Cystic malformation (lymphangioma) of the thoracic lymphatic duct (peculiarities of clinical and radiological diagnosis, a brief review)

   Introduction. The thoracic lymphatic duct in the upper half of the body collects lymph from the left lung, the left half of the heart, the walls of the left half of the chest, from the left upper limb and the left half of the neck and head. Therefore, the left-sided localization of the revealed pathological changes, with the involvement of the mediastinum and neck in the process at the same time, can be a sign of damage to the thoracic lymphatic duct (at the location), and therefore the key to a successful diagnosis of the disease.

   Aim. Demonstration of modern possibilities of clinical and radiological diagnosis of cystic malformation of the thoracic lymphatic duct (CMTLD).

   Results. In the literature, the pathology of the thoracic lymphatic duct is designated by two terms: “lymphangioma” and “cystic malformation of the thoracic lymphatic duct”. According to the generally accepted classification of vascular formations ISSVA (adopted at the 20th ISSVA conference in Melbourne in April 2014, last revised in May 2018), the term “lymphangioma” with regard to lymphatic malformations is considered obsolete, its use is recommended for true benign tumors of the lymphatic vessels. CMTLD – benign, extremely rare lymphovascular lesions resulting from embryological disorders (weakness of the duct wall and valve mechanism) in the development of the lymphatic system. The median prevalence is less than 1 % among all mediastinal cystic formations. Effective non-invasive diagnostics at the initial visit of the patient already at the outpatient stage deserves professional evaluation. We present the own clinical and radiological observation of a 26-year-old patient without any special clinical manifestations, in whom volumetric changes in the mediastinum on the left were detected during routine fluorography. The nature (X-ray semiotics) of preoperative imaging using computed tomography has been clarified. The revealed X-ray and pathoanatomical changes were compared.

   Conclusion. CMTLD – congenital lymphatic anomalies, more frequent and symptomatic in children. In older patients, acquired and asymptomatic CMTLD are most often diagnosed. The logistics of modern imaging technologies makes it possible to successfully diagnose such a rare disease as CMTLD already at the stage of the patient’s initial visit to the doctor.

1. Monroe E. J. Brief description of ISSVA classification for radiologists // Tech. Vasc. Interv. Radiol. 2019. Vol. 22, Iss. 4. Article number: 100628. https://doi.org/10.1016/j.tvir.2019.100628

2. North P. E. Pediatric vascular tumors and malformations // Surg. Pathol. Clin. 2010. Vol. 3, Iss. 3. P. 455−494. https://doi.org/10.1016/j.path.2010.07.002

3. Takeda S., Miyoshi S., Minami M., Ohta M., Masaoka A., Matsuda H. Clinical spectrum of mediastinal cysts // Chest. 2003. Vol. 124, Iss. 1. P. 125−132. https://doi.org/10.1378/chest.124.1.12

4. Машинец Н. В. Лимфангиома подмышечной области гигантских размеров: обзор литературы и собственные наблюдения / Н. В. Машинец [и др.] // Пренатальная диагностика. – 2017. – Т. 16, № 4. – С. 319−324.

5. Le Pimpec-Barthes F., Cazes A., Bagan P., Badia A., Vlas C., Hernigou A., Pricopi C., Riquet M. Mediastinal cysts: clinical approach and treatment // Rev. Pneumol. Clin. 2010. Vol. 66, Iss. 1. P. 52−62. https://doi.org/10.1016/j.pneumo.2009.12.009

6. Traibi A., El Hammoumi M., El Oueriachi F., Arsalane A., Kabiri E. H. Benign cysts of the mediastinum: series of 28 cases // Rev. Mal. Respir. 2012. Vol. 29, Iss. 9. P. 1111−1115. https://doi.org/10.1016/j.rmr.2011.09.053

7. Esme H., Eren S., Sezer M., Solak O. Primary mediastinal cysts: clinical evaluation and surgical results of 32 cases // Tex. Heart Inst. J. 2011. Vol. 38, Iss. 4. P. 371−374.

8. Correia F. M., Seabra B., Rego A., Duarte R., Miranda J. Cystic lymphangioma of the mediastinum // J. Bras. Pneumol. 2008. Vol. 34, Iss. 11. P. 982−984. https://doi.org/10.1590/s1806-37132008001100015

9. Gupta M., Lovelace T. D., Sukumar M., Gosselin M. V. Cervical thoracic duct cyst // J. Thorac. Imaging. 2005. Vol. 20, Iss. 2. P. 107−109. https://doi.org/10.1097/01.rti.0000148208.74252.84

10. Jeung M. Y., Gasser B., Gangi A., Bogorin A., Charneau D., Wihlm J. M., Dietemann J. L., Roy C. Imaging of cystic masses of the mediastinum // Radiographics. 2002. Vol. 22 (Spec No). P. S79−S93. https://doi.org/10.1148/radiographics.22.suppl_1.g02oc09s79

11. Shaffer K., Rosado-de-Christenson M. L., Patz E. F. Jr., Young S., Farver C. F. Thoracic lymphangioma in adults: CT and MR imaging features // Am. J. Roentgenol. 1994. Vol. 162, Iss. 2. P. 283–289. https://doi.org/10.2214/ajr.162.2.8310910

12. Hazmiri F. E., Nachite F., Skandour D., Raji A., El Ganouni N. C. I., Rais H. Lateral cervical thymic cyst in a child: a case report // BMC Res. Notes. 2018. Vol. 30, Iss. 11. Article number: 85. https://doi.org/10.1186/s13104-018-3208-x

13. Chen F., Bando T., Hanaoka N., Terada Y., Ike O., Wada H., Hitomi S. Mediastinal thoracic duct cyst // Chest. 1999. Vol. 115, Iss. 2. P. 584−585. https://doi.org/10.1378/chest.115.2.584

14. Salehi F., Landis M., Inculet R., Wiseman D. Case report of a rare cystic mediastinal lymphangioma mimicking recurrent pleural effusion // Case Rep. Radiol. 2019. Vol. 2019. Article number: 1301845. https://doi.org/10.1155/2019/1301845

15. Пикин О. В. Лимфатическая мальформация шейно-надключичной области с медиастинальным компонентом больших размеров / О. В. Пикин [и др.] // Хирургия. Журнал им. Н. И. Пирогова. – 2018. – № 12. – С. 132−135. https://doi.org/10.17116/hirurgia2018121132

16. Carreira-Delgado M., Fernández-Rodríguez E., Martínez-Míguez M., Álvarez-Martín M. J., Nuño Vázquez-Garza J. M. Cervical thoracic duct cyst: An uncommon entity // Cir. Cir. 2017. Vol. 85, Suppl. 1. P. 40−43. https://doi.org/10.1016/j.circir.2016.11.010

17. Frola C., Loria F., Cantoni S. Thoracic duct dilatation in a case of lymphangioleiomyomatosis. The computed tomographic and magnetic resonance aspects // Radiol. Med. 1994. Vol. 88, Iss. 5. P. 687−688.

18. Legras A., Mordant P., Le Pimpec-Barthes F., Riquet M. Lymphangioma and lymphangiectasia // Rev. Pneumol. Clin. 2013. Vol. 69, Iss. 5. P. 272−277. https://doi.org/10.1016/j.pneumo.2013.04.002

19. Степанова Ю. А. Возможности лучевых методов исследования в диагностике кистозных лимфангиом на этапах хирургического лечения / Ю. А. Степанова [и др.] // Медицинская визуализация. – 2008. – № 1. – С. 52−66.

20. Перенести в английский вариант

21. Monroe E. J. Brief description of ISSVA classification for radiologists // Tech. Vasc. Interv. Radiol. 2019. Vol. 22, Iss. 4. Article number: 100628. https://doi.org/10.1016/j.tvir.2019.100628

22. North P. E. Pediatric vascular tumors and malformations // Surg. Pathol. Clin. 2010. Vol. 3, Iss. 3. P. 455−494. https://doi.org/10.1016/j.path.2010.07.002

23. Takeda S., Miyoshi S., Minami M., Ohta M., Masaoka A., Matsuda H. Clinical spectrum of mediastinal cysts // Chest. 2003. Vol. 124, Iss. 1. P. 125−132. https://doi.org/10.1378/chest.124.1.12

24. Mashinets N. V., Demidov V. N., Podurovskaya Y. L., Dorofeeva E. I., Panin A. P. [Giant axillary lymphangioma: literature review and own case reports]. Prenatal'naya diagnostika = Prenatal Diagnosis 2017; 16 (4): 319−324 (in Russian).

25. Le Pimpec-Barthes F., Cazes A., Bagan P., Badia A., Vlas C., Hernigou A., Pricopi C., Riquet M. Mediastinal cysts: clinical approach and treatment // Rev. Pneumol. Clin. 2010. Vol. 66, Iss. 1. P. 52−62. https://doi.org/10.1016/j.pneumo.2009.12.009

26. Traibi A., El Hammoumi M., El Oueriachi F., Arsalane A., Kabiri E. H. Benign cysts of the mediastinum: series of 28 cases // Rev. Mal. Respir. 2012. Vol. 29, Iss. 9. P. 1111−1115. https://doi.org/10.1016/j.rmr.2011.09.053

27. Esme H., Eren S., Sezer M., Solak O. Primary mediastinal cysts: clinical evaluation and surgical results of 32 cases // Tex. Heart Inst. J. 2011. Vol. 38, Iss. 4. P. 371−374.

28. Correia F. M., Seabra B., Rego A., Duarte R., Miranda J. Cystic lymphangioma of the mediastinum // J. Bras. Pneumol. 2008. Vol. 34, Iss. 11. P. 982−984. https://doi.org/10.1590/s1806-37132008001100015

29. Gupta M., Lovelace T. D., Sukumar M., Gosselin M. V. Cervical thoracic duct cyst // J. Thorac. Imaging. 2005. Vol. 20, Iss. 2. P. 107−109. https://doi.org/10.1097/01.rti.0000148208.74252.84

30. Jeung M. Y., Gasser B., Gangi A., Bogorin A., Charneau D., Wihlm J. M., Dietemann J. L., Roy C. Imaging of cystic masses of the mediastinum // Radiographics. 2002. Vol. 22 (Spec No). P. S79−S93. https://doi.org/10.1148/radiographics.22.suppl_1.g02oc09s79

31. Shaffer K., Rosado-de-Christenson M. L., Patz E. F. Jr., Young S., Farver C. F. Thoracic lymphangioma in adults: CT and MR imaging features // Am. J. Roentgenol. 1994. Vol. 162, Iss. 2. P. 283–289. https://doi.org/10.2214/ajr.162.2.8310910

32. Hazmiri F. E., Nachite F., Skandour D., Raji A., El Ganouni N. C. I., Rais H. Lateral cervical thymic cyst in a child: a case report // BMC Res. Notes. 2018. Vol. 30, Iss. 11. Article number: 85. https://doi.org/10.1186/s13104-018-3208-x

33. Chen F., Bando T., Hanaoka N., Terada Y., Ike O., Wada H., Hitomi S. Mediastinal thoracic duct cyst // Chest. 1999. Vol. 115, Iss. 2. P. 584−585. https://doi.org/10.1378/chest.115.2.584

34. Salehi F., Landis M., Inculet R., Wiseman D. Case report of a rare cystic mediastinal lymphangioma mimicking recurrent pleural effusion // Case Rep. Radiol. 2019. Vol. 2019. Article number: 1301845. https://doi.org/10.1155/2019/1301845

35. Pikin O. V., Ryabov A. B., Glushko V. A., Kolbanov K. I., Vursol D. A., Aleksandrov O. A. [Lymphatic malformation of cervical-supraclavicular area with large mediastinal component]. Pirogov Russian Journal of Surgery = Khirurgiya. Zurnal im. N. I. Pirogova 2018; (12): 132−135 (in Russian). https://doi.org/10.17116/hirurgia2018121132

36. Carreira-Delgado M., Fernández-Rodríguez E., Martínez-Míguez M., Álvarez-Martín M. J., Nuño Vázquez-Garza J. M. Cervical thoracic duct cyst: An uncommon entity // Cir. Cir. 2017. Vol. 85, Suppl. 1. P. 40−43. https://doi.org/10.1016/j.circir.2016.11.010

37. Frola C., Loria F., Cantoni S. Thoracic duct dilatation in a case of lymphangioleiomyomatosis. The computed tomographic and magnetic resonance aspects // Radiol. Med. 1994. Vol. 88, Iss. 5. P. 687−688.

38. Legras A., Mordant P., Le Pimpec-Barthes F., Riquet M. Lymphangioma and lymphangiectasia // Rev. Pneumol. Clin. 2013. Vol. 69, Iss. 5. P. 272−277. https://doi.org/10.1016/j.pneumo.2013.04.002

39. Stepanova J. A., Karmazanovsky G. G., Kubjshkin V. A., Dalgatov K. D., Shchegolev A. I., Tsvirkun V. V., Nikitaev N. S., Osipova N. Yu., Dubova E. A. [Abilities of imaging modalities in diagnostics of cystic lymphangiomas at a stage of a surgical treatment]. Meditsinskaya vizualizatsiya = Medical visualization 2008; (1): 52−66 (in Russian).