Summary
Paraneoplastic syndromes are a set of clinical features that are caused by either an altered immune response to a systemic malignancy or because of substances (e.g., hormones, cellular proteins) produced by tumors. They are caused neither by the local mass effect nor by metastasis of the associated cancer. Paraneoplastic syndromes present with a wide range of findings and are most commonly associated with lung, ovarian, lymphatic, and breast cancers. Small cell lung cancer is especially notorious for its numerous and distinct paraneoplastic syndromes. Some paraneoplasias are associated with many different malignancies, such as tumor-induced hypercalcemia, which is caused by parathyroid hormone-related protein (PTHrP) produced from tumor cells. Other paraneoplasias (e.g., malignant acanthosis nigricans) are almost pathognomonic for specific malignancies and their presence demands an immediate search for the underlying tumor. A general overview of the most common paraneoplastic syndromes and their associations is presented here. Further details for individual syndromes can be found in articles of associated conditions.
Overview
Common paraneoplastic manifestations are cachexia, hyperthermia, and increased risk of thrombosis. [1]
Overview of the most common paraneoplastic syndromes [1][2] | ||||
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Type | Etiology | Associated cancer(s) | Characteristic features | |
Neuromuscular | Lambert-Eaton myasthenic syndrome |
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Myasthenia gravis |
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Stiff person syndrome |
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Polymyositis |
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Dermatomyositis |
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Neurological | Paraneoplastic encephalomyelitis |
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Paraneoplastic cerebellar degeneration |
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Opsoclonus-myoclonus syndrome |
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Limbic encephalitis [4] |
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Paraneoplastic pure sensory neuropathy |
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Endocrine | Cushing syndrome |
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Syndrome of inappropriate ADH secretion |
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Hypercalcemia of malignancy |
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Mucocutaneous | Malignant acanthosis nigricans |
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Leser-Trélat sign |
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Necrolytic migratory erythema |
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Hypertrichosis lanuginosa acquisita |
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Skeletal | Hypertrophic pulmonary osteoarthropathy (Bamberger-Marie syndrome) |
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Tumor-induced osteomalacia |
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Hematological | Polycythemia | |||
Pure red cell aplasia |
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Good syndrome |
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Trousseau syndrome (thrombophlebitis migrans) |
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Nonbacterial thrombotic endocarditis |
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Miscellaneous | Neoplastic fever |
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Membranous glomerulonephritis |
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“Rising or high hematocrit levels are paraneoplastic!” Renal cell carcinoma, Hepatocellular carcinoma, Hemangioblastoma, Leiomyoma, and Pheochromocytoma are associated with polycythemia.
Patients with OMS are described as having “dancing eyes” (opsoclonus) and “dancing feet” (myoclonus).