Lung cancer is a dreadful disease, and even with some recent improvements in treatment the prognosis is dismal. In the United States lung cancer is 15% of cancer diagnoses, but 29% of cancer deaths. In the early 20th century it was a rare disease, but it increased across the century and doubled between 1950 and 1970. With the reduction in smoking the incidence in men is now falling, but it continues to rise in women. In some countries lung cancer has overtaken breast cancer as the major cancer cause of death in women.
Lung cancer is not a single disease: as with other tumour sites, there is a complex array that can afflict the lung. Three main types amount to around 85% of cases. Small cell carcinoma (also called poorly differentiated neuroendocrine carcinoma); squamous cell carcinoma, and adenocarcinoma. The latter two have until recently been grouped together as non-small cell carcinoma, recent advances in treatment of pulmonary adenocarcinoma mean that these two tumour types are less commonly grouped together. Small cell carcinoma is almost always metastatic at first diagnosis, but has a high initial response to chemotherapy. Squamous cell carcinoma and adenocarcinoma are less often metastatic, but are less responsive to treatment.
Survival statistics for lung cancer are widely published in medical texts and on the internet. Standard British medical texts are the most gloomy, those from the US are more optimistic. Public facing websites of cancer charities tend to be the most optimistic. In the US 1 year survival was reported to have improved from 34% in 1975, to 41% in 2007, as a result of better surgery. The 5 year survival of all stages was reported to be 16% in the US. In the UK it is reported in a standard undergraduate level text published in 2010 that around 70% of patients die within 1 year of diagnosis, and only around 6-8% survive 5 years. The best prognosis is achieved in localised squamous cell carcinoma that has not spread, and is anatomically suitable for surgery (for example it must not involve the major vessels, or oesophagus or trachea, and the patient must be fit for surgery, with adequate heart and lung function).
All malignant tumours are staged at diagnosis. The stage is a representation of the extent of the tumour. Precise stage definitions are published by the International Union against Cancer, and are used worldwide, including in the UK. The smallest and most localised tumours are stage 1. The most advanced, with distant spread, are stage 4.
Average survival of untreated stage 4 small cell carcinoma is 2-4 months, survival of untreated non-small cell carcinoma averages 7 months.
The causation of lung cancer has been studied very extensively for more than 60 years. Smoking is the major but not sole cause. Adenocarcinomas of lung are more often seen in non-smokers than the other 2 types, which are particularly associated with smoking. Lung cancer is not a psychosomatic condition; it has physical causes related to mutations in genes that control cell proliferation and cell death. Lung cancer does not develop in someone because they have been “relieved of a necessity to maintain a front and hold things together”. Lung cancer is not a somatic manifestation of grief. It is interesting to note that in the 19th century there were psychological theories concerning the development of tuberculosis. These largely vanished when in 1882 Koch discovered the tubercle bacillus and identified the infectious nature of the disease. Thomas Mann’s The Magic Mountain, from which Jim Perrin quotes, is an interesting if overlong book, which has much to say about the human condition, but with respect to causation of disease is entirely without relevance. Lung cancer is not caused by “iatrogenic megalomania”, it has an objective reality and it is not a subconscious creation. It does not go away if ignored.
Diagnosis and treatment of cancer in the UK is carried out by multidisciplinary teams. Such a team for lung cancer will include chest physicians, oncologists, radiologists, histopathologists and specialist nurses. Diagnosis takes into account clinical presentation, x-ray appearances, bronchoscopic features, and the results of biopsies. In around 70% of cases a biopsy is available to support the diagnosis, and provide tissue for molecular evaluation where indicated (particularly in adenocarcinomas to look for evidence of BRAF oncogene mutation). Ordinarily the patient will be told the diagnosis by a member of the team who is aware of the whole diagnostic picture, and most important, knows what treatment, if any, is available. Significant numbers of patients are beyond all treatment other than palliation and symptom relief at first diagnosis. A very large lung tumour can grow without symptoms unless it erodes or occludes an airway, and the central origin of most, close to the great blood vessels, means that relatively few are suitable for surgery. Surgery is generally not used for small cell carcinoma.
In West Perrin states that he has “upper lobe carcinoma with metastases in both lungs”. This is stage 4 cancer and it would carry the likely prognosis given above, a matter of a few months untreated. Perrin describes being given the news by a female GP with “extravagant cleavage”. This would be most unusual, given that generally GPs are not part of the team. A wise old general physician would be a “hospital boy”, and would be unlikely to suggest “those boys at the hospital are a bit knife happy”. The tumour Perrin describes himself as having would be very unlikely to be treated by surgery, and more likely to be offered palliation only.
The wise physician may well have said (if Perrin had had a consultation) “I wouldn’t have thought much harm would be done if we just wait and see for a while”, as such an advanced tumour would most probably kill the patient with or without treatment. If Perrin with tumour had gone to the Pyrenees, his tumour would have continued to develop.
What then of the possibility of medical misdiagnosis? All human judgements may be wrong, and medical judgement is no less fallible than any other. 70% of lung cancer diagnoses are supported by a biopsy. The other 30% in which the diagnosis is radiological, and biopsy is not possible (often for reasons of tumour site) are more likely to be erroneous. In the investigation of patients with possible cancer, X-rays may disclose lesions in the lung which cannot be characterized as either benign or malignant. A common strategy is to wait 3 months and repeat the X-ray. If the lesion has not progressed it is not cancer. There are many causes of shadows on lung X-rays that are not cancer, and Perrin may have such a lesion, but if he did not have cancer the misdiagnosis would be clear within less than a year, and given the timescale of writing and publishing West it would be possible for the misdiagnosis to be reported.
Perrin states that “all human testimony is unreliable” and that the “actual becomes subservient to the story”. He also says that “illness at a level deeply subconscious was of my own creation”. I find the account of the diagnosis of Perrin’s cancer to be very unlikely, and the lack of progression of a tumour is strong evidence that he has not, and has never had lung cancer. In the quotations earlier in this paragraph I think that Perrin is indicating that the story of his cancer is fictionalised or fictional. This then raises questions about the rest of the book. How much is true, and how much is fictionalised or fictional? If I can’t believe the few pages I have considered here, what am I to make of the rest of the book?
All the information here is easily available to anyone with an internet connection. The pages of West examined include 300 and 301, and pages 149 and 150. Books to look at include Robbins and Cotran Pathologic Basis of Disease, and Davidson’s Principles and Practice of Medicine (there is a newly published 2014 edition which I have not yet seen). The Cancer Research UK website carries much information; there are many other good websites easily found with the search engine of your choice. It is therefore open to anyone to read the evidence and come to their own conclusion.
Jac’s sisters wish to repeat their thanks to the consultant who so generously compiled and contributed these notes.