Abstract
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.
Similar content being viewed by others
References
Bermejo Casero EJ, Mongil Poce R, Arrabal Sanchez R, Fernandez de Rota Avecilla A, Benitez Domenech A, Fernandez Bermudez JL (2004) Diffuse thoracic lymphangiomatosis: diagnosis and treatment. Arch Bronconeumol 40:599–601
Byrne AM, Bouchier-Hayes DJ, Harmey JH (2005) Angiogenic and cell survival functions of vascular endothelial growth factor (VEGF). J Cell Mol Med 9:777–794
Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA (2000) Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 161:1037–1046
Huang HY, Ho CC, Huang PH, Hsu SM (2001) Co-expression of VEGF-C and its receptors, VEGFR-2 and VEGFR-3, in endothelial cells of lymphangioma. Implication in autocrine or paracrine regulation of lymphangioma. Lab Invest 81:1729–1734
Konez O, Vyas PK, Goyal M (2000) Disseminated lymphangiomatosis presenting with massive chylothorax. Pediatr Radiol 30:35–37
Ramani P, Shah A (1993) Lymphangiomatosis. Histologic and immunohistochemical analysis of four cases. Am J Surg Pathol 17:329–335
Salven P, Lymboussaki A, Heikkila P, Jaaskela-Saari H, Enholm B, Alitalo K, Joensuu H (1998) Vascular endothelial growth factors VEGF-B and VEGF-C are expressed in human tumors. Am J Pathol 153:103–108
Stacker SA, Achen MG, Jussila L, Baldwin ME, Alitalo K (2002) Lymphangiogenesis and cancer metastasis. Nat Rev Cancer 2:573–583
Takahashi K, Takahashi H, Maeda K, Homma S, Uekusa T, Dambara T, Kira S (1995) An adult case of lymphangiomatosis of the mediastinum, pulmonary interstitium and retroperitoneum complicated by chronic disseminated intravascular coagulation. Eur Respir J 8:1799–1802
Acknowledgement
This paper is supported by grants from the Ministry of Education, Culture, Sports, Science and Technology.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ikeda, Ji., Morii, E., Tomita, Y. et al. Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma. Virchows Arch 450, 211–214 (2007). https://doi.org/10.1007/s00428-006-0333-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00428-006-0333-z