INTRODUCTION

Mesenteric cystic lymphangiomas (MCLs) are rarecongenital benign malformations of the lymphaticsystem that are commonly located on the small bo-wel mesentery and less typically on the omentum,mesocolon and retroperitoneum (1). Most lymp-hangiomas are found in the head and neck; intra-abdominal locations are very unusual. Abdominalcystic lymphangiomas are more frequent in boys(5:2), with a mean age at presentation of 2 years(2). The common presentations of intra-abdominalcystic lymphangiomas are abdominal mass anddistension, and most have cystic and septal com-ponents (3). To the best of our knowledge, only six

mesenteric calcified cystic lymphangiomas inadults have ever been reported (4). We describe anextraordinary case of mesenteric calcified cysticlymphangioma in a 32-year-old woman.

CASE REPORT

A 32-year-old female patient presented to our cli-nic with stomachache that had been continuousfor approximately eight months. Her arterial blo-od pressure was 120/80 mmHg, pulse rate 85 be-ats/minute and respiration 19/minute, and hersurface temperature was normal. An approxima-tely 10 cm in diameter mass was palpable in her

Manuscript received: 09.02.2010 Accepted: 27.04.2010

Turk J Gastroenterol 2011; 22 (3): 341-343doi: 10.4318/tjg.2011.0224

Address for correspondence: Yavuz ALBAYRAKErzurum Region Education and Research Hospital, Department ofGeneral Surgery, Erzurum, TurkeyPhone: + 90 442 242 22 75E-mail: yavuzalbayrakdr@gmail.com

Mesenteric calcified cystic lymphangioma in anadult patient

Yavuz ALBAYRAK1, Fatih ALBAYRAK2, Serdar ARSLAN1, ‹lknur ÇALIK3

Department of 1General Surgery and 3Pathology, Erzurum Region Education and Research Hospital, ErzurumDepartment of 2Internal Medicine, Atatürk University, School of Medicine, Erzurum

Abdominal kistik lenfanjiomlar, lenfatik sistemin benign kistik malformasyonlar›d›r. Bildi¤imiz kadar›yla literatürde flu ana ka-dar alt› tane kalsifiye kistik lenfanjiom olgusu bildirilmifltir. Biz yaklafl›k sekiz ayd›r mide a¤r›s› flikayeti ile baflvuran bir bayanhastay› sunaca¤›z. Hastan›n abdominal bilgisayarl› tomografisinde kistik lezyon izlenmiflti. Eksplorasyonda kist total olarak eksi-ze edildi. Spesmenin histopatolojik de¤erlendirilmesinde kalsifiye kistik lenfanjiom oldu¤u tespit edildi. Eriflkinlerde çok nadir gö-rülmesine ra¤men, kar›n a¤r›s› flikayeti nedeni olarak mezenterik lenfanjiomalar ak›lda tutulmal›d›r. Tedavide kistin total eksiz-yonu uygulan›r. Bazen komflu dokularda birlikte eksize edilebilir.

Anahtar kelimeler: Kalsifiye kistik lenfanjioma, CD31, mezenter

Abdominal cystic lymphangiomas are rare congenital benign malformations of the lymphatic system. To the best of our knowledge,only 6 mesenteric calcified cystic lymphangiomas have ever been reported. We herein describe a woman who presented to our hospi-tal with stomachache that had been continuous for approximately 8 months. An abdominal computed tomography showed a cysticlesion. In the exploration, the cyst was totally excised. Based on the histomorphological data, a case of “calcified cystic lymphangio-ma” was diagnosed. Although mesenteric lymphangiomas are rare, especially in adults, they should be considered as a possible cau-se of abdominal pain. Treatment is surgical with resection of the mass, sometimes including resection of adjacent bowel.

Key words: Calcified cystic lymphangioma, CD31, mesentery

CASE REPORT

Yetiflkin hastada mezenterik kalsifiye kistik lenfanjioma

abdomen. The rest of the abdomen and a digitalrectal examination were unremarkable. Labora-tory results were within normal limits. The chestX-ray was normal. An abdominal computed to-mography showed an 8x7x7 cm cystic lesion withsharp boundaries and lobular contours in themiddle abdominal region between the jejunal loop,starting at the L2 vertebra level and ending at theL4 vertebra level. This was thought to be a mesen-teric hydatid or diverticular cyst, when evaluatedtogether with the abdominal ultrasonography ta-ken in correlation (Figure 1).

Surgery was planned for the patient. In the explo-ration, a white cystic lesion of approximately 8 cmin diameter, with increased vascularization andlobular contours, was observed in the intestinalmesentery, 200 cm proximal to the ileocecal valve.A decision was made to excise the lesion (Figure2). The cyst ruptured during excision and a milkywhite liquid drained out. It was totally excised.

The pathological study of slices taken from themacroscopically 7x5x3 cm, grayish yellow, irregu-lar surfaced tissue sample of the excised cyst sho-wed a cystic structure with 1-2 mm thick walls fil-led with a milky liquid. The histopathologicalstudy was carried out on hematoxylin and eosin(H&E)-dyed slices prepared from tissue samplestaken from the cyst wall after the follow-up. Largenumbers of lymphatic veins covered with flattenedendothelial cells, generally having smooth musclebundles in their walls and showing cystic expansi-on in places, were observed. In some areas of thevein walls, lymphoid aggregates and dystrophiccalcification were noted. The immunohistochemi-cal CD31 test showed immune reactivity in the en-dothelial cells. Based on the histomorphologicaland immunohistochemical data, a case of “calcifi-ed cystic lymphangioma” was diagnosed (Figure3).

DISCUSSION

Mesenteric cystic lymphangiomas (MCLs) occur atall ages, although most (65%) are present at birth.In general, 90% become symptomatic before thesecond year of life, and nearly 60% are diagnosedbefore the fifth year of life (5). MCLs are encoun-tered only rarely in surgical practice; the frequ-ency is less than 1 per 100,000 hospital admissi-ons (6).

Losanoff et al. (3) defined four different types ofMCLs: pedicled, sessile, retroperitoneal extended,

ALBAYRAK et al.

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FFiigguurree 11.. Computed tomography imaging of the abdomen showsa cystic lesion with sharp boundaries and lobular contours in themiddle abdominal region between the jejunal loop (arrow).

FFiigguurree 22.. Surgical findings with a lobulated cystic lesion withsoft consistency in the intestinal mesentery.

FFiigguurree 33.. Immunostaining showing immunoreactivity to CD31.

and multicentric. The etiology of lymphangiomasis still a matter of discussion. A well-establishedtheory suggests that lymphangiomas arise fromsequestrations of lymphatic tissue during embryo-nic development (1,7,8). On the other hand, Godarpostulated that premature lymphatics appear asmesenchymal slits, which coalesce and normallycommunicate with the venous system. Failure toestablish this communication may lead to alymphangioma (8). Fifty percent of cases involvethe head and neck, with only 10% occurring in in-ternal organs. Intra-abdominal cystic lymphangio-mas may arise from the retroperitoneum, the me-sentery and the visceral organs (2). There are onlya few case reports of mesenteric lymphangioma inadolescence (3,7).

The common presentations of intra-abdominalcystic lymphangiomas are abdominal mass anddistension, and most have cystic and septal com-ponents (2). Most MCL patients are initially asym-ptomatic, with vague and obscure abdominalsymptoms emerging early or late, depending onthe size and location of the cyst. The clinical symp-toms are protean and include pain, nausea, vomi-ting, or alterations in bowel habits (9). Our patienthad chronic stomach pains that had been ongoingfor eight months. Known complications that mayassociate with mesenteric cysts include torsion,

rupture, hemorrhage, intestinal obstruction due tovolvulus, extraneous pressure on nearby organs,and very rarely, malignant transformation – usu-ally sarcoma, although adenocarcinoma has alsobeen reported (10,11). To our knowledge, only sixmesenteric calcified cystic lymphangiomas havebeen reported previously (4). Ultrasonography andcomputed tomography are considered the most ap-propriate radiodiagnostic modalities to evaluatecysts of the mesentery, although usually ultraso-nography alone will suffice (2,12).

The differential diagnosis of abdominal cysticlymphangiomas must include other fluid-filled le-sions such as pseudocysts, dermoid cysts, entericduplications, lymphoceles, or neoplasms like me-sotheliomas, pancreatic tumors, lipomas, terato-mas, leiomyosarcomas, neurofibromas, or liposar-comas (13). The treatment of choice is radical exci-sion, since incomplete resection may lead to recur-rence (14). We completely excised the cystic lesionpresent in the intestinal mesentery of our patient.

Although mesenteric lymphangiomas are rare, es-pecially in adults, they should be considered as apossible cause of abdominal pain. Treatment issurgical with resection of the mass, sometimes inc-luding resection of the adjacent bowel. After sur-gery, recurrence rates are low and are usually theresult of incomplete excision.

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