A 34-year-old patient was scheduled for valve replacement to treat a symptomatic mitral regurgitation. The preoperative work-up incidentally discovered an intra-abdominal cystic tumour extending from the epigastrium to the pelvic region on a computed tomography scan. The patient had no abdominal symptoms by the giant cyst from unkown origin. An open “en bloc” resection disclosed a large cyst in the mesocolon.  Pathological examination, including immunohistochemistry, enabled the diagnosis of a mesenteric cystic lymphangioma.  Long-term follow-up of 12 months shows no recurrence. Mesenteric cystic lymphangioma, which is extremely rare in adults, is a challenge to diagnose and needs complete resection to ensure dignity and to avoid recurrence

Breitenstein, S

Clavien, P A

Emmert, M Y

Kambakamba, P

Lesurtel, M

Wilhelm, M J

Journal of Surgical Case Reports

P. Kambakamba

M. Lesurtel

S. Breitenstein

M. Emmert

M. Wilhelm

P. Clavien

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Giant mesenteric cystic lymphangioma of mesocolic origin in an asymptomatic adult patient

ZORA

Zurich Open Repository andArchiveUniversity of ZurichMain LibraryStrickhofstrasse 39CH-8057 Zurichwww.zora.uzh.chYear: 2012Giant mesenteric cystic lymphangioma of mesocolic origin in anasymptomatic adult patientKambakamba, P; Lesurtel, M; Breitenstein, S; Emmert, M Y; Wilhelm, M J; Clavien, P AAbstract: A 34-year-old patient was scheduled for valve replacement to treat a symptomatic mitral regur-gitation. The preoperative work-up incidentally discovered an intra-abdominal cystic tumour extendingfrom the epigastrium to the pelvic region on a computed tomography scan. The patient had no abdomi-nal symptoms by the giant cyst from unkown origin. An open “en bloc” resection disclosed a large cystin the mesocolon. Pathological examination, including immunohistochemistry, enabled the diagnosis ofa mesenteric cystic lymphangioma. Long-term follow-up of 12 months shows no recurrence. Mesentericcystic lymphangioma, which is extremely rare in adults, is a challenge to diagnose and needs completeresection to ensure dignity and to avoid recurrence.Posted at the Zurich Open Repository and Archive, University of ZurichZORA URL: http://doi.org/10.5167/uzh-72855Originally published at:Kambakamba, P; Lesurtel, M; Breitenstein, S; Emmert, M Y; Wilhelm, M J; Clavien, P A (2012). Giantmesenteric cystic lymphangioma of mesocolic origin in an asymptomatic adult patient. Journal of SurgicalCase Reports, (6):online.JSCRJournal of Surgical Case Reportshttp://jscr.co.ukGiant mesenteric cystic lymphangioma of mesocolic originin an asymptomatic adult patient Authors: P Kambakamba, M Lesurtel, S Breitenstein, MY Emmert, MJ Wilhelm & PA ClavienLocation: University Hospital of Zurich, Zurich, Switzerland.Citation: Kambakamba P, Lesurtel M, Breitenstein S, Emmert MY, Wilhelm MJ, Clavien PA.Giant mesenteric cystic lymphangioma of mesocolic origin in an asymptomatic adult patient.JSCR 2012. 6:4ABSTRACTA 34-year-old patient was scheduled for valve replacement to treat a symptomatic mitralregurgitation. The preoperative work-up incidentally discovered an intra-abdominal cystictumour extending from the epigastrium to the pelvic region on a computed tomography scan.The patient had no abdominal symptoms by the giant cyst from unkown origin. An open “enbloc” resection disclosed a large cyst in the mesocolon.  Pathological examination, includingimmunohistochemistry, enabled the diagnosis of a mesenteric cystic lymphangioma. Long-term follow-up of 12 months shows no recurrence. Mesenteric cystic lymphangioma,which is extremely rare in adults, is a challenge to diagnose and needs complete resection toensure dignity and to avoid recurrence. INTRODUCTIONLymphangiomas are malformations of the lymphatic drainage system and mostly occur inchildhood. The localization of these cystic tumours is mainly the cervical or axillary region; inaddition there are rare reports regarding their occurrence in the mediastinum, in theretroperitoneum as well as in the abdominal cavity appearing as mesenteric cysts. With anestimated incidence of 1/1000000 and fewer than 200 cases described in literature themesenteric form in adults represents a very rare entity. Here, we present one case of a giantmesenteric cystic lymphangioma in an asymptomatic patient CASE REPORTA 34-year-old patient was scheduled for minimal invasive mitral valve reconstruction to treat aBarlow syndrome with a prolapse of the posterior mitral leaflet. The patient’s medicationconsisted of a Proton pump inhibitor due to a chronic gastro-esophagial reflux disease.Otherwise the patient had no other digestive symptoms, in particular there was no history ofpancreatic disease.  Preoperative computed tomography (CT) incidentally showed a hugeabdominal cystic lesion of 34x17x25cm (Fig. 1). page 1 / 4JSCRJournal of Surgical Case Reportshttp://jscr.co.ukEchinoccus serology was negative and the aspiration ofthe fluid showed normal levels of amylase and no cytological signs of malignancy. Alaparoscopic resection was attempted; however, because of lack of exposure due to the largesize of the cyst, a conversion to a median laparotomy was necessary. After emptying of thecyst by aspiration of 3 litres of fluid, an “en bloc” resection of the cyst, originating from themesocolon, was performed (Fig. 2). The postoperative course was uneventful and the patientwas discharged at postoperative day 5. Macroscopic examination found a unilocular cyst withfibrotic wall. Microscopically, prominent giant cells and xanthomatous inflammation reactionwere present in the wall. Further light brown histiocytes with focal expression of CD31 andD2-40 were found in the specimen (Fig. 3), favouring lymphangioma with cystic-regressivechanges. No signs of malignancy were found. After a short period ofrecovery, the required mitral valve reconstruction was successfully performed by a minimalinvasive approach. A 1-year follow-up examination including abdominal ultrasonographyshowed no recurrence. DISCUSSIONpage 2 / 4JSCRJournal of Surgical Case Reportshttp://jscr.co.ukThree types of lymphagniomas are distinguished in the literature: the cavernouslymphangioma, the lymphangioma simplex, and the cystic lymphangioma (1). The cystic formof a lymphagioma was described for the first time in 1828 by Redenbacker (2). Cysticlymphangiomas are very rare and mainly occur during childhood;  in 90% of the cases, theyare diagnosed before 2 years of age (1,3,4). The estimated incidence of cystic lamphagiomasis approximately one per 20,000 admissions to pediatric hospitals (5,6). Only very few reportsexist regarding cystic lymphangioma in adults (7). Pathogenesis remains unclear. The mostaccepted theory is based on a lymphatic congestion due to interrupted communication of thedraining lymphatic system. However, this does not explain the occurrence of these cystictumours in the adult population. Therefore, some authors believe that adult manifestations arethe result of a delayed proliferation of congenital or acquired lymphoid nests after stimuli suchas respiratory infection or local trauma (8). Our patient did indeed have a long history ofcombat sports. The clinical presentation of mesenteric lymphatic cysts as described in thepresent case is variable. Unspecific abdominal symptoms such as pain, distension, nauseaand vomiting, diarrhoea, constipation, and weight loss are most common. Different imagingmodalities have been used to diagnose cystic intra-abdominal tumours. Some studiesdemonstrated ultrasonography as being superior compared with CT regarding theassessment of intracystic structures such as the presence of echogenic contents, a thicknessof capsule, and intracystic septation, which may indicate internal bleeding or infection. Incystic lymphangioma, the fluid content is usually described as serous with a density similar towater (Houndfield unit (HU) = 0). Differential diagnosis of intraperitoneal cystic massesincludes pancreatic pseudocysts, echinococcal cyst, enteric duplication cysts, cysticmesotheliomas and ascites.  Abdominal CT in our patient revealed a density of 18 HU.Ecchinococcus serology was negative as well as the bacteriological and cytological analysisof the aspirated fluid. Complete surgical resection of lymphangioma remains the treatment ofchoice in order to reveal symptoms and to prevent recurrences.  While aspiration of cysticfluid decreases the size of the cyst for a short time only and has the risk of infection,incomplete excision of lymphangiomas carries a risk of tumour-recurrence ranging from0-13.6% (9). However, a malignant degeneration was neither seen in the present case, nordescribed in the literature. A laparoscopic approach to resect mesenteric cysticlymphangioma has been described in selected cases. In our case, a lack of exposure andimpaired visibility caused early conversion to a median laparotomy. Histological analysisconfirmed the total resection of the lesion in our case. Specific immunohistochemicalmarkers for lymphatic endothelial cells have been used such as lymphatic vessel endothelialreceptor 1, vascular endothelial growth factor receptor 3, and Prox-1. In addition to CD31, amore recent monoclonal antibody D2-40, specifically binding lymphatic endothelium, wasapplied by our pathologist (10). In summary, mesenteric cystic lymphangioma is extremely rarein adults but must be considered as a differential diagnosis of abdominal cystic lesions.Complete surgical resection provides definitive histological diagnosis, and prevents recurrence.REFERENCES1. Yildirim E, Dural K, Kaplan T, Sakinci U: Cystic lymphangioma: report of two atypicalcases. Interact Cardiovasc Thorac Surg 2004 , 3:63-652. John G. Park, MD, Marrie- Christine Aubry, MD, John A. Godfrey, MD and David E.page 3 / 4JSCRJournal of Surgical Case Reportshttp://jscr.co.ukMidthun, MD. Mediastinal Lymphangioma: Mayo Clinic Experience of 25 cases. MayoClinic Proceedings. 2006 Sep, vol 81, No.9. 1197-12033. Losanoff JE, Kjossev KT. Mesenteric cystic lymphangioma: unusual cause ofintra-abdominal catastrophe in an adult. Int J Clin Pract. 2005 Aug;59 (8):986-7 4. Gelal F, Yucel K, Tugsel E, Guney S: Axillary cystic lymphangioma presenting inpregnancy. Turk J Med Sci 1998, 28:571-5725. Chung MA, Brandt ML, St-Vil D, Yazbeck S. Mesenteric cysts in children. J Pediatr Surg1991; 26:1306-8 6. Vanek VW, Phillips AK. Retroperitoneal, mesenteric, and omental cysts. Arch Surg1984;119:838-427. Cutillo DP, Swayne LC, Cucco J, Dougan H. CT and MR imaging in cystic abdominal. JComput Assist Tomogr. 1989, 13(3):534-68. David Guinier, M.D., Pierre O. Denue, M.D., George A. Mantion, Ph.D Intra-abdominalcystic lymphangioma. The American Journal of surgery. 2006, 191(5):706-7079. Prashant N Mohite , Ashok M Bhatnagar and Shailesh N Parikh. A huge OmentalLymphangioma with extention into Labia Majorae: A case report. BMC Surg. 2006, 6:1810. Yonemura Y, Endou Y, Tabachi K, Kawamura T, Yun HY, Kameya T, Hayashi I, BandouE, Sasaki T, Miura M: Evaluation of lymphatic invasion in primary gastric cancer by a newmonoclonal antibody, D2-40. Hum Pathol 2006 , 37:1193-1199Powered by TCPDF (www.tcpdf.org)page 4 / 4

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Giant mesenteric cystic lymphangioma of mesocolic origin in an asymptomatic adult patient

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