Lymphangioma Workup

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 18, 2012
 

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.

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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.[11, 12] 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. 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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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD  Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Carrie L Kovarik, MD  Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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A 44-year-old woman with plaque on her forearm since birth that clinically appeared to be typical lymphangioma circumscriptum. Histologically, however, it had features of both hemangioma and lymphangioma.
Close-up demonstrating the clinical morphology to better advantage.
A 16-year-old obese boy with large unilateral verrucous lymphangioma.
 
 
 
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