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Symptomatic features of paraneoplastic syndrome cultivate in four ways: endocrine, neurological, mucocutaneous, and hematological.The most common presentation is a fever (release of endogenous pyrogens often related to lymphokines or tissue pyrogens), but the overall picture will often include several clinical cases observed which may specifically simulate more common benign conditions.
Renal cell carcinoma (RCC) is a kidney cancer that originates in the lining of the proximal convoluted tubule, a part of the very small tubes in the kidney that transport primary urine. RCC is the most common type of kidney cancer in adults, responsible for approximately 90–95% of cases. [1]
Dermatomyositis is an autoimmune disorder featuring both humoral and T-cell autoimmune processes. [3] Dermatomyositis may develop as a paraneoplastic syndrome associated with several forms of malignancy. [4] It is known to be associated with several viruses, especially coxsackievirus, but no definitive causal link has been found. [3]
Stauffer syndrome causes abnormal liver function tests, especially those that reflect the presence of cholestasis, i.e. abnormal bile flow. Hepatosplenomegaly may also be observed. The symptoms and signs resolve if the renal cell carcinoma (or another associated tumor) is successfully ablated. It is due to release of IL-6 from cancerous cell. [1]
LEMS is often associated with lung cancer (50–70%), specifically small-cell carcinoma, [4] making LEMS a paraneoplastic syndrome. [5] Of the people with small-cell lung cancer, 1–3% have LEMS. [3] In most of these cases, LEMS is the first symptom of the lung cancer, and it is otherwise asymptomatic. [3]
The estimated number of cases of the disease is one in 1.5 million people per year. [9] [7] The condition is relatively common compared to other paraneoplastic disorders. [2] About 80% of those affected are female. [2] It typically occurs in adults younger than 45 years old, but it can occur at any age.
It predisposes individuals to renal cell cancer, an association denominated hereditary leiomyomatosis and renal cell cancer. [1] [2] It is also associated with increased risk of uterine leiomyosarcoma. [3] The syndrome is caused by a mutation in the fumarate hydratase gene, which leads to an accumulation of fumarate.
In hematology, plasma cell dyscrasias (also termed plasma cell disorders and plasma cell proliferative diseases) are a spectrum of progressively more severe monoclonal gammopathies in which a clone or multiple clones of pre-malignant or malignant plasma cells (sometimes in association with lymphoplasmacytoid cells or B lymphocytes) over-produce and secrete into the blood stream a myeloma ...