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J. White



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    ED 12 - Caring for the Lung Cancer Patient (ID 12)

    • Event: WCLC 2015
    • Type: Education Session
    • Track: Nursing and Allied Professionals
    • Presentations: 1
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      ED12.01 - Management of Paraneoplastic Syndrome (ID 1818)

      14:15 - 15:45  |  Author(s): J. White

      • Abstract
      • Presentation

      Abstract:
      Paraneoplastic Syndromes (PNS) are a group of clinical disorders that are associated with malignant diseases that are not directly related to the physical effects of the primary tumour (Spiro 2007). The mechanisms by which PNS occur is not fully understood (Dalmau 2014) but is related to the production of polypeptide hormones or cytokines by the cancer itself or an immune response to the cancer. PNS may occur in 10% of patients with lung cancer and with the type of cancer influencing the nature of syndrome (Kanaji 2014). A wide variety of PNS have been associated with lung cancer with the most common syndromes being endocrine and neurological syndromes. This paper will only address these two groups of syndromes. Paraneoplastic Endocrine Syndromes include: Inappropriate Antidiuretic Hormone Secretion: hyponatremia is seen in 30-70% of patients with lung cancer with only 1-5% having symptoms and mostly associated with small cell lung cancer (SCLC); symptoms include confusion, seizure, reduced consciousness and coma (Spiro 2007). Hypercalcaemia: the incidence ranges from 2-12% in lung cancer; symptoms include nausea, vomiting, abdominal pain, constipation, polyuria, thirst, dehydration, confusion and irritability (Spiro 2007). Cushing Syndrome: production of adrenocorticotropic hormone(ACTH) is the most common explanation with approximately 50% being neuroendocrine lung cancers with 36%-46% carcinoid tumours and 8%-20% SCLC of cases; symptoms include weakness, muscle wasting, drowsiness, confusion, psychosis, oedema, hypokalaemia alkalosis and hyperglycaemia. hypertrophic osteoarthropathy (HOA) and finger clubbing: 90% cases in lung cancer Prevalence of 5-15% in lung cancer most common with squamous cell and adenocarcinoma. occurs due to proliferation of connective tissue beneath the nail bed with HOA causing distal expansion of the long bones; The Paraneoplastic Neurological Syndromes most commonly displayed are peripheral neuropathies and include Lambert-Eaton Myasthenic Syndrome, necrotizing myelopathy, cerebral encephalopathy, visual loss and visceral neuropathy. Symptoms include muscle weakness, cognitive and personality changes, ataxia, cranial nerve deficits or numbness. Classical and Non-Classical Symptoms have been defined (Graus 2004 Table 1) to aid in diagnosis. These syndromes are often associated with the presence of anti-Hu antibodies which are produced together with antigen-specific T lymphocytes and attack parts of the nervous system (Pelosof 2010). This syndrome is detected in 80% of cases before cancer is diagnosed (Honnorat 2007) and upto 20 per cent of those with SCLC have detectable antibodies although this syndrome will not necessarily develop (Darnell 2003). There is a reported 4-5% incidence in SCLC. Diagnostic Pathway Patients with lung cancer usually present with multiple symptoms with a time delay between symptom recognition and the ultimate diagnosis of lung cancer. The presence of a PNS may not necessarily preclude treatment with a curative intent which may help in the improvement of symptoms. Pathways of care commence with clinical evaluation, CT scan, blood screen and rapid evaluation for the next diagnostic test. It is at this point where a PNS may become apparent and can aid in the diagnosis of lung cancer. Where there are concerning symptoms for Paraneoplastic Neurological Syndrome and there is difficulty in identifying the underlying cancer: antibody screening can be considered but require considered interpretation as patients without cancer can harbour paraneoplastic antibodies, variation in the consistency of the presence of antibodies can be associated with different syndromes and the absence of antibodies may not exclude a syndrome; PET may identify neurological syndrome activity; electrophysiology may confirm the syndrome but are not always associated with cancer; MRI can assist in the diagnosis of limbic encephalitis as atrophy can be detected. Treatment As symptoms often present prior to the confirmed diagnosis it is important to treat the signs and symptoms of: Hypercalcaemia with: an increase in fluid intake; Bisphosphonates. Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) with: fluid restriction; chemotherapy (effective in 80% of SCLC cases); Demeclocycline; Vasopressin receptor antagonists (Conivaptan, Tolvaptan). direct inhibition of cortisol secretion to reduce circulating glucocorticoids (eg Ketoconazole); Octreotide (blocking the release of ACTH); bilateral adrenalectomy. Cushing syndrome by: Once the diagnosis of lung cancer has been established the optimal treatment for any PNS is to treat the disease. There is no randomised control trial data for treatment for PNS on which to base practice (Giometto 2012) but there is evidence that the anti-cancer treatments (surgery, radiotherapy, chemotherapy, biological therapies, immunotherapies, immunomodulation and immunosuppression including glucocorticoids) may be beneficial. One of the challenges is that irreversible neuronal damage may have already happened by the time the diagnosis has been established. Supportive Care The symptoms of PNS can have a major impact on the patient’s quality of life and on individual care requirements. It is recommended that a Health Needs Assessment (DoH 2011) is undertaken when symptoms first present, prior to starting any intervention and at the end of treatment. This assessment should take into account the patient’s physical, psychological, social, spiritual and practical needs and will enable interventions to be put in place to maximise the quality of daily life. When symptoms present the psychological and physical impact can be devastating as immobility, discomfort, cognitive dysfunction and loss of the ability to self-care can affect the patient and care providers. In the UK the role of the Lung Cancer Nurse Specialist (RCLCF 2014) is ideally placed to address these concerns not only when the diagnosis is first made but throughout treatment and beyond by providing psychological support and symptom management, home care coordination and referrals to other health professional such as Physiotherapists and Occupational Therapists. Once primary treatment has taken place the ongoing monitoring of response to treatment is required together with a comparison of presenting symptoms. Patients should know what to do if these reoccur and how they should access their clinical team. Conclusion The management of PNS is complex and in the absence of randomised trials there is available guidance to help with the management of Paraneoplastic Syndromes. The individual care of each patient should be tailored to the diagnosis, symptoms and holistic needs. References Dalmau J, Rosenfeld M (2014) Overview of paraneoplastic syndromes of the nervous system (Accessed 20[th] June 2015) http://www.uptodate.com/contents/overview-of-paraneoplastic-syndromes-of-the-nervous-system#subscribeMessage Darnell R, Posner J (2003) Paraneoplastic syndromes involving the nervous system. NEJM 349(16):1543-54. Department of Health (2011) Improving Outcomes: A strategy for Cancer. DoH Giometto B, Vitaliani R, Lindeck-Pozza E et al (2012) Treatment for Paraneoplastic Neuropathies. Cochrane Library (Assessed 30[th] June 2015) http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD007625.pub2/abstract Graus F, Delattre J, Antoine J et al (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. Journal of Neurology, Neurosurgery and Psychiatry 75(8):1135-40 Honnorat J, Antoine, JC (2007) Paraneoplastic neurological syndromes Orphanet Journal of Rare Diseases 2.22 Kanaji N, Watanabe N, Kita N et al (2014) Paraneoplastic syndromes associated with lung cancer. World Journal of Clinical Oncology, 5(3), 197-223 Pelosof L, Gerber D (2010) Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clinic Proceedings 85:838–854. Roy Castle Lung Cancer Foundation (2013) Understanding the Value of Lung Cancer Nurse Specialists. RCLCF Spiro S, Gould M, Colice G (2007) Initial evaluation of the patient with lung cancer: symptoms, signs, laboratory tests, and paraneoplastic syndromes: ACCP evidenced-based clinical practice guidelines (2nd edition) Chest. 132:149S–160S. Figure 1



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