Rare case of retroperitoneal cystic lymphangioma in an adult

International Surgery Journal Tandup C et al. Int Surg J. 2019 Jun;6(6):2213-2215 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 DOI: http://dx.doi.org/10.18203/2349-2902.isj20192397 Case Report Rare case of retroperitoneal cystic lymphangioma in an adult Cherring Tandup*, Anish Chowdhury, Yashwant Raj Sakaray, Nasika Venkata Kanaka Naga Karthik Department of General Surgery, Post Graduate Institute of Medical Education & Research, Chandigarh, India Received: 19 March 2019 Accepted: 17 May 2019 *Correspondence: Dr. Cherring Tandup, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Cystic lymphangioma is benign tumor originating from the lymphatic vessels. Most commonly occurs in childhood and in the head or neck regions, retroperitoneal localization and presentations in adulthood are rare. Making a preoperative diagnosis is challenging, and only possible subsequent to the histological examination. En bloc resection is the recommended treatment for cystic lymphangioma, and recurrence occurs due to an incomplete excision. We here present a case of 40 year female presenting with progressive palpable lump associated with pain in right quadrant of abdomen, which on computed tomography revealed a large retroperitoneal cystic lesion probably lymphanigoma or cystic mesothelioma. Keywords: Lymphangioma, Cyst, Retroperitoneal, Adult INTRODUCTION Retroperitoneal lymphangiomas occurs due to malformations of retroperitoneal lymphatic systems are extremely rare condition affecting adult patients.1 Lymphangiomas being benign congenital malformation of lymphatic system are usually diagnosed in children and young adults and mostly located in head, neck and axilla where the loose connective tissue surrounding the structures allow lymphatic channels to expand and assume large proportions.2-4 Less than 5% of all lymphangiomas occur intra-abdominally and of them <1% occur in the retroperitoneum.5 When present intraabdominally they generally present as diffuse lymphangiomatosis involving multiple organs such as liver, kidney, spleen, GI tract mesentery , mediastinum, bones and soft tissue.1,7 Hence solitary lymphangioma of retroperitoneum in an adult is a very rare tumor and very few cases have been documented. With this report we would like to share our experience in managing a female adult with a giant cystic lymphangioma of the retroperitoneum complicated with kyphosis. CASE REPORT 40 year old female, known case of kyphosis of dorsolumbar spine presented with complain of progressively increasing lump in the right side of abdomen for the past one and half year associated with dull aching pain over right quadrant .The patient had history of caesarean 14 years back. On examination there was a palpable soft mass in the right hypochondrium reaching up to umbilicus and hypogastrium not moving with respiration. Infraumbilical midline scar of previous LSCS was noted. Ultrasonography of abdomen revealed large multiloculated cystic lesion in the sub-hepatic region extending upto the pelvis. Contrast enhanced CT showed a large smooth thin walled multiloculated cystic lesion in sub-hepatic space extending upto right iliac fossa and hypogastrium region (Figures 1-3). It measured 19.5cm×12.4cm×12.8cm. There was multiple thin International Surgery Journal | June 2019 | Vol 6 | Issue 6 Page 2213 Tandup C et al. Int Surg J. 2019 Jun;6(6):2213-2215 enhancing septa. No evidence of solid components or calcifications were seen. Superiorly it indented the liver and pushed the bowel loops anteromedially. multiple cysts ranging in size from 1-3cm). On microscopic examination multiple sections showed variably sized spaces lined by flattened cells enclosing pale eosinophilic lymph. The interstitial tissue showed paucicellular fibrocollagenous stroma with minimal lymphomononuclear inflammatory cell infiltrate and a few capillaries with occasional smooth muscle fibre. Figure 1: Contrast-enhanced CT axial scan shows infrahepatic retroperitoneal lobulated-septated cystic mass. Figure 3: Contrast-enhanced CT sagittal scan shows retroperitoneal lobulated-septated cystic mass extent from infrahepatic to hypogastrium region. Figure 2: Contrast-enhanced CT axial scan shows retroperitoneal lobulated large cystic lesion reaching upto right iliac fossa region. We performed exploratory laparotomy and en bloc resection of retroperitoneal lymphangioma (Figure 4). There was a large multiloculated cystic mass extending from infrahepatic region to right iliac fossa. It was attached to right perirenal fascia, infrahepatic, subhepatic region and ascending colon. Around 700 ml of pale straw colored fluid was aspirated and en bloc resection was done. Gross pathology revealed a well encapsulated mass measuring 19cm×11cm×17cm. Outer surface was lobulated and cut surface predominantly cystic (98% Figure 4: En bloc resection specimen of cystic lymphangioma. Following resection patient had an uneventful recovery. She was discharged on post op day 8 and is on regular follow up and currently is asymptomatic. DISCUSSION Lymphangiomas are generally considered to represent congenital malformation of lymphatic systems. Majority occurring relatively early in life. They develop as a result of lymphatic stasis due to congenital blockade or hypoplasia of regional lymphatic drainage.8 Isolated retroperitoneal lymphamgiomas are extremely rare tumor, International Surgery Journal | June 2019 | Vol 6 | Issue 6 Page 2214 Tandup C et al. Int Surg J. 2019 Jun;6(6):2213-2215 more so when diagnosed in an adult patient. Despite controversy surrounding the hamartomous, neoplastic or lymphangiectatic origin of this entity, general consensus points towards a benign etiology without any proven malignant potential. Retroperitoneal lymphangiomas are mostly diagnosed incidentally or presenting with non specific symptoms such as vague abdominal pain or mass in affected site. The pain may be due to compression of surrounding structure. Sudden acute pain with peritoneal signs is not an unusual presentation and Heralds cystic fluid leakage from the tumor.9 Typical USG or CT feature of lymphangioma is a cystic/multicystic intraabdominal mass with internal septations. However it may be difficult to come to an accurate preoperative diagnosis due to nonspecific nature of these findings. These findings may hold true for a myriad of other cystic conditions including but not limited to cyst adenocarcinoma, cystic teratoma, cystic mesothelioma, pseudomyxoma retroperitonii etc. although not done in our case MRI may offer better insight as this tumor is characterised by multilobulated heterogenous mass with low signal intensity in T1 and high signal intensity in T2 weighted images.10 Accurate diagnosis sadly can be made only by pathological examination. They are classified into three subtypes according to congenital dilated lymphatic channel: capillary (supermicrocystic), cavernous (microcystic) or cystic (macrocystic). Histologially they comprise lymphatic spaces lined by attenuated endothelium and filled with proteinaceous fluid (lymph) and may be unilocular or multilocular. Cystic lymphangiomas have the unique feature of having flat endothelial lining in place of cuboidal epithelium, lymphoid aggregates in thee cyst wall and presence of smooth muscle fibres. Retroperitoneal lymphangiomas are usually of cavernous or cystic type. Immunohistochemistry may show positive staining with CD31 or factor VIII related antigen but negative staining with cytokeratin. We did not performed IHC in our patient. Preoperative diagnosis is difficult due to the rarity of disease and other more common cystic lesions must be kept in mind while arriving at a diagnosis. Diagnostic FNAC is not indicated due to risk of bleeding from wall and septa and the chance of secondary seeding in case of hydatidosis. Surgical excision with en bloc resection is must to avoid recurrence. Other treatment modalities are not well established and enbloc resection should be attempted wherever possible. Owing to the high risk of recurrence patient must be followed up closely post-surgery. Clinical follow up and radiological investigations such as ultrasonography and CT scan are to be done at repeated intervals. Funding: No funding sources Conflict of interest: None declared Ethical approval: Not required REFERENCES 1. Stavropoulos M, Vagianos C, Scopa CD, Dragotis C, Androulakis J. Solitary hepatic lymphangioma a rare benign tumour: a case report. HPB Surg. 1994;8:33–6. 2. Bertino G, Ardiri A, Demma S, GiuseppeCalvagno S, Toro A, Basile E, et al. Rare benign tumors of the liver: still rare? J Gastrointest Cancer. 2014;45(2):202-17. 3. Asch MJ, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery. 1974;76(2):334–9. 4. Zhang YZ, Ye YS, Tian L, Li W. Rare case of a solitary huge hepatic cystic lymphangioma. World J Clin Cases. 2013;1(4):152–4. 5. Losanoff JE, Richman BW, El-Sherif A, Rider KD, Jones JW. Mesenteric cystic lymphangioma. J Am Coll Surg. 2003;196(4):598–603. 6. Gümüştaş OG, Sanal M, Güner O, Tümay V. Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge. Case Rep Pediatr. 2013;2013:292053. 7. Koh CC, Sheu JC. Hepatic lymphangioma: a case report. Pediatr Surg Int. 2000;16(7):515–6. 8. Enzinger FM, Weiss SW. Soft tissue tumours. 2nd edn. St Louis: CV Mosby Company; 1988: 614– 637. 9. Ishikawa H, Uchida S, Yokokura Y, Iwasaki Y, Horiuchi H, Hiraki M, et al. Nonparasitic solitary huge liver cysts causing intracystic hemorrhage or obstructive jaundice. J Hepatobiliary Pancreatic Surg. 2002;9:764–8. 10. Ayyappan AP, Jhaveri KS, Haider MA. Radiological assessment of mesenteric and retroperitoneal cysts in adults: is there a role for chemical shift MRI? Clin Imaging. 2011;35(2):127–32. Cite this article as: Tandup C, Chowdhury A, Sakaray YR, Karthik NVKN. Rare case of retroperitoneal cystic lymphangioma in an adult. Int Surg J 2019;6:2213-5. International Surgery Journal | June 2019 | Vol 6 | Issue 6 Page 2215