Synonyms
Definition
Lymphangiomas are benign malformations of the lymphatic system with channels and cystic spaces of varying size that may occur throughout the body. They are categorized into microcystic type (cavernous lymphangioma), macrocystic type (cystic hygroma), or mixed type.
Clinical Features
Incidence
Lymphangiomas represent 4% of all vascular tumors and 25% of all benign pediatric vascular tumors, with an incidence of approximately 1 in 2000–4000 live births in congenital cases (Miceli and Stewart, 2019; Sun et al., 2017).
Age
Lymphangiomas typically present at birth or in the first few years of life. They are often diagnosed prenatally (Goldblum et al., 2013).
Sex
There is no gender bias in terms of their occurrence (Goldblum et al., 2013).
Site
The most common sites are the head and neck (48%). Other reported sites are the trunk and extremities (42%). Some cases arise at the intrathoracic or abdominal compartment (10%) (Sun et al., 2017...
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References
Beham, A. (2013). Lymphangioma. In Fletcher, C. D. M., Bridge, J. A., Hogendoorn, P. C. W., & Mertens, F. (Ed.), WHO Classification of Tumors of Soft Tissue & Bone (4th ed., pp. 144–145)
Goldblum, J. R., Folpe, A. L., & Weiss, S. W. (2013). Tumors and malformations of lymphatic vessels. In F. M. Enzinger & S. W. Weiss (Eds.), Enzinger and Weiss’s soft tissue tumors (6th ed., pp. 733–748). Philadelphia: Mosby Elsevier.
Miceli, A., & Stewart, K. M. (2019). Lymphangioma. StatPearls [Internet]. Treasure Island: StatPearls Publishing. https://www.ncbi.nlm.nih.gov/pubmed/?term=lymphangioma+statpearls. Accessed 10 July 2019.
Sun, R. W., Tuchin, V. V., Zharov, V. P., Galanzha, E. I., & Richter, G. T. (2017). Current status, pitfalls and future directions in the diagnosis and therapy of lymphatic malformation. Journal of Biophotonics, 11, e201700124.
Suthiwartnarueput, W., Kiatipunsodsai, S., Kwankua, A., & Chaumrattanakul, U. (2012). Lymphangioma of the small bowel mesentery: A case report and review of the literature. World Journal of Gastroenterology, 18(43), 6328–6332.
Wu, J. K., Kitajewski, C., Reiley, M., Keung, C. H., Monteagudo, J., Andrew, J. P., Liou, P., Thirumoorthi, A., Wong, A., & Kandel, J. J. (2015). Aberrant lymphatic endothelial progenitors in lymphatic malformation development. PLoS One, 10(2), e0117352. https://doi.org/10.1371/journal.pone.0117352.
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Abe, C., Mori, T., Yamada, Y., Oda, Y. (2020). Lymphangioma, Soft Tissue. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5487-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5487-1
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