Surgical Science, 2011, 2, 209-211
doi:10.4236/ss.2011.24046 Published Online June 2011 (
Copyright © 2011 SciRes. SS
Retroperitoneal Cystic Lymphangioma: Case Report
S. A. Fanaei1, S. A. Ziaee2
1Baqiyatall a h Uni versi t y of Medi c al Sci e nce, Tehran, Iran
2Clinical research Unit, Emergency Department, Atieh Hospital, Tehran, Iran
Received January 8, 2011; revised March 30, 2011; accepted May 9, 2011.
Introduction: Retroperitoneal cystic lymphangioma is a rare benign tumor of the retroperitoneal lymphatics
that usually manifests in infancy. It is worth to Report of unexpected presentation especially in Adult. Case
Presentation: An adult patient with Left Lower abdominal pain was referred to our hospital. Abdominal ul-
trasonography revealed a large, multilocular, cystic mass with an obscure margin. CT of the abdomen
showed a large homogeneous mass. Laparoscopy revealed a huge retroperitoneal cystic lymphangioma con-
firmed by histopathology. Conclusions: Adult cyst lymphangioma is the rare benign tumor with unclear intra
abdominal manifestation. In our report the retroperitoneum was the rare location of this tumor.
Keywords: Lymphangioma, Cystic, Retroperitoneal
1. Background
One of the developmental malformations of lymphatic
tissue is cystic lymphangioma. It is essentially a malfor-
mation of one of the abdominal lymph vessels where a
portion is dilated and forms a lymph fluid-filled cyst.
Symptoms may vary depending on the exact location an d
size of the cyst. Cystic lymphangioma is a rare benign
tumor. It usually manifests in infancy in head and neck
[1]. We report a rare case of adult retroperitoneal lym-
2. Case Report
A 26-year-old Female patient with a mass in left inguinal
fossa and left lower abdominal pain was referred to our
hospital. Her family and Gynecological and medical his-
tory were unremarkable. She had no history of previous
abdominal surgery. On physical examination, a soft,
non-tender mass was palpated in the left lower abdo men.
Auscultation of the abdomen revealed normal bowel
sounds. Laboratory data including tumor markers were
within normal limits. A plain film of the abdomen
showed several loops of small intestine without dilatation
or air-fluid level. Abdominal ultrasonography revealed a
large, multilocular, cystic mass with an obscure margin.
CT of the abdomen showed a large homogeneous mass
measuring 14 cm × 6 cm × 5 cm in size with multiple
enhancement septa by contrast medium. This lesion was
located near the descending colon, causing compression
and stretching of the bowel.
Preoperative diagnosis based on imaging investiga-
tions could not be made with any certainty and a percu-
taneous biopsy of the lesion was deemed too high-risk
due to the location and the possibility of malignant seed-
ing if the lesion was neoplastic.
A laparoscopy was ultimately performed. A large
retroperitoneal multicystic tumour attached descending
colon was identified. The cyst was excised intact. The
histopathological examination of the cyst wall demon-
strated fibrosis, inflammatory cell infiltrate and the
presence of endothelial lined vascular channels. (Figure
1) These findings confirmed a benign lymphangiomatous
cyst and a diagnosis of retroperitoneal cystic lymphan-
gioma. Recovery of the patient was uncomplicated.
Although macroscopically, the resection was complete,
lymphangioma recurred during a follow up period in this
case with chylascos presentation. (Figure 2 )
3. Discussion
The differential diagnosis of cystic tumour in the retrop-
eritoneum raises several possibilities. These include both
malignant and benign tumours. Malignant causes include
necrotic neoplasms, germ cell tumours (teratoma), un-
differentiated sarcoma, cystic me tastases (gastric/o varian),
Figure 1. Retroperitoneal cystic lymphangioma in a 24-
year-old woman. Photomicrograph (original magnification,
×40; hematoxylin-eosin [H-E] stain) shows variable-sized
thin-walled cystic spaces in the stroma that are lined with
endothelial cells and contain lymphoid aggregation.
Figure 2. laparoscopic excision of retroperitoneal cyst lym-
malignant mesenchymoma, biliary cystadenoma/carci-
noma and cystic mesothelioma [1]. Benign cystic lesions
of the retroperitoneum include lymphangioma, microcys-
tic pancreatic adenoma and cysts of urothelial and fore-
gut origin. Cysts of foregut origin are subdivided into
bronchogenic cysts, which contain cartilage or se-
romucinous respirator y glands, oesophageal cysts, which
are composed of wel l -developed laye rs o f s mooth muscle
without cartilage, and simple foregut cysts, which have
none of these distingui s hi ng features [1].
Retroperitoneal cystic lymphangioma is a rare, benign
mesodermal tumour arising from the retroperitoneal
lymphatics. Lymphangiomas are histologically classified
as capillary, cavernous and cystic with the retroperito-
neal types being almost always cystic [2]. These cystic
lesions may be unilocular or multilocular and contain
serous or chylous fluid. The majority (more than 95%)
are situated in the head, neck, axilla and extremeties.
Other sites including the abdomen are seen only in 5% of
cases [2]. The intraabdominal cystic lymphangiomas
occur in less than 1 per 100,000 hospital admissions.
Retroperitoneal lymphangiomas are rarer than abdominal
lymphangiomas of mesenteric origin. The postulated
mechanism for the formation of lymphangioma is the
early developmental sequestration of lymphatic vessels
that fail to establish connection with normal draining
vessels at about 14 to 20 weeks of intrauterine life [2].
Due to its potential to grow, invade vital structures and
develop life-threatening complications, complete laparo-
scopic excision should be considered as a therapeutic
option to treat retroperitoneal cystic lymphangioma [3].
If surgical excision is u sed in treatment, it needs to be as
complete as possible to reduce the risk of recurrence.
Trindade et al excised retroperitoneal lymphangioma [3].
The last report of retroperitoneal lymphangioma was
done by Ergul et al. [4] only 3 cases of lymphangioma
located in the lesser sac have been reported in the litera-
ture. [5-7] Hiroaki Tsukada et al. reported a rare case of
small bowel lymphangioma which was diagnosed with
laparoscopy [8]. However, J.-Y. Choi et al. showed that
a rare case of cavernous lymphang io ma origin ating in the
gallbladder. [9]
Outcomes following complete resection of retroperi-
toneal lymphangiomas are generally good. [3] Surgery is
often required for symptom control or diagnosis [1]. Re-
currence of symptoms with incomplete excision is possi-
ble. Dissemination in the retroperitoneum is very rare but
potentially a fatal complication [1]. Hauser et al. sug-
gested that isolation and ligation of the cystic lymphan-
gioma’s peduncle as well as ligation of lymph channels
can prevent recurrences and chylascos. [10]
4. Conclusions
First of all, diagnostic tools in such cases are not able to
support your differential diagnosis. In such circum-
stances surgeons and practitioners are recommended to
consider the rare cases .Adult retroperitoneal lymphan-
gioma is the rare case which can be diagnosed simply by
5. References
[1] M. Cherk, M. Nikfarjam and C. Christophi, “Retroperi-
toneal Lymphangioma,” Asian Journal of Surgery, Vol.
29, 2006, pp. 51-54. doi:10.1016/S1015-9584(09)60297-9
[2] D. V. Rani, R. Srilakshmi, S. Malathi, V. Raghupathy and
R. K. Bagdi, “Unusual Presentation of a Retroperitoneal
Lymphangioma,” Indian Journal of Pediatrics, 2006: 73;
617-618. doi:10.1007/BF02759928
Copyright © 2011 SciRes. SS
Copyright © 2011 SciRes. SS
[3] E. N. Trindade, M. R. Trindade, J. C. Boza, V. Von Die-
men and R. B. Ilgenfritz, “Laparoscpic Excision of a
Retroperitoneal Cystic Lymphangioma in an Elderly Pa-
tient,” Minerva Chirurgica, 2007; Vol. 62, No. 2, pp.
[4] E. Ergul, S. M. Sarikaya, B. Korukluoglu, A. E. Ucar and
A. Kusdemir: “Retroperitoneal Cystic Lymphangioma,”
The Internet Journal of Surgery, 2008,15,1.
[5] G. Uggeri, M. Arcidiaco, U. Pandolfi, et al. “Cystic Lym-
phangioma of Lesser Epiploon (On a Case Brought to
Our Attention),” Annali Italiani di Chirurgia, 1986, Vol.
56, pp. 159-170.
[6] Tezuka K, Ogawa Y, Satake K, et al. “Lymphangioma of
Lesser Omentum Associated with Abdominal and Eso-
phageal Carcinoma: Report of a Case,” Surgery Today,
2002; Vol. 32, pp. 362-366. doi:10.1007/s005950200053
[7] M. Ouaissi, O. Emungania, H. Sebbag, et al. “Cystic
Lymphangioma of Lesser Omentum,” La Presse Médi-
cale, 2003, Vol. 32, pp. 1165-1166.
[8] H. Tsukada, K. Takaori, S. Ishiguro, T. Tsuda, S. Ota and
T. Giant “Cysti lymphangioma of the Small Bowel Mes-
entery: Report of a Case,” Surgery Today, 2002, Vol. 32,
No. 8, pp. 734-737.
[9] J.-Y. Choi, M.-J. Kim, J.-J. Chung, S.-I. Park, J. T. Lee,
H. S. Yoo, L. Kim and J.-S Choi, “Gallbladder Lym-
phangioma: MR Findings,” Abdominal Imaging, 2002,
Vol. 27, No. 1.
[10] H. Hauser, H. J. Mischinger, A. Beham, A. Berger, H.
Cerwenka, J. Razmara, H. Fruhwirth and G. Werkgartner
“Cystic Retroperitoneal Lymphangiomas in Adults,” Eu-
ropean Journal Surgery Oncology, 1997, Vol. 23, pp.
322-326. doi:10.1016/S0748-7983(97)90777-0