CASE REPORT

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AN 80-YEAR-OLD WOMAN WITH LEFT SHOULDER PAIN

1. Difficulties in diagnosing Pancoast tumour

Non-typical presentation of Pancoast tumour

Pancoast tumour is a malignant tumour of the superior sulcus of the lung. Although this is a lung tumour, pulmonary symptoms of cough, dyspnoea and haemoptysis are rare.Very often by the time it is discovered, it has infiltrated to the surrounding brachial plexus and cervical sympathetic trunk.1 Pancoast syndrome is the term given to a combination of constant and characteristic clinical presentations of pancoast tumour.1,2

  1. Severe pain localised in the shoulder (90% of cases) - Pain may radiate towards the axilla and scapula and ulnar aspect of the upper limb along C8, T1 dermatomal distribution with or without atrophy of the arm and hand muscles.
  2. Horner's syndrome (62% of cases) - Involvement of sympathetic chain results in ptosis, miosis, hemianhydrosis and enopthalmus.
  3. Invasion or destruction of ribs and vertebrae.
  4. Superior vena cava syndrome (not a typical presentation of Pancoast syndrome).

In the early stages, Pancoast tumors are difficult to detect on chest X-ray films because the top of the lung is located in an area of the body that is difficult to visualize clearly on an X-ray film.Many patients are initially treated for presumed local musculoskeletal problems of bursitis and vertebral osteoarthritis with radicular pain.1,2 In 1998 series by Maggi and colleagues, symptoms of Pancoast tumour was shown to last for 2-36 months with a mean duration of 9.7 months.3

Healthy aging process/missing cues/prematured convergence of hypothesis of doctors/compartmental medicine/ “doctor hopping and shopping” of patient.

Normal aging refers to the common complex of diseases and impairments that characterise many of the elderly. In successful healthy aging deleterious effects are minimized, functions are preserved, and aging is not necessarily accompanied by debilitating disease and disability.4

Chronological age refers to the number of years a person has lived. Functional age refers to a person’s ability to contribute to society and benefit others and himself. Many people may be chronologically old but remain physically fit, mentally active and productive members. Comprehensive health assessment, the cornerstone of quality care for the elderly, is a multidimensional assessment that incorporates physical, psychological and social function as well as medical health.5 There was no justification to assume that because YN was already 80 years old, there was nothing more to be done for her “rheumatic pain”.

Presence of a mass in the apex of the left lung was obvious on the X-ray film done in August 2008 but was not picked up by the radiologist. As neuropathic pain was not identified, doctors focused their attention on nociceptive pain due to arthritis or possible bone fracture even though there was no history of a fall. The radiologist confined his attention to looking for fractures on the X-ray film as ordered by the physician, so much so that he missed out the big mass at the apex of the left lung. For the same reason, the endocrinologist and ophthalmologist missed out the presence of neuropathic pain and Horner’s syndrome because they focused only on diabetes mellitus and diabetic retinopathy. Premature convergence of hypothesis led to failure to ‘search for further hypothesis, missing other more important facets of the whole’.6 To avoid making this mistake doctors should try to include all appropriate diagnostic hypotheses based on probability, seriousness, ‘pay off’ and treatability.7

Presence of Horner’s syndrome associated with radicular pain should have led to the suspicion of an infiltrative lesion at the thoracic outlet involving the brachial plexus and extending to the sympathetic chain. Medical sub-specialization has led to practice of “compartmental medicine” where each specialist looks after a particular organ system. The many doctors and specialists consulted by YN failed to relate the eye problem to the shoulder pain, combination of which gave a strong clue to the diagnosis of Pancoast syndrome.

“Doctor hopping and shopping” by patient is common practice in our Malaysian healthcare system. No one particular doctor was responsible or committed to looking after YN’s shoulder pain. Michael Balint in his concept of “collusion of anonymity” emphasized that “if a patient is seen by a variety of physicians, not one of them is willing to be responsible or accept the total management of the patient’s problems”.8

2. Pain management

Pain assessment

Pain is one of the more easily controlled symptoms in palliative medicine. Cancer pain can be effectively treated in 85% to 95% of patients with an integrated program of systemic, pharmacologic and anticancer therapy.9

Acute pain is of sudden onset, has a clear cause, lasts for hours to days, and disappears once the underlying cause is treated. It is beneficial to the patient as it signals that there is something wrong and motivates the person to get help. Chronic pain usually starts as an acute pain and continues beyond the normal time expected for resolution of the problem or recurs for various other reasons. It is not therapeutically beneficial to the patient. In acute pain, attention is focused on treatment of the cause of pain whereas in chronic pain, the emphasis is laid upon reducing the pain to give relief, limit disability and improve function.10

Nociceptive pain arises from damage to tissues other than nerve fibers. It usually presents as well localized, constant, dull nagging pain or throbbing and toothache-like pain if it involves the bones. Cutaneous nociceptive somatic pain results from injury to the skin, while deep nociceptive somatic pain is from musculoskeletal tissues.10

Nocieptive visceral pain results from injury to the internal organs such as the heart, lungs, brain, stomach, intestines, liver and urinary bladder. It usually presents as diffuse, non-localized, constant, dull, aching pain or as colicky pain in hollow viscus for example abdominal and ureteric colic.10

Neuropathic pain results from damage to the nervous tissue. It can be either central when the brain and spinal cord are injured or peripheral when the peripheral nervous system is injured. Neuropathic pain is described as severe, sharp, lancinating, lightning-like, stabbing, burning, cutting, prickling, or abnormal sensations of coldness, numbness, tingling or weakness.11

Inadequate pain assessment

Initially YN’s pain was non specific and was confined to the shoulder, justifying the impression that it was shoulder arthritis or suspected fracture. Pancoast tumours are uncommon, comprises of only 5% of lung tumours2 so it would not be the first diagnosis to come to the mind. However when localized pain progressed to “lancinating, cutting, lightning-type” of pain extending down the medial ulnar aspect of the left arm and hand, one had to be on the alert that there was presence of neuropathic pain corresponding to dermatomal distribution of C8 and T1. This type of radicular pain is often caused by nerve root compression either in the cervical spine or on the brachial plexus. Lack of recognition of neuropathic pain further delayed the diagnosis of an infiltrative lesion in the left upper thoracic outlet.

Pain control

Neuropathic pain is widely recognized as one of the most difficult pain syndromes to treat. It presents a significant challenge to clinicians as it often does not respond to conventional analgesic therapies.12 Adjuvant analgesics’ are non-conventional painkillers with a primary indication other than pain, but with analgesic properties in some painful conditions. They include anti-epileptics (carbamazepine and gabapentin), antidepressants (particularly amitriptyline) and antiarrythmic drugs (mexiletene). They can be effective in controlling neuropathic pain.13,14 Although they can be used alone, they are usually co-administered with analgesics (acetaminophen, non-steroidal anti-inflammatory drugs, opioids) when treating cancer pain.15

YN’s pain was not diagnosed as nociceptive visceral pain of the left shoulder and peripheral neuropathic pain (brachial plexopathy) down the left arm for nearly a year. None of the doctors that YN had consulted prescribed adjuvant analgesics (anticonvulsant or antidepressant) for her neuropathic pain. Either her pain was not recognised as neuropathic pain or it was not known that adjuvant analgesics were helpful in relieving neuropathic pain.

3. Ethical issues

Biopsy procedures

Majority of cases of Pancoast syndrome are non–small cell lung carcinoma (NSCLC), with more than 95% located in the superior sulcus. Most of it lies outside the lung and involves the chest wall, ribs, nerve roots, lower trunks of the brachial plexus and sympathetic chain.1 Common varieties include squamous cell carcinoma (about one third of cases), adenocarcinoma (about two third of cases) and large cell carcinoma (<10%). Small cell carcinoma is a rare cause of Pancoast syndrome (<5%).2

Non-malignant causes include lymphoma, plasma cell granuloma, infections, vascular aneurysms and cervical rib syndrome. Because of the wide variety of diseases that can produce Pancoast syndrome, a histologic diagnosis is mandatory before initiating definitive treatment. Imaging and biopsy are the cornerstones of evaluation of Pancoast tumour.2

YN’s family was advised not to subject YN to further biopsy but to just refer her to a private oncologist for low dose palliative radiotherapy merely for pain control. On one hand, we could argue that it was justified not to subject patient to an invasive test for in view of her age, she would not be a likely candidate for aggressive curative treatment. Low dose radiotherapy shrinks the tumour mass and relieves the compression on the nerve mainly for pain relief.

On the other hand, without a biopsy report, was it justified to subject YN to radiotherapy? What if it was not a malignant growth? In view of the “slow growth” of the tumour, a HPE result may indicate a well differentiated radiosensitive tumour. If so, will curative doses of DXT be able to prolong survival for YN? The infiltrative nature and damaging effects of the tumour on surrounding structures suggested high likelihood of malignancy. Nevertheless, two attempts of lung biopsy were carried out but were not successful in detecting malignant cells.

Palliative versus curative DXT

In the past, superior sulcus tumours were considered inoperable and incurable because of their relative inaccessibility and extensive local invasion of the thoracic inlet. Recent clinical studies showed preoperative irradiation decreases local recurrences, prevents the growth of disseminated tumour cells, and increases survival compared with irradiation or surgery alone. The five-year survival rate after surgery is approximately 30%.16 In view of YN’s age and extensive tumour infiltration, it was unlikely that surgery would be recommended.

The aim of palliative radiotherapy is to provide symptomatic relief and improve quality of life with minimal side effects. Radiation in doses of 40-60 Gy administered over a period of three weeks, elicits pain relief in 90% of patients.2 In some cases, the intention of treatment between curative and palliative are not clearly distinguished. There is variation of practice among clinical oncologists.17

Without a biopsy report, the intention of delivering ten fractions of low dose radiotherapy with minimal side effects to YN merely for pain control was justified. It was later increased to 25 fractions when there was substantial reduction in the size of the tumour after delivery of the first few fractions.

Limited treatment plan

Limited treatment plan is a decision not to initiate treatment or medical interventions for the patient if benefits are outweighed by burdens of those procedures. When death is inevitable and cannot be prevented by available treatment it is morally permissible to withhold treatment. But limited treatment plan does not equate to no treatment or no care. Medical professionals are obliged to care for patients, offering palliation as well as emotional support.18

With this incurable, life threatening and late stage disease, it was no longer relevant to try to treat YN’s hypertension and diabetes mellitus to the ideal level to prevent future complications. As long as her blood pressure and blood sugar were not dangerously high, palliation of pain and other symptoms, good nursing care and patient comfort had to be given priority.

Conspiracy of silence

Conspiracy of silence is generally an act of love or a need to protect another from pain. Family members often argue they know the patient better than healthcare professionals. They may argue that telling the truth may take away hopes.19 However, hiding the truth from YN did not allow her the autonomy to make decisions with regard to specific cancer treatment plan. Withholding the truth prohibits patient and carers from sorting out practical issues, denies them opportunities to reorganize and adapt their lives towards attainment of more achievable goals.20 The best coping strategy would be confrontation of the real situation, open discussions and a good network support for the patient.

4. Palliative care

Delirium

It was thought that YN’s sudden change of behaviour was due to hypoglycemia, stress of biopsy procedures or brain metastases. In view of a RBS result of 3.5 mmol/L, a normal head CT scan report, an interval of five days between last biopsy procedure and onset of altered behaviour, it was unlikely these were specific causes. More important factors contributing to her delirium were morphine intake for the first time, several months of uncontrolled pain, worries and anxiety. The stress of going in and out of hospitals with their peculiar strange, unfamiliar faces and surroundings were enough to topple any elderly person into a delirium. Identifying the factors contributing to YN’s delirium and rectifying them would be a more useful approach.

Acute hospital care versus home palliative care

Acute hospital care management with forced artificial Ryle’s tube feeding, intravenous infusion, in-situ catheterization, were causing tremendous sufferings to YN. These procedures could cause more harm than good. Intravenous infusion can easily lead to fluid overloading and acute pulmonary oedema; catheterization predisposed immunologically incompetent patients to urinary tract infections and septicaemia. Ryle’s tube feeding may predispose patient to aspiration pneumonia, may create a barrier to communication, breathing and talking, not to mention the severe discomfort it incurs in a conscious patient. Confinement to bed weakens the already weak muscles of the elderly and predisposes them to bedsores, pneumonia, sepsis and deep vein thrombosis. Confusion would be even worse! These factors provided explanations as to why YN’s delirium did not clear for three weeks in the hospital but she recovered completely once she came home.

The bedrock of palliative care philosophy is that of patient centred holistic care focusing on quality of life and extending support to family and carers. This is encompassed in the following quotation by Dames Cicely Saunders, “You matter because you are you, and you matter until the last moment of your life. We will do all that we can to help you not only to die peacefully, but to live until you die”.21

A study by J Townsend et al. in assessing the preference of terminally ill patients with cancer for their place of final care, showed that majority of them preferred to die in their own home.22 “Most people if given a choice and a supportive home, would choose to die at home and the care of the dying is a traditional part of good general practice. It is a different and rather special form of medical practice and it can be both very demanding and rewarding” (Denis Pereira Gray, President of RCGP).23 In Malaysia, where palliative care is not well established, preference to die at home may not be an option for many patients even if they wish to do so.

Definition of a good death

YN never recovered from her inability to walk till the end. For the elderly, dependency on others and loss of dignity is more feared than death itself. A study conducted by M Miyashita et al. on good-death concept for Japanese cancer care concluded that the main domains on top of the list to define a “good death” are: physical and psychological comfort, dying in a favourite place, good relationship with family and medical staff, maintaining hope and pleasure, not being a burden to others, physical and cognitive control, environmental comfort, being respected as an individual and life completion.24

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