Retroperitoneal Cystic Lymphangioma: Case Report

doi:10.4236/ss.2011.24046

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Surgical Science, 2011, 2, 209-211

doi:10.4236/ss.2011.24046 Published Online June 2011 (http://www.SciRP.org/journal/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

E-mail: [email protected]

Received January 8, 2011; revised March 30, 2011; accepted May 9, 2011.

Abstract

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-

phangioma.

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),

210 S. A. FANAEI ET AL.

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-

phangioma.

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

laparoscopy.

5. References

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[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

S. A. FANAEI ET AL.

211

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