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BY-NC-ND 3.0 license Open Access Published by De Gruyter Open Access April 8, 2011

Mediastinal lymphangioma and generalized lymphangiomatosis a case report

Edin Jusufovic, Dragan Keser and Edin Zukic
From the journal Open Medicine

Abstract

Lymphangioma is a malformation composed of a mass of dilated lymph vessels typically found in the cervical region in children. Mediastinal lymphangioma is a rare condition and accounts for 0.01% to 4.5% of all mediastinal tumors. Only 4 cases of mediastinal lymphangioma involving the heart and great vessels in adults have been described in the available literature. Extremely rarely, lymphangiomas occur as a generalized lymphangiomatosis. We present a case of a woman who coughed up small amounts of fresh blood during 6 months and showed signs of cardiac failure. Several years previously, the patient underwent surgical removal of cystic lymphangiomas from the left ovary, both fallopian tubes and small intestine. A chest radiograph showed an 8-cm round shadow located in the middle lobe. A computerized tomography (CT) scan of the chest with contrast verified the existence of a cyst in the anterior mediastinum. The radiologist suggested that the cyst could have originated in the pericardium. One small cyst also appeared in the projection of the left cardiophrenic sinus. A CT scan with contrast of the patient’s abdomen showed multiple cystic formations in the liver, spleen, kidneys, and left parapelvic region. A fine-needle biopsy of the mediastinal tumor verified the cystic lymphangioma, which was then completely removed surgically. A pathohistological examination confirmed the existence of cystic lymphangioma. The patient was discharged after a period of recovery.

[1] Correia FM, Seabra B, Rego A, Duarte R, Miranda J. Cystic lymphangioma of the mediastinum. J Bras Pneumol. 2008, 34,11, 982–984 http://dx.doi.org/10.1590/S1806-3713200800110001510.1590/S1806-37132008001100015Search in Google Scholar

[2] Park J.G., Aubry M.C., Godfrey J.A., Midthun D.E. Mediastinal Lymphangioma: Mayo Clinic Experience of 25 Cases. Mayo Clin Proc., 2006, 81,9, 1197–1203 http://dx.doi.org/10.4065/81.9.119710.4065/81.9.1197Search in Google Scholar

[3] Yildirim E., Dural K., Kaplan T., Sakinci U. Cystic lymphangioma: report of two atypical cases. Interactive Cardiovascular and Thoracic Surgery, 2004, 3, 63–65 http://dx.doi.org/10.1016/S1569-9293(03)00225-110.1016/S1569-9293(03)00225-1Search in Google Scholar

[4] Saleiro S., Magalhães A., Moura C.S., Hespanhol V. Mediastinal cystic lymphangioma. Rev Port Pneumol., 2006, 12,6, 731–735 10.1016/S2173-5115(06)70433-XSearch in Google Scholar

[5] http://www.ncbi.nlm.nih.gov/pubmed?term=meiastinal%20lymphangioma%20heart. Date last accessed: February, 2nd 2011. Search in Google Scholar

[6] McLoughlin G.S., Nuchtern J.G., Dauser R.C., Sciubba D.M., Gokaslan Z.L., Wolinsky J.P. Mediastinal lymphangioma presenting as an acute epidural hematoma. J Neurosurg Pediatrics., 2008, 1,6, 474–476 http://dx.doi.org/10.3171/PED/2008/1/6/47410.3171/PED/2008/1/6/474Search in Google Scholar PubMed

[7] Zisis C., Spiliotopoulos K., Patronis M., Filippakis G., Stratakos G., Tzelepis G., Bellenis I. Diffuse lymphangiomatosis: are there any clinical or therapeutic standards? J Thorac Cardiovasc Surg., 2007, 133,6, 1664–1665 http://dx.doi.org/10.1016/j.jtcvs.2007.02.00510.1016/j.jtcvs.2007.02.005Search in Google Scholar PubMed

[8] Bilgin M., Akçali Y., Oguzkaya F., Öktem T. Mediastinal Cystic Lymphangioma: A Rare Mediastinal Tumour. Turkish Respiratory Journal, 2004, 5,3, 187–188 Search in Google Scholar

[9] Shenoy S.S., Barua N.R., Patel A.R., et al. Mediastinal lymphangioma. J Surg Oncol, 1978, 10, 523–528 http://dx.doi.org/10.1002/jso.293010060810.1002/jso.2930100608Search in Google Scholar PubMed

[10] Oshikiri T., Morikawa T., Jinushi E., Kawakami Y., Katoh H. Five Cases of Lymphangioma of Mediastinum in Adult. Ann Thorac Cardiovasc Surg., 2001, 7, 2 Search in Google Scholar

[11] Teramoto K., Suzumura Y. Mediastinal cavernous lymphangioma in an adult. Gen Thorac Cardiovasc Surg., 2008, 56,2, 88–90 http://dx.doi.org/10.1007/s11748-007-0198-310.1007/s11748-007-0198-3Search in Google Scholar PubMed

[12] Sinzelle E., Van Huyen J.P.D, Breiteneder-Geleff S., Braunberger E., Deloche A., Kerjaschki D., Bruneval P. Intrapericardial lymphangioma with podoplanin immunohistochemical characterization of lymphatic endothelial cells. Blackwell Science Ltd, Histopathology, 2000, 37, 85–94 10.1046/j.1365-2559.2000.00955-8.xSearch in Google Scholar PubMed

[13] Dumoff K.L., Chu C., Xu X., Pasha T., Zhang P.J., Acs G. Low D2-40 immunoreactivity correlates with lymphatic invasion and nodal metastasis in earlystage squamous cell carcinoma of the uterine cervix. Mod Pathol., 2005, 18,1, 97–104 http://dx.doi.org/10.1038/modpathol.380026910.1038/modpathol.3800269Search in Google Scholar PubMed

[14] Kahn H.J., Bailey D., Marks A. Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi’s and a subset of angiosarcomas. Mod Pathol., 2002, 15, 434–440 http://dx.doi.org/10.1038/modpathol.388054310.1038/modpathol.3880543Search in Google Scholar PubMed

[15] Ramani P., Shah A. Lymphangiomatosis: histologic and immunohistochemical analysis of four cases. Am J Surg Pathol., 1993, 17, 329–335 http://dx.doi.org/10.1097/00000478-199304000-0000210.1097/00000478-199304000-00002Search in Google Scholar

[16] Shin M.S., Berland L.L., Ho K.J. Mediastinal cytic hygromas. CT characteristics and pathogenetic consideration. J Comput Assist Tomogr., 1985, 9, 297–301 http://dx.doi.org/10.1097/00004728-198503000-0001210.1097/00004728-198503000-00012Search in Google Scholar

[17] http://www.ncbi.nlm.nih.gov/pubmed?term=mediastinal%20lymphangioma%20and%20lymph angiomatosis. Date last accessed: February, 2nd 2011 Search in Google Scholar

[18] Marom E.M., Moran C.A., Munden R.F. Generalized Lymphangiomatosis. Radiologic-Pathologic Conferences of The University of Texas M. D. Anderson Cancer Center., 2004, AJR 182 10.2214/ajr.182.4.1821068Search in Google Scholar PubMed

Published Online: 2011-4-8
Published in Print: 2011-6-1

© 2011 Versita Warsaw

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

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