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A 10-year-old boy is referred by his pediatrician for evaluation of a growth on his left ear lobe. His parents first noted the lesion several months ago and the child denies any discomfort at the site. He is otherwise in good health with normal mentation and average weight and height. Physical examination reveals a flesh-colored, readily compressible nodule in the affected area. No similar lesions are noted elsewhere.
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The patient’s lesion was excised and pathology report revealed a subcutaneous deep-type lymphangioma. Lymphangiomas are benign congenital lesions caused by malformation within the lymphatic system. Various terms have been used to describe these lesions including cystic hygroma (macrocystic-type), cavernous lymphangioma, cystic lymphangioma, and lymphangioma circumscriptum (microcystic type).1 The majority of lesions appear at birth through age 2 years.1 New-onset lymphangioma in an adult is quite uncommon.2 Areas of the body most often affected by lymphangioma are the head and neck.
Lymphatic malformations with skin involvement usually present as soft, compressible, nontender growths. The cause is unknown although a significant number of patients have specific gene alterations that promote cellular overgrowth.3 Lymphangiomas will slowly expand as the child grows, although some lesions may spontaneously decrease in size.1 Trauma and infection may accelerate growth. Complications associated with lymphangiomas include inflammation, bleeding, and, in aggressive forms, growth into surrounding structures.3
A number of therapies have proven successful in the management of uncomplicated lesions including excision, drainage, sclerotherapy, and laser ablation.1 Lymphangiomas are benign but can lead to cosmetic disfigurement and functional impairment of organs.1-3
Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Lymphatic Malformations: National Organization for Rare Disorders. Last updated June 17, 2021. Accessed January 23, 2023. https://rarediseases.org/rare-diseases/lymphatic-malformations/
2. Kurumety S, Morris M, Aydi ZB. New-onset axillary lymphangioma: a case report. J Med Case Rep. 2022;16(1):242. doi: 10.1186/s13256-022-03461-0
3. Scharf JL, Gembicki M, Dracopoulos C, et al. Lymphangioma of the fetal neck within the PIK3CA-related-overgrowth spectrum (PROS): a case report. Clin Case Rep. 2021;9(7):e04527. doi:10.1002/ccr3.4527
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