Neglected conditions

The day after the third annual Worldwide NET (neuroendocrine tumour) Cancer Awareness Day, Kelsall1 pointed out the multiplication of disease recognition days in a CMAJ editorial and called for “articles on neglected conditions that may benefit from increased attention.”

Despite the recent rise in incidence of this rare malignancy, patients with neuroendocrine tumours experience diagnostic delays of up to seven years after presentation of initial symptoms. This delay may be due to rarity, non-specific symptoms, unknown risk factors or a lack of awareness among primary care and specialized physicians.2^,3 In Ontario, patients with neuroendocrine tumours visited primary care physicians a median of 14 times before receiving a diagnosis.3^,4 The combination of slow progression and hormonal production in advanced neuroendocrine tumours produces debilitating symptoms, which lead to substantial deterioration of quality of life.

Neuroendocrine tumours represent a rare “chronic cancer” that forces patients to cope with steady debilitation. Beyond fragmented care, patient support is compromised because little information on prognosis and treatment is available to physicians. Large-scale analyses of epidemiology, behaviour, and health care delivery and utilization surrounding neuroendocrine tumours are paramount to patients and physicians in terms of timely diagnosis and delivery of effective therapies.

Neuroendocrine tumours must cease to be a diagnosis of exclusion that is established after years of investigation. We strongly believe that research is desperately needed to create targeted programs to raise awareness in the medical community and to improve outcomes and physician and patient experience. A comprehensive review of the current knowledge surrounding neuroendocrine tumours is the first step. It is often said that when hearing hoofbeats, one should think horses, not zebras ... but sometimes one is not far from the Serengeti and has to know about zebras.