|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 380-381
Intra-abdominal cystic lymphangioma in an adult female masquerading ovarian tumor
Sankappa P Sinhasan1, KR Nagesha2
1 Department of Pathology, Indira Gandhi Medical College and Research Institute, Pondicherry, India
2 Department of Pathology, Hassan Institute of Medical Sciences, Hassan, Karnataka, India
|Date of Web Publication||18-Feb-2016|
Sankappa P Sinhasan
Department of Pathology, Indira Gandhi Medical College and Research Institute, Pondicherry
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sinhasan SP, Nagesha K R. Intra-abdominal cystic lymphangioma in an adult female masquerading ovarian tumor. Indian J Cancer 2015;52:380-1
Cystic lymphangioma is a rare congenital malformation of the lymphatics, which is found predominantly in children, and abdominal cystic lymphangioma (ACL) is far more extremely rare, especially in adult population. It is usually located in the neck (75%), axilla (20%), and other parts of the body (4-5%), and less than 1% of lymphangiomas affect the mesentery, greater omentum, and retroperitoneum. Although an ACL is considered benign, it may become locally invasive. It is important to distinguish ACL from other cystic neoplasms such as mesenteric cyst, enteric duplication cyst, peritoneal mesothelioma, ovarian cystic tumors, and retroperitoneal cystic neoplasms like cystic nephroma. Because of the overlap in imaging features of mesenteric or omental cysts and other cystic masses, histopathologic analysis is usually necessary to establish a diagnosis.
A 28-year-old woman came with complaints of progressive distension of abdomen and chronic pelvic discomfort. There was no history of anorexia or weight loss. On examination, her abdomen was distended. A vague soft, cystic mass was palpated in the lower abdomen. An abdominal ultrasound showed a giant cystic lesion with internal echogenic particles and septations of uncertain origin. Pre-operatively, the origin of the lesion could not be established and was suspected to be of ovarian origin. Provisional working diagnosis of ovarian cyst was made by the gynecologist and patient was posted for laparotomy. Intraoperatively, a huge multicystic lesion arising from small bowel mesentery was found. Uterus and both ovaries appeared normal. Both the kidneys were normal. Tumor was well encapsulated and was contained within the leaflets of the grossly distorted small bowel mesentery. Per-operatively, gynecologist requested assistance by the general surgeon, who did total excision of the cystic tumor by releasing adhesions to small bowel mesentery.
Grossly, the tumor was a huge, thin-walled, well-encapsulated cystic mass measuring 24 × 14 × 10 cm and weighting 650 g. Multiple cysts were seen scattered on the surface, giving it the bosselated appearance, showing congested blood vessels and entrapped mesenteric fat [Figure 1]. On cut open, the tumor revealed multiloculated, multi-septate cystic mass of cysts of various sizes filled with clear to straw-colored fluid [Figure 2]. Histopathologic examination revealed a lesion which was multilocular with cystic spaces of varying sizes that showed lining by single layer of attenuated endothelial cells. These cystic spaces were separated by thin to thick fibrocollagenous stroma showing lymphocytic infiltration with formation of lymphoid aggregates were seen [Figure 3]. The lumen of innumerable thin-walled channels was filled with proteinaceous, homogenous, eosinophilic material (lymph) with a few clusters of cyst macrophages. The histopathologic diagnosis of giant multilocular intra-abdominal cystic lymphangioma was given.
|Figure 1: A giant well-encapsulated tumor showing multiple cysts scattered over bosselated surface with entrapped mesenteric fat|
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|Figure 2: The cut section of tumor revealed multilocular cystic mass of cysts of various sizes filled with clear to straw-colored fluid|
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|Figure 3: Histopathology showing multilocular cystic tumor with fibrocollagenous stroma showing lymphocytic infiltrate; the cystic spaces lined by attenuated endothelial cells and showing lymph in the lumen (H and E, ×20)|
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Lymphangiomas are hamartomas of the lymphatic vessels that have the potential to infiltrate surrounding structures. About 50% are present at birth and up to 90% become evident by the age of 2 years. ACLs are more frequent in boys (5:2) with a mean age at presentation of 2 years. ACL can occur at any age and most are asymptomatic. In adults, symptoms and signs are commonly insidious in onset rather than acute. These cystic lesions can be discovered as an incidental finding during laparotomy for another condition, or they can manifest as a chronic or acute abdomen. Chronic symptoms include progressive abdominal distension and pain. Mimics of ovarian cystic masses include peritoneal inclusion cyst, paraovarian cyst, mucocele of the appendix, obstructed fallopian tube, spinal meningeal cyst, lymphocele, cystic degeneration of lymph nodes, lymphangioleiomyomatosis, hematoma, and abscess. However, the different types of cystic pelvic masses may have similar imaging appearances, and radiologic evaluation may be of limited diagnostic use. In doubtful cases, histopathologist can carry out immunohistochemistry stain like D2-40 antibody, which is a relatively newer marker for lymphatic endothelium.
| » Conclusion|| |
It is important to understand the relationship of a cystic intra-abdominal mass with its anatomic location, identify normal ovaries at imaging, and relate imaging findings to the patient's clinical history to avoid misdiagnosis. One should keep different histological entities in mind while operating intra-abdominal cystic masses of uncertain origin; a gynecologist or operating surgeon should always be prepared to perform a different surgery than planned, according to intraoperative findings. The ACLs are rare tumors that can present in adult life occasionally and masquerade as a malignant gynecologic lesion. This case may add to host of postulations related to rare intra-abdominal mesenteric lymphatic cysts.
| » References|| |
Kim JH, Ryu WS, Min BW, Song TJ, Son GS, Kim SJ, et al
. Acquired omental cystic lymphangioma after subtotal gastrectomy: A case report. J Korean Med Sci 2009;24:1212-5.
Kumar S, Agrawal N, Khanna R, Khanna AK. Giant lymphatic cyst of omentum: A case report. Cases J 2009;2:23.
Stoupis C, Ros PR, Abbitt PL, Burton SS, Gauger J. Bubbles in the belly: Imaging of cystic mesenteric or omental masses. Radiographics 1994;14:729-37.
Mohite PN, Bhatnagar AM, Parikh SN. A huge omental lymphangioma with extension into labia majorae: A case report. BMC Surg 2006;6:18-20.
De Perrot M, Rostan O, Morel P, Le Coultre C. Abdominal lymphangioma in adults and children. Br J Surg 1998;85:395-7.
[Figure 1], [Figure 2], [Figure 3]
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