Paraneoplastic Syndrome
Original Editors -Brittany Chorley &Seth Chorleyfrom Bellarmine University's Pathophysiology of Complex Patient Problems project.
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Introduction[edit | edit source]
Paraneoplastic syndromes (PNS) occur due to the systemic effect of a malignancy and occur remotely to the primary malignancy. Symptoms are brought about by cytokines, hormones or immune cross-reactivity. These syndromes can cause a manifold of symptoms and can affect numerous systems.[1] PNS can occur concurrently with tumour diagnosis before a tumour is diagnosed and even after tumours have been resected[2]. The symptoms typically present in the middle-aged to older population. Also, it is common in individuals with lung, ovarian, lymphatic, or breast cancer. The most common cancer associated with paraneoplastic syndrome is small-cell cancer of the lungs. [3]
There is no cure for paraneoplastic syndromes and the treatment does not stop neurological damage.[4]
Aetiology[edit | edit source]
PNS are largely due to two main causes:
- Those due to tumour secretions of hormones, functionally active peptides, enzymes cytokines
- Those due to tumours operating through auto-immune/immunological mechanisms with cross-reacting antibodies between neoplastic and normal tissues. Nb Remission of symptoms often follows resection of humoral secretory tumours but not always of tumours due to immunological mechanisms[2].
Types of Paraneoplastic Syndrome[edit | edit source]
The Paraneoplastic Syndromes are classified based on the organ systems involved or on the etiological groups and are as follows[5] :
- Lambert-Eaton Myasthenic Syndrome
- Stiff-Person Syndrome
- Encephalomyelitis
- Myasthenia Gravis
- Cerebellar Degeneration
- Limbic or Brainstem Encephalitis
- Neuromyotonia
- Opsoclonus
- Sensory Neuropathy
Epidemiology[edit | edit source]
PNS occur in up to 15% of patients with cancer.[1] Neurological manifestation in the form of neuropathies is common. Males and females are affected equally.[6]
Clinical Presentation[edit | edit source]
PNS can affect multiple systems and have various presentations. Symptoms usually start before a tumour is found, slowly developing over a few days or weeks. Some of the more common symptoms include - Difficulty walking or swallowing; Loss of muscle tone; Loss of fine motor coordination; Slurred speech; Memory loss; Vision problems; Sleep disturbances; Dementia; Seizures; Sensory loss in the limbs; Vertigo or dizziness.[4]
Examples are given below based on the organ system involved.
- Paraneoplastic neurological syndromes: encephalitis (paraneoplastic rhombencephalitis, limbic encephalitis); Lambert-Eaton myasthenic syndrome; Optic neuropathy; Cerebellar degeneration.
- Endocrine eg Cushing syndrome; Syndrome of inappropriate antidiuretic hormone secretion (SIADH); Hypercalcemia
- Rheumatological eg Paraneoplastic polyarthritis; Polymyalgia rheumatica; Multicentric reticulohistiocytosis; Hypertrophic osteoarthropathy
- Paraneoplastic haematologic syndromes: Good syndrome; Polycythaemia[1]
- Dermatological eg Acanthosis nigricans; Paraneoplastic pemphigus; Sweet syndrome; Leukocytoclastic vasculitis; Dermatomyositis
- Renal eg Electrolyte imbalance (hypokalemia, hypo or hypernatremia, hyperphosphatemia) causing nephropathy and acid-base disturbance due to ectopic hormones produced by tumour cells such as ACTH and ADH. Nephrotic syndrome can also be one of the manifestations of paraneoplastic syndrome.
- Miscellaneous eg Fever, cachexia, anorexia, dysgeusia[6]
Management[edit | edit source]
Management of the patients is based on the type, severity, and location of the paraneoplastic syndrome. First, therapeutic options are to treat underlying malignancy with chemotherapy, radiation, or surgery.
- Other therapeutic options are immunosuppression with corticosteroids or other immunosuppressive drugs, intravenous immunoglobulins, plasma exchange, or plasmapheresis.
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
First, a healthcare provider will perform a clinical exam that would include a general physical and neurological screening.
- Neurological screening: reflexes, sensation, myotomes, balance, and coordination.
- Laboratory tests that could be utilized to diagnose paraneoplastic syndrome include: Blood tests, Spinal tap
- Imaging tests that could be utilized to diagnose paraneoplastic syndrome include: CT Scan; MRI; PET Scan; and PET-CT, which may enhance the detection rate of small cancers
If the physicians cannot find a malignant tumour, the syndrome may be the cause of a tumour that is too small to locate. In this instance, the physician will continue to have follow-up imaging conducted every three to six months for several years unless the cause is identified. [7]
Physical Therapy Management[edit | edit source]
People with paraneoplastic syndrome can have difficulty with walking, balance, coordination, muscle tone, sensory of where the body is in space, and vertigo.
- All of these symptoms the physical therapist can treat with traditional therapy.
- Precautions must be taken into account for the cancer or neoplasm that is involved.[3]
Conclusion[edit | edit source]
The diagnosis and management of paraneoplastic syndromes is difficult.
- In most cases, there is an underlying malignancy responsible.
- Due to the numerous causes, the condition is best managed by an interprofessional team (including a pathologist, oncologist, radiologist, haematologist, nurse specialist, and internist).
- Once the cause is discovered, it needs to be treated.
The management of the patients is based on the type, severity, and location of the paraneoplastic syndrome.
- First, therapeutic options are to treat underlying malignancy with chemotherapy, radiation, or surgery.
- Other therapeutic options are immunosuppression with corticosteroids or other immunosuppressive drugs, intravenous immunoglobulins, plasma exchange, or plasmapheresis.[6]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 Radiopedia Paraneoplastic syndromes Available: https://radiopaedia.org/articles/paraneoplastic-syndromes(accessed 24.1.2023)
- ↑ 2.0 2.1 Henry K. Paraneoplastic syndromes: definitions, classification, pathophysiology and principles of treatment. InSeminars in diagnostic pathology 2019 Jul 1 (Vol. 36, No. 4, pp. 204-210). WB Saunders.Available from:https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 (last accessed 15.7.2020)
- ↑ 3.0 3.1 National Institute of Neurological Disorders and Stroke. NINDS Paraneoplastic Syndromes Information Page. http://www.ninds.nih.gov/disorders/paraneoplastic/paraneoplastic.htm. (accessed 18 March 2013).
- ↑ 4.0 4.1 NIH Paraneoplastic Syndromes Available:https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes (accessed 24.1.2023)
- ↑ Henry K. Paraneoplastic syndromes: Definitions, classification, pathophysiology and principles of treatment. InSeminars in diagnostic pathology 2019 Jul 1 (Vol. 36, No. 4, pp. 204-210). WB Saunders.
- ↑ 6.0 6.1 6.2 Thapa B, Ramphul K. Paraneoplastic syndromes. InStatPearls [Internet] 2020 Jun 27. StatPearls Publishing.Available from:https://www.ncbi.nlm.nih.gov/books/NBK507890/ (last accessed 15.7.2020)
- ↑ Mayo Clinic. Paraneoplastic syndromes of the nervous system. http://www.mayoclinic.com/health/paraneoplastic-syndromes/DS00840. (accessed 18 March 2013).