Definition
It is a chronic disease with extracellular amyloid deposits in the genitourinary organs.
Clinical-Pathological Classification
Systemic:
Primary: Commonly AL type amyloid typically associated with some lymphoproliferative disorders.
Secondary: AA type amyloid, linked to various chronic inflammatory diseases (rheumatoid arthritis, Crohn disease, ankylosing spondylitis, myeloma, and familial Mediterranean fever).
Localized: Deposition of AL type amyloid of unknown etiology.
Familial: Frequently associated with Transthyretin mutations.
Clinical Features
Incidence
The true incidence of systemic and localized amyloidosis in genitourinary organs is not currently available.
Age
5th–6th decade.
Sex
Systemic: Men are mainly affected by AL amyloidosis, women by AA amyloidosis.
Localized: No sex prevalence.
Site
Systemic: Kidney most commonly affected (Chitale et al. 2007).
Localized: Renal pelvis and lower genitourinary tract, particularly urinary bladder but also urethra (Monge et al.
References and Further Reading
Caballero MartÃnez, M. C., Gomez, M., Angel Cuesta Alcalá J, et al. (2003). Amyloidosis of the seminal vesicles: Clinical and pathological review of 8 cases diagnosed from surgical specimens. Archivos Españoles de UrologÃa, 56, 431.
Chitale, S., Morsey, M., Peat, D., & Webb, R. (2007). Amyloidosis of lower genitourinary tract: A review. European Urology Supplements, 5, 70–76.
Jain, M., Kumari, N., Chhabra, P., & Gupta, R. (2008). Localized amyloidosis of urinary bladder: A diagnostic dilemma. Indian Journal of Pathology & Microbiology, 51, 247–249.
Li, Y., Mongan, J., Behr, S. C., et al. (2016). Beyond prostate adenocarcinoma: Expanding the differential diagnosis in prostate pathologic conditions. Radiographics, 36, 1055–1075.
Monge, M., Chauveau, D., Cordonnier, C., et al. (2011). Localized amyloidosis of the genitourinary tract: Report of 5 new cases and review of the literature. Medicine, 90, 212–222.
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Fattorini, C., Lopez-Beltran, A., Raspollini, M.R. (2019). Amyloidosis in the Genitourinary Tract. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4779-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4779-1
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