Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma.

Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma.
2006

Ikeda JI,
Morii E,
Tomita Y,
Zhang B,
Tokunaga T,
Inoue M,
Minami M,
Okumura M,
Aozasa K.

Department of Pathology, Graduate School of Medicine, Osaka University, Yamada-oka 2-2, Suita, 565-0871, Japan, morii@patho.med.osaka-u.ac.jp.

Mediastinal lymphangiomatosis in a 70-year-old woman was diagnosed on a medical checkup. The tumor was resistant to sclerotherapy with OK432 or bleomycin. The patient continued on a downhill course and died approximately 3 years after the initial diagnosis. Autopsy revealed a large tumor mass occupying the anterior mediastinum and firmly adhered to the pericardium and the pleura.

The tumor consisted of two intermingled lesions: dilated vessels lined with D2-40-positive lymphatic endothelium and CD5-positive atypical cell nests with focal keratinization. The former was diagnosed as lymphangiomatosis and the latter as thymic squamous cell carcinoma. Vascular endothelial growth factor (VEGF)-C, a growth factor for lymphatic endothelial cells, was expressed by the carcinoma, and VEGF-C receptor was expressed by the endothelium of lymphangiomatosis.

These findings suggested that VEGF-C derived from the thymic carcinoma induced the lymphangiomatosis lesion in a paracrine manner.

Keywords: Lymphangiomatosis - Thymic carcinoma - VEGF-C

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