Lymphangioma Workup

Updated: Apr 23, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Workup

Imaging Studies

MRI can help define the degree of involvement and the entire anatomy of the lymphangioma lesion. MRI can help prevent unnecessary extensive, incomplete surgical resection, because of the association with a high recurrence rate.

The scrotum is not a usual site, but a painless scrotal swelling was documented as a lymphangioma by typical sonography and MRI findings, followed by excision and pathologic confirmation. [22]

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Other Tests

Immunohistochemical study is useful in differentiating lymphangiomas from hemangiomas in difficult cases. Test results with factor VIII – related antigen are positive for hemangiomas but negative or weakly positive in the endothelium of lymphangiomas. Immunohistochemical studies for laminin show the typical multilayered basal lamina of normal blood vessels and the discontinuous basal lamina in lymphangiomas.

Dermoscopic findings may aid in the diagnosis of cutaneous lymphangioma circumscriptum. [23, 24, 25] Nodules filled with clear fluid show light brown lacunas surrounded by paler septa. Those tinged with blood may have focal reddish areas inside the lagoons, pink diffuse coloration, and/or reddish to violaceous lacunar structures. In those lacunae that contained blood, it accumulated in the lowest part. [26] Thus, they are characterized by a lacunar pattern and with a marked hematic content may be indistinguishable from a hemangioma.

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Procedures

The diagnosis of lymphangiomas is based mainly on the clinical history and findings from physical examination and conventional light microscopy.

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Histologic Findings

Microscopically, the vesicles in lymphangioma circumscriptum are greatly dilated lymph channels that cause the papillary dermis to expand. They may be associated with acanthosis and hyperkeratosis. These channels are numerous in the upper dermis and often extend to the subcutis. These deeper vessels seem to have a large caliber, and they often have a thick wall that contains smooth muscle. The lumen is filled with lymphatic fluid, but it often contains red blood cells, lymphocytes, macrophages, and neutrophils. These channels are lined by flat endothelial cells, which stain positive for Ulex europaeus agglutinin-I. The interstitium often has numerous lymphoid cells and shows evidence of fibroplasia.

Nodules in cavernous lymphangioma are characterized by large, irregular channels in the reticular dermis and subcutaneous tissue that are lined by a single layer of endothelial cells. An incomplete layer of smooth muscle often lines the walls of these malformed channels. The surrounding stroma consists of loose or fibrotic connective tissue with a number of inflammatory cells. These tumors often penetrate muscle.

Cystic hygroma is indistinguishable from cavernous lymphangiomas on histology.

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