of bone sarcomas as an example of the end-result idea in hospital organization. 1924. Clinical Orthopaedics and Related Research, 467(11), 2766–70. doi:10.1007/s11999-009-1048-7
Dahlin, D. C., & Coventry, M. B. (1967). Osteogenic sarcoma. A study of six hundred cases. The Journal of Bone and Joint Surgery. American Volume, 49(1), 101–10. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/5225072
Enneking, W. F., Dunham, W., Gebhardt, M. C., Malawar, M., & Pritchard, D. J. (1993). A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clinical Orthopaedics and Related Research, (286), 241–6. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8425352
Enneking, W. F., Spanier, S. S., & Goodman, M. A. (2003). A system for the surgical staging of musculoskeletal sarcoma. Clinical Orthopaedics and Related Research, (153), 106–20. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/7449206
Mankin, H. J., Lange, T. A., & Spanier, S. S. (1982). The hazards of biopsy in patients with malignant primary bone and soft-tissue tumors. The Journal of Bone and Joint Surgery. American Volume, 64(8), 1121–7. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/16951637
Mirels, H. (1989). Metastatic disease in long bones. A proposed scoring system for diagnosing impending pathologic fractures. Clinical Orthopaedics and Related Research, (249), 256–64. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2684463
O’Sullivan, B., Davis, A. M., Turcotte, R., Bell, R., Catton, C., Chabot, P., … Zee, B. (2002). Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet, 359(9325), 2235–41. doi:10.1016/S0140-6736(02)09292-9
Rosen, G., Murphy, M. L., Huvos, A. G., Gutierrez, M., & Marcove, R. C. (1976). Chemotherapy, en bloc resection, and prosthetic bone replacement in the treatment of osteogenic sarcoma. Cancer, 37(1), 1–11. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/1082364
Rosenberg, S. A., Tepper, J., Glatstein, E., Costa, J., Baker, A., Brennan, M., … Wesley, R. (1982). The Treatment of Soft-tissue Sarcomas of the Extremities. Prospective Randomized Evaluations of (1) Limb-sparing Surgery Plus Radiation Therapy Compared with Amputation and (2) the Role of Adjuvant Chemotherapy. Annals of Surgery, 196(3), 305–315. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1352604/pdf/annsurg00139-0081.pdf

What’s missing? Your comments welcomed.

Mifamurtide (MTP-PE)

What is it?

Mifamurtide is the brand name for muramyl tripeptide phosphatidylethanolamine or MTP-PE for short. It is a synthetic derivative of muramyl dipeptide (MDP), which is an immune-stimulatory component of the bacterial cell wall. It’s advantage is that it has a longer half-life in plasma than MDP.

Mifamurtide is licensed for use as an adjunct in the treatment of high-grade resectable non- metastatic osteosarcoma after macroscopically complete surgical resection in children, adolescents and young adults. There has been much debate over the evidence to support it’s use but after much deliberation the National Institute for Health and Clinical Excellence (NICE) granted permission for it’s use in the UK in October 2011.  Read more about the NICE guidance.

How does it work?

Nucleotide-binding oligomerization domain-containing protein 2, or NOD2 for short, is a receptor found in monocytes and macrophages, which recognises bacterial peptidoglycan, stimulating an immune response. MTP-PE is recognised by NOD2 and thus simulates a bacterial infection, inducing an immune response and a cascade of cytokines including Tumour Necrosis Factor alpha (TNFα), which in turn generates a macrophage-mediated attack on the cancer cells.

The use of an immune stimulant like MTP-PE makes intuitive sense in the treatment of osteosarcoma, as post-resection infection has been shown to increase overall survival. Read more about infection in osteosarcoma.

The Evidence

There is one randomised controlled trial looking at the effect of Mifamurtide as an adjunct in the treatment of osteosarcoma. Meyers et al and the Children’s Oncology Group published the trial of 662 young people with localised, resectable osteosarcoma. They were randomly assigned to high-dose methotrexate, cisplatin, and doxorubicin plus or minus ifosfamide in a 2×2 factorial design, which also included a randomised evaluation of MTP. The study concluded that the addition of MTP to chemotherapy improved 6-year overall survival from 70% to 78% (P=0.03). The study has however generated much controversy. Read the paper for yourself in the Journal of Clinical Oncology.

Critics of the paper cite differences in the chemotherapy regimes in the different arms of the study, with cisplatin omitted from pre-operative chemotherapy in the ifosfamide-containing arm. Questions have also been raised about the apparent lack of effect of MTP on event-free survival (EFS) while at the same time overall survival (OS) is improved, as other studies suggest that EFS and OS are inextricably linked.

In defence of the paper it is pointed out that the trial was only powered to show a difference in effect from the MTP, which it does when the addition of MTP is looked at in isolation. It is also pointed out that MTP-containing arms of the trial had worse tumour necrosis at the time of resection, normally associated with a poorer prognosis, and yet those patients had the reported improvement in survival despite this.

You can read an eloquent commentary on some of the controversies in the Journal of Clinical Oncology.

You can read a riposte from the authors of the RCT of MTP, also in the Journal of Clinical Oncology.

  1. Meyers PA, Schwartz CL, Krailo MD, et al: Osteosarcoma: The addition of muramyl tripeptide to chemotherapy improves overall survival—A report from the Children’s Oncology Group. J Clin Oncol 26:633-638, 2008