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Correct - Multiple Hereditary Exostoses, also known as Diaphyseal Aclasia, is an autosomal dominant condition predisposing to the development of ostoechondromas.
Incorrect - The surface of the lesion is covered with growth plate cartilage. Once the patient reaches skeletal maturity, usually in the early twenties, the normal growth plates shut down, as do the growth plates on the osteochondromas. Growth in a skeletally mature patient is concerning for malignant transformation.
Incorrect - The risk is hard to predict, but malignant transformation to low grade chondrosarcoma certainly does occur. The risk seems to be about 1 in 1000 in sporadic tumours, and about 1-25% for patients with MHE.
Incorrect - The best primary investigation is a plain X-Ray. In some areas like the pelvis a CT scan may be required due to the complexity of the anatomy. If there is concern for malignant change, an MRI may be required to assess the cartilage cap.
A 35 year old man presents to his family doctor with a fast growing, 15 cm soft tissue mass in his right thigh. He has noticed it for three months. You examine him and think the lesion is deep. What is the next best step?
Incorrect - Unless an MRI can be obtained urgently, this will only delay things. Most of the multi-disciplinary centres have access to urgent MRIs that will be done according to tumour protocols. However, if you have access to an MRI in the next few days, then it may be reasonable to proceed with it.
Incorrect - The vast majority of soft tissue sarcoma patients can be worked up as outpatients. There is no doubt that this man is in an urgent situation, but most centres will see him, arrange his staging and his biopsy and have a treatment plan just as quickly as an outpatient.
Incorrect - A poorly placed biopsy will lead to a higher risk of amputation being required as these tumours are highly transplantable.
Correct - With a history like this there is no need to do any investigations as this is highly suspicious for a soft tissue sarcoma.
Incorrect - Although most common in childhood, there is a second peak of incidence in late adulthood.
Correct - With modern chemotherapy and surgery the majority of patients presenting with an osteosarcoma can be cured of the disease. There is a lot of research and work to try and improve this number further.
Incorrect - Although there are several subtypes of osteosarcoma, they all have the potential to metastasize.
Incorrect - Although, unfortunately, amputation is occasionally required, most of the time modern techniques can result in limb salvage.
A patient undergoes treatment including 5000cGy of external beam radiation and wide resection surgery for a large, deep high grade soft tissue sarcoma in the thigh. There is no evidence of metastases. How will you counsel the patient?
Incorrect - While this is serious, and more follow up is needed, it is not inevitable that this patient will die from this disease.
Correct - Survival rates for this scenario are between 50 and 60% in most studies.
Incorrect - In some circumstances resection of metastases while they are small can be associated with prolonged disease free intervals, so it is reasonable to screen patients for disease.
Incorrect - Potential long term effects include swelling, joint stiffness and second malignancy
A patient has a superficial lesion that is 4 cm in size. It feels like a lipoma. Is it safe for the family doctor to remove this in her office?
Correct - Even if this does turn out to be a sarcoma, re-excision of these lesions by a multidisciplinary team has excellent outcomes.
Incorrect - There is no need to send every mass, but if she was concerned then calling a local subspecialist for advice would never be wrong.
Giant Cell Tumour of Bone:
Incorrect - Although 1% of Giant Cell Tumours of Bone can metastasize, the vast majority behave in a benign fashion.
Incorrect - Giant Cell Tumours of Bone typically occur at the ends of the long bones.
Correct - They are one of the more common benign bone tumours.
Incorrect - Giant Cell Tumours of Bone almost never occur prior to skeletal maturity.
An MRI scan can definitively diagnose a soft tissue sarcoma:
Incorrect - A definitive diagnosis almost always requires a biopsy.
Correct - While an MRI can often suggest the diagnosis, a tissue diagnosis is sensible before undertaking major interventions.
Referring to the causation of sarcomas:
Correct - Most sarcomas are apparently random events that can affect anyone of any age.
Incorrect - Although some conditions such as Li Fraumeni syndrome can predispose to sarcoma occurence, these are uncommon.
Incorrect - A small percentage (approximately 1 in 1000) of patients receiving Radiation Therapy will go on to develop a radiation-induced sarcoma.
Incorrect - Although many patients will recall a history of trauma to the affected area, and often attribute their mass to that event, there is no evidence that acute trauma will cause a sarcoma. This is a good example of recall bias.
In the treatment of malignant primary bone tumours (bone sarcomas), Radiation Therapy has an important role in the primary treatment of:
Correct - In fact, there are some instances where radiation may be given without surgery being required.
Incorrect - Radiation Therapy is generally only used in palliative care settings.
Incorrect - Radiation Therapy is not particularly effective against chondroid tumours and is generally only used in palliative care settings.
Multidiscplinary teams include specialists from which of the following disciplines?
Correct - Multidisciplinary teams are important to bring a wide range of expertise to bear on these difficult problems.