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27 January 2016 - Case of the Week #377

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Case of the Week #377

Clinical history:
A 29 year old old woman had a swelling at the nape of neck since 10 years of age, now measuring 2 x 1.5 cm and diagnosed clinically as lipoma. The lesion was excised.

Gross description:
The specimen was a skin covered nodule measuring 2 x 1.5 cm. The cut surface was cystic, containing gelatinous fluid and a central greyish white soft area measuring 1.0 x 0.3 cm.

Micro images:

What is your diagnosis?

Lymphatic malformation with solid and cystic (mixed large and smaller) features

Histopathology shows skin with an attenuated epidermis and large, variably-sized cystic spaces in the dermis containing eosinophilic, proteinaceous lymphatic fluid. These cystic spaces are lined by flattened, banal endothelial cells. Within the interstitium are small lymphoid aggregates. The channels are highlighted by D2-40, a lymphatic endothelial marker.

Lymphatic malformations usually occur in the cervical region and involve the posterior triangle of the neck, and were classified by Mulliken and Young into microcystic, macrocystic and mixed types (Mulliken, Young: Vascular birthmarks: hemangiomas and malformations, 1988). The microcystic type includes lymphangioma circumscriptum, verrucous hemangioma and angiokeratoma circumscriptum. The macrocystic type includes cavernous lymphangioma and cystic hygroma and is often multiloculated with thin-walled septa. Focal lymphoid aggregates are often seen, which may also contain lymphatic spaces. Some cases, including the presented case, may have extensive lymphoid aggregates which can be mistaken for pseudolymphoma. Pseudolymphoma would demonstrate germinal centers mixed with plasma cells. A malignant vascular lesion can be excluded by the lack of significant cytologic atypia among endothelial cells and a reassuring growth pattern of the lesion (well-formed vascular channels and relative circumscription).

Treatment is usually by surgical excision, but nonsurgical techniques such as cryotherapy have been used with lesser results. The prognosis is excellent and there is no malignant transformation associated with lymphatic malformation. Of note, the term "lymphangioma" has the potential to cause anxiety to patients as it implies a neoplastic lesion; thus the designation "cystic lymphatic malformation" may be preferred for localized malformations of lymphatics.

References: Patterson: Weedon's Skin Pathology, 2015, J Cutan Aesthet Surg 2010;3:139, Barnhill: Dermatopathology: Third Edition, 2010, J Maxillofac Oral Surg 2010;9:284

Discussion edited by Dr. Jennifer R. Kaley, University of Arkansas for Medical Sciences (USA).

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