Lymphangioma-Like Kaposi Sarcoma.

August 2012

We report on a recent lesion in a 75-year-old woman who

had been diagnosed with classic Kaposi sarcoma (KS) 8 years earlier, at which time she presented with plaques on the right thigh and right forearm (Fig. 1A) and no metastasis was detected. Findings from the initial biopsy of the forearm lesions were compatible with nodular KS.

During the 8-year follow-up, the patient developed 7 recurrent skin tumors on the forearm and legs. Each tumor had the same clinical appearance in the form of plaques (Fig. 1B), and no systemic involvement was detected at any time. The lesions were treated with liposomal doxorubicin. Biopsies were taken from the forearm at each recurrence and histology continued to show findings consistent with nodular KS mixed with lymphangioma-like areas. 

These areas were comprised of irregularly-dilated ectatic vascular spaces in the reticular dermis that were lined by moderately atypical endothelial cells. These spaces were greater in number and size than normal lymphatic vessels (Fig. 2). Immunohistochemistry showed strong CD34 positivity. Endothelial cells lining the lymphangioma-like areas of the tumor were

also positive on staining with human herpes virus type 8

(HHV-8) antibody (Fig. 2C) and the lymphatic endothelial

marker podoplanin (D2-40) (Fig. 2D). Based on these findings, the patient was diagnosed with lymphangioma-like KS.

Seven months after completing the most recent cycle of

chemotherapy with doxorubicin, the patient presented with a serious recurrence consisting of localized, multinodular, ulcerated lesions on both legs but more severe on the right thigh; severe lymphedema was also noted (Fig. 1C). She was given radiotherapy, second-line chemotherapy with paclitaxel (taxol), and thorough local treatments. At the time of writing the lesions remained ulcerated and had not changed in size. The patient attended scheduled follow-up visits and did not show signs of systemic spread of disease.

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Elsevier