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Year : 2003  |  Volume : 13  |  Issue : 3  |  Page : 347-348
Cystic lymphangioma of the retroperitoneum


J-13/42, Rajouri Garden, New Delhi 110027, India

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How to cite this article:
Narula M K, Gupta A. Cystic lymphangioma of the retroperitoneum. Indian J Radiol Imaging 2003;13:347-8

How to cite this URL:
Narula M K, Gupta A. Cystic lymphangioma of the retroperitoneum. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Feb 28];13:347-8. Available from: http://www.ijri.org/text.asp?2003/13/3/347/30486
Sir,

A ten-year-old girl presented with pain in abdomen of two months duration. There was no accompanying fever, weight loss or gastrointestinal symptoms. Clinical examination revealed a mass in the left hypochondrium. Routine laboratory investigations were within normal limits. Chest X-ray was normal. Plain X-ray abdomen revealed a soft tissue mass in the left upper abdomen, displacing the splenic flexure inferomedially. No evidence of calcification, fat-lucency or bone erosion was noted. Ultra-sonography revealed a 6.5 x 7 cm multiloculated cystic mass, with sharply defined borders [Figure - 1]. The cyst occupied the region between the tail of pancreas and the splenic hilum. The mass was seen to extend inferiorly, lying anterior to the left kidney, which was displaced. Retroperitoneal lymphangioma, cystic teratoma and hydatid cyst were considered as possibilities. CT showed a multicystic mass with internal septae. The attenuation values ranged from -20HU to +37HU. The septae were thin and non-enhancing [Figure - 2]. These features favoured the diagnosis of lymphangioma. Peroperatively, a multicystic mass was seen in the retroperitoneum with adhesions to the pancreatic tail and splenic hilum which was removed without resecting the pancreas or spleen. Histopathology confirmed the diagnosis of cystic lymphangioma. Post-operative ultrasound showed no cysts and further follow-up for 6 months showed no recurrence.

Lymphangiomas are uncommon retroperitoneal masses and are always benign. They are developmental malformations following the failure of lymphatic tissue to establish normal communication with the remainder of the lymphatic system. Pathologically, they are classified into simple, cavernous and cystic types [1]. Histologically, they appear as well circumscribed lesions composed of cysts of varying sizes and lined by flat endothelium. The adventitial layer may have fascicles of poorly developed smooth muscle and scattered aggregates of lymphoid tissue [2].

Lymphangiomas are most commonly reported in the head and neck region, followed by trunk and extremities. All other sites, including the abdomen, account for less than 5% of cases. Retroperitoneal lymphangiomas are even rarer than abdominal lymphangiomas of mesenteric origin [3]. Lymphangiomas tend to occur more commonly in the younger age group, with a slight male preponderance. They are frequently symptomatic. The symptoms may be pain, tenderness, fever, fatigue, weight loss and abdominal distension [2],[4]. The common complications associated with these tumors include infection and hemorrhage. These cysts may be associated with ascites [4].

Radiologically, a soft tissue haze may be noted in the abdomen, with occasional calcification within the mass [4]. It may cause significant displacement, pressure or entrapment of various organs, but without any invasion or mucosal irregularity. Sonographically, they appear as multicystic, multiseptate sharply defined masses. The cyst content could be fluid or fluid with solid elements owing to infection or hemorrhage [2]. CT demonstrates a unilocular or multilocular cystic mass lesion with attenuation values in the range of fat and water. Although a rare lesion, cystic lymphangioma should be considered in the differential diagnosis of multicystic mass in a child.

In the Indian setting, such cystic lesions have formerly been mistaken for encysted fluid collections in tuberculosis [3]. The retroperitoneal location, multicystic appearance and fat density on CT aided us in arriving at the correct diagnosis.

The treatment of choice for these lesions is complete enucleation. Retroperitoneal lymphangiomas have more chances of recurrence due to incomplete removal [3]. Hence, a regular post-operative follow-up with repeated sonography is suggested.

 
   References Top

1.Leonidas JC, Brill PW, Bhan I, Smith TH. Cystic retroperitoneal lymphangioma in infants and children. Radiology 1978;127:203-208.   Back to cited text no. 1  [PUBMED]  
2.Davidson AJ, Hartman DS. Lymphangioma of the retroperitoneum : CT and sonographic characteristics. Radiology 1990; 175: 507-510.   Back to cited text no. 2  [PUBMED]  
3.Patel AP, Kothari JM, Patel KM, Shukla RB, Anand A, Parikh BJ, Giri DD, Shah PM. Retroperitoneal Lymphangioma. The Ind. J. of Paed. 1994; 61(2) : 194-197.  Back to cited text no. 3    
4.Takiff H, Calabria R, Yin L, Stabile BE. Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch. Surg. 1985; 120: 1266-1269.  Back to cited text no. 4    

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M K Narula
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India
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