Lymphangioma.Lymphangiomas are rare congenital tumors, with up to 70% reported in the head and neck. They are separated into three types: cystic (cystic hygroma), capillary, and cavernous. Lymphangiomas account for approximately 25% of all vascular neoplasms in children and adolescents. About 25% of cervical cysts are lymphangiomas. Roughly two-thirds of all lymphangiomas are noted shortly after birth, and 95% are present by the end of the second year of life. Cystic hygroma may also be detected in utero by ultrasonography. Cystic hygroma is commonly associated with fetal hydrops and Turner syndrome. In general, symptoms relate to pressure caused by the enlarging mass in the posterior neck, although the tumor may extend into the anterior compartment, upward into the cheek, or down into the mediastinum mediastinum /me·di·as·ti·num/ (me?de-ah-sti´num) pl. mediasti´na [L.] 1. a median septum or partition. 2. or axilla axilla /ax·il·la/ (ak-sil´ah) pl. axil´lae [L.] the armpit.ax´illary ax·il·la n. pl. ax·il·lae See armpit. . When located superior to the hyoid bone hyoid bone n. A U-shaped bone at the base of the tongue that supports the muscles of the tongue. hyoid bone (hī´oid), n , the tumor may cause dysphagia or airway compression. The appearance of cystic hygromas varies from a single soft mass with a pseudocontour to lobulated lobulated /lob·u·lat·ed/ (lob´ul-at-id) made up of lobules. lobulated made up of lobules. multicystlc masses. Lymphangiomas (specifically, the cystic type) are made up of dilated thin-walled spaces that are filled with eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik) 1. readily stainable with eosin. 2. pertaining to eosinophils. 3. pertaining to or characterized by eosinophilia. proteinaceous fluid and lined with flat endothelial cells (figure 1). The intervening stroma stroma /stro·ma/ (stro´mah) pl. stro´mata [Gr.] the matrix or supporting tissue of an organ.stro´malstromat´ic stro·ma n. pl. stro·ma·ta 1. contains scattered lymphoid aggregates and wisps of smooth muscle fibers (figure 2). Fibrosis may be increased in lesions that have been present for a long duration. Endothelial markers (e.g., factor VIII-RAg, CD31, and CD34) will be positive. The most common differential diagnosis is cavernous hemangioma, which is filled with red blood cells Red blood cells Cells that carry hemoglobin (the molecule that transports oxygen) and help remove wastes from tissues throughout the body. Mentioned in: Bone Marrow Transplantation red blood cells and lacks valve structures. Metastatic papillary carcinoma of the thyroid may exhibit flattened cells along the spaces, but TTF-1 or thyroglobulin thyroglobulin /thy·ro·glob·u·lin/ (thi?ro-glob´u-lin) an iodine-containing glycoprotein of high molecular weight, occurring in the colloid of the follicles of the thyroid gland; the iodinated tyrosine moieties of thyroglobulin form the will be positive and a lymph node architecture should be seen. [FIGURES 1-2 OMITTED] Recurrence rates range from 15 to 50%. Mortality rates are between 3 and 7%, specifically related to pressure destruction of vital structures of the neck. Lymphangiomas may occasionally become infected. Surgery is the treatment of choice, while laser treatment and injected sclerosing agents are alternate therapies. Suggested reading Coffin CM, Dehner LP. Vascular tumors in children and adolescents: A clmicopathologic study of 228 tumors in 222 patients. Pathol Annu 1993:28:97-120. Weiss SW, Goldblum JR. Tumors of lymph vessels. In: Weiss SW, Goldblum JR, eds. Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St Louis: Mosby, 2001:955-83. From the Department of Pathology, Woodland Hills Medical Center. Southern California Permanente Medical Group, Woodland Hills, Calif. Lester D.R. Thompson, MD |
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