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| Year : 2004 | Volume
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| Issue : 4 | Page : 413-414 |
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| Retroperitoneal cystic lymphangioma |
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IBS Nijjar, JP Singh, V Arora, R Abrol, KS Gill, R Chopra, Roopa
Nijjar Scan & Diagnostic Centre Amritsar, India
Click here for correspondence address and email
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Keywords: Lymphangioma, multiseptated retroperitoneal cystic mass
How to cite this article:
Nijjar I, Singh J P, Arora V, Abrol R, Gill K S, Chopra R, Roopa. Retroperitoneal cystic lymphangioma. Indian J Radiol Imaging 2004;14:413-4 |
How to cite this URL:
Nijjar I, Singh J P, Arora V, Abrol R, Gill K S, Chopra R, Roopa. Retroperitoneal cystic lymphangioma. Indian J Radiol Imaging [serial online] 2004 [cited 2021 Feb 25];14:413-4. Available from: https://www.ijri.org/text.asp?2004/14/4/413/28684 |
 Introduction | |  |
We wish to report "Retroperitoneal cystic lymphangioma" diagnosed by various radiological imaging modalities and confirmed by histopathology.
| Case report | | |
A ten year old girl came to our centre for abdominal Ultrasonography, with complaint of blood in stools, on and off for the past one month. There was no history of mucus in stools, pain in abdomen, vomiting and fever.
Ultrasound abdomen was done with 3.5 MHZ convex transducer which showed an ill defined multiseptated cystic mass with good through transmission, measuring 11.0 x 5.5cm in size in right lumbar region, retroperitoneal in position [Figure - 1]. No movement was seen with respiration. The right kidney was seen to slide over the mass. A provisional diagnosis of retroperitoneal cystic lymphangioma was given.
The patient was taken up for CECT abdomen with oral and rectal contrast. CT abdomen revealed an irregular, multiloculated, lobulated, hypodense, retroperitoneal, non enhancing mass measuring 11.0 x 5.5 x 6.5cm in the right lumbar region [Figure - 2]. The lesion showed attenuation value ranging from -20 to +10 HU. It was lying postero-lateral to the lower pole of right kidney extending upto the right iliac fossa. Linear band like extension of the mass was seen postero-medial to the psoas and medial to the lower pole of right kidney upto the cisterna chyli in the aortocaval region. No calcification was observed in it. The ascending colon was displaced antero-medially by the mass. Right kidney was also displaced anteriorly. CT abdomen confirmed the sonographic findings.
On surgical exploration a large retroperitoneal, cystic, soft and fluctuating lesion was found which was separate from the mesentery and showed clear cleavage from all retroperitoneal organs. It was removed in toto and an incision over the specimen showed milky white chyle in the cyst, which confirmed the radiological diagnosis. The histopathology also confirmed it as lymphangioma [Figure - 3].
| Discussion | | |
Embryologically the lymphatic system is formed by the 8th week of intrauterine life and arises from six primitive sacs ; paired jugular sacs located lateral to the jugular veins, an unpaired retroperitoneal sac at the root of the mesentery, an unpaired cisterna chyli dorsal to the aorta and adrenal glands and paired posterior sacs located in relation to the sciatic veins. The postulated mechanism of formation of lymphangioma is early developmental sequestration of lymphatic vessels, that fail to establish connection with normal draining vessels at about 14-20 weeks of intrauterine life. They become markedly dilated under the pressure effect of accumulating lymph.
Lymphangiomas are mostly located in the neck, axilla and mediastinum (95%). The remaining 5% are found in the abdominal cavity (mesentery, omentum, retroperitoneal). Retroperitoneal lymphangioma are rarer than abdominal lymphangiomas of mesenteric origin. They tend to occur in the younger age group with slight male preponderance.
Three histological types are described: capillary, cavernous and cystic lymphangiomas. The cystic lymphangioma are usually multilocular, although they may be unilocular. They contain serous or chylous fluid. Clinical signs and symptoms are related to the size of the lesion.
Preoperative radiological diagnosis is possible only in 25% case. Plain radiograph shows a soft tissue haze with occasional calcification (10%) in the mass. It may cause displacement, pressure or entrapment of various organs including gut, but without any invasion or mucosal irregularity. Sonographically they appear as multi septate well defined cystic masses, CT demonstrates multilocular cystic mass lesion with attenuation values in the range of fat and water. Differential Diagnosis include mesenteric cyst, enteric duplication cyst and lymphocele.
Management of choice is complete surgical enucleation.[7]
| References | | |
| 1. | Davidson AJ and Hartman DS . Lymphangioma of the retro peritoneum : CT and sonographic characteristics. Radiology, 1990; 175 : 507-510.  |
| 2. | Hamrick-Turner JE, Chiechi MV, Abbitt PL. Neoplastic and inflammatory process of the peritoneum, omentum and mesentery : diagnosis with CT. Radiographics 1992 ; 12 : 1051-1068. |
| 3. | Kurtz RJ, Heimann TM . Mesenteric retroperitoneal cysts. Am Surg 1986; 203: 109. |
| 4. | Pandit SK, Rattan KN, Budhiraja S, Solanki PS. Cystic lymphangioma with special reference to rare sites. Indian journal of pediatrics 2000 ; 67 (5) : 339-41. |
| 5. | Parakh P, Shah V, Udawat M, Lalwani N. Congenital cystic lymphangioma with an uncommon presentation. Ind. J Radiol. Imaging 2002 ; 12:3 : 347-348. |
| 6. | Radian R, Weiner S, Koenigsberg M, Gold M, Bernstein R. Retroperitoneal cystic Lymphangioma. American journal of Radiology 1983 ;140; 733-734. |
| 7. | Yasui T, Akita H, Sasaki S, Veda V, Kabayashik K, Kohri K . Cystic Lymphangioma of retroperitoneum and groin. Urologia international 2000 ; 64 (2) : 115-7. |
Correspondence Address:
IBS Nijjar
Nijjar Scan & Diagnostic Centre, Amritsar 143001 (Pb).
India
 Source of Support: None, Conflict of Interest: None  | Check |
Figures
[Figure - 1], [Figure - 2], [Figure - 3] |
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