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DOI: 10.4103/0971-3026.190416
Young patient with generalized lymphangiomatosis: Differentiating the differential
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Abstract
We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.
Keywords
Cystic mediastinal mass - generalized lymphangiomatosis - pulmonary interstitial thickening - pulmonary lymphangiectasiaPublication History
Article published online:
30 July 2021
© 2016. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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References
- 1 Azouz EM, Saigal G, Rodriguez MM, Podda A. Langerhans' cell histiocytosis: Pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005;35:103-15.
- 2 Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161(Pt 1):1037-46.
- 3 Satria MN, Pacheco-Rodriguez G, Moss J. Pulmonary lymphangiomatosis. Lymphat Res Biol 2011;9:191-3.
- 4 de Lima AS, Martynychen MG, Florêncio RT, Rabello LM, de Barros JA, Escuissato DL. Pulmonary lymphangiomatosis: A report of two cases. J Bras Pneumol 2007;33:229–33.
- 5 Wunderbaldinger P, Paya K, Partik B, Turetschek K, Hörmann M, Horcher E, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. AJR Am J Roentgenol 2000;174:827-32.
- 6 Yang DH, Goo HW. Generalized lymphangiomatosis: Radiologic findings in three pediatric patients. Kor J Radiol 2006;7:287-91.
- 7 Kadakia KC, Patel SM, Yi ES, Limper AH. Diffuse pulmonary lymphangiomatosis. Can Respir J 2013;20:52-4.
- 8 Wadsworth DT, Newman B, Abramson SJ, Carpenter BL, Lorenzo RL. Splenic lymphangiomatosis in children. Radiology 1997;202:173-6.
- 9 Cutillo DP, Swayne LC, Cucco J, Dougan H. CT and MR imaging in cystic abdominal lymphangiomatosis.J Comput Assist Tomogr 1989;13:534-6.
- 10 Tazelaar HD, Kerr D, Yousem SA, Saldana MJ, Langston C, Colby TV. Diffuse pulmonary lymphangiomatosis. Hum Pathol 1993;24:1313-22.
- 11 Johnson DW, Klazynski PT, Gordon WH, Russell DA. Mediastinal lymphangioma and chylothorax: The role of radiotherapy. Ann Thorac Surg 1986;41:325-8.
- 12 Kandil A, Rostom AY, Mourad WA, Khafaga Y, Gershuny AR, el-Hosseiny G. Successful control of extensive thoracic lymphangiomatosis by irradiation. Clin Oncol (R Coll Radiol) 1997;9:407-11.
- 13 Dajee H, Woodhouse R. Lymphangiomatosis of the mediastinum with chylothorax and chylopericardium: Role of radiation treatment. J Thorac Cardiovasc Surg 1994;108:594-5.
- 14 Laverdière C, David M, Dubois J, Russo P, Hershon L, Lapierre JG. Improvement of disseminated lymphangiomatosis with recombinant interferon therapy. Pediatr Pulmonol 2000;29:321-4.
- 15 Tran D, Fallat ME, Buchino JJ. Lymphangiomatosis: A case report. South Med J 2005;98:669-71.
- 16 Ozeki M, Fukao T, Kondo N. Propranolol for intractable diffuse lymphangiomatosis. N Engl J Med 2011;364:1380-2.
- 17 Brown LR, Reiman HM, Rosenow EC 3rd, Gloviczki PM, Divertie MB. Intrathoracic lymphangioma. Mayo Clin Proc 1986;61:882-92.
- 18 Singh S, Baboo ML, Pathak IC. Cystic lymphangioma in children: Report of 32 cases including lesions at rare sites. Surgery 1971;69:947-51.