Dr. Champion - Independent Medical Opinion

Thank you for asking me to comment on the care provided to the Appellant. I was asked to comment on whether the treatment and eventual outcome would have been better had the diagnosis of hemangiopericytoma been made in 1999.

I am a fellow of the Royal College of Physicians of Canada in the specialty of Internal Medicine with a Certificate of Special Competence in medical oncology. I am licensed in the province of Prince Edward Island, and practice as a general oncologist in a community cancer center. My practice includes treatment of a wide variety of cancers, and a small number of patients with brain tumors. I am not a neuro-oncologist. I also treat a small number of patients with soft tissue sarcomas, but have not personally treated a case of hemangiopericytoma. My comments are based on general principles of cancer treatment, and on published details specific to hemangiopericytoma.

I have reviewed the file provided by Laura Kell with 320 pages of the Appellant’s file from British Columbia and Nova Scotia, and an opinion from Dr. David A. Ramsay, a neuro-oncologist. He reviewed the pathologic findings and their clinical relevance. I have done a literature review of recent publications on hemangiopericytoma. Dr. Ramsay was asked to address questions specific to the standard of care beginning in 1999.

To summarize the clinical history briefly, the Appellant presented in 1998, with headache suggestive of sinusitis fluctuating over several months. Eventually, imaging showed a tumor in the occipital para-saggital region. He had a gross total resection in 1999, and adjuvant radiation therapy a few weeks later. The pathologic diagnosis was atypical meningioma, as detailed in the Dr. Ramsay’s report. He had close follow-up with clinical exams and imaging of the head early on, but less frequently as the years passed. He moved from British Columbia to Nova Scotia, and continued appropriate follow-up with a neuro-oncologist in Halifax.

In August 2010 he presented with abdominal pain, and CT scan showed widespread metastatic cancer in the abdomen. A biopsy suggested sarcoma. His prior brain tissue was compared to the new biopsy, and at that time it was realized that this was the same disease, hemangiopericytoma, rather than atypical meningioma. He was treated with at least one of the usual drugs for metastatic sarcoma, but the disease did not respond, and he died from the cancer metastases.

Any death from cancer in a young man is distressing for everyone involved, and learning that the original diagnosis was incorrect undoubtedly worsens that distress. The obvious question is whether the outcome would have been better with the correct diagnosis from the outset. The question of whether his work exposure with military weapons handling was the cause of this disease was discussed by a number of consultants, but no definitive answer is possible.

Headache is a common condition, with many possible causes, most of which are not life threatening. Early identification of the occasional patient who has a brain tumor can be very difficult. The notes I reviewed did not suggest any strong warning signs that would have lead the average family doctor to order a CT scan or specialist referral any earlier. Whether surgery 4-5 months earlier would have prevented metastatic disease that eventually took his life is uncertain. Many tumors start to spread before the patient has any symptoms.

The clinical behavior of atypical meningioma differs from hemangiopericytoma. Hemangiopericytoma makes up only 0.4% of intracranial tumors. It is a type of sarcoma, and occasionally spreads outside the head. Meningiomas rarely metastasize. However, for both diseases, the main treatment remains optimal local control of the primary site of disease. Patients who are able to have a complete surgical resection have a much better survival. Post-operative radiation therapy to the surrounding tissues is widely recommended for both diseases, in the hope of eliminating any residual cancer cells. However, the best review I have located (Melone, et al, World Neurosurgery 2014:556-562) found that radiotherapy did not appear to improve the long-term survival. They reported on 43 patients, 5 of whom relapsed outside the brain. The average time to recurrence was 72 months. At ten years, 72% were still alive, but only 29% of the 43 patients were free of relapse. For many cancers of other parts of the body, giving chemotherapy drugs after surgery has been shown to improve survival. For other types of sarcomas this has been studied extensively, but the results have been disappointing. Hemangiopericytoma is so rare that research on new treatments is limited to reports on small numbers of patients, and there is no recommendation for adjuvant chemotherapy, even in 2014.

Some patients with recurrence of other subtypes of soft tissue sarcomas have been candidates for surgical resection, if the disease is localized and slow growing. However, few patients would be cured by that approach, and to my knowledge there is no published guideline or consensus of experts to recommend that patients like the Appellant should have frequent scans of the whole body over many years. In fact, across all types of cancer there are only a few situations where aggressive follow-up tests in asymptomatic patients can be shown to improve cure rates. The general principal is to give the best treatment up front, and the details of follow-up are less important.

I note a letter in the DVA file from Dr. Bruce Colwell, dated January 29, 2013, and my comments generally agree with his. In summary, it is unfortunate that the Appellant was given the wrong histologic diagnosis at the outset, but the treatment he received would have been the same had hemangiopericytoma been recognized. It is quite likely that physicians following his case would have been more vigilant looking for spread elsewhere in the body, had they known the diagnosis, but given the limitations of available salvage therapies, it is unlikely that such monitoring would have lead to a significantly better outcome.