Retroperitoneal Cystic Lymphangioma Diagnosed by Endoscopic Ultrasound-Guided Fine Needle Aspiration

Abstract

Retroperitoneal cystic lymphangiomas are rare tumors of the lymphatic system. These tumors usually present in childhood and are often diagnosed incidentally with imaging procedures. Although benign, they can grow to large sizes and become symptomatic due to their compressive effects. They can cause diagnostic dilemmas with other retroperitoneal cystic tumors including those arising from the liver, kidney, and pancreas. Endoscopic ultrasound (EUS) has become an invaluable tool in the assessment of cystic lesions in the region of the pancreas. This case describes a 66-year-old female who presented with 3 months of abdominal pain. Radiographic imaging was suggestive of a cystic lesion in the region of the pancreas. EUS was performed confirming a cystic lesion adjacent to the tail of the pancreas with subsequent fine needle aspiration fluid analysis consistent with a cystic lymphangioma.

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INTRODUCTION
Cystic lymphangiomas are rare benign tumors of the lym-
phatic system. Ninety percentage of cystic lymphangiomas
are found in the neck and axillary regions.1 Retroperitoneal
lymphangiomas account for nearly 1% of all lymphangio-
mas.2 Although oen asymptomatic, they can present with a
palpable abdominal mass and nonspecific gastrointestinal
symptoms such as abdominal pain and nausea/vomiting. ey
are often found incidentally during diagnostic procedures
performed for unrelated clinical reasons.3 Approximately 90%
of retroperitoneal lymphangiomas are diagnosed in the rst 2
years of life; however, they can present at all ages and will of-
ten attain a large size prior to becoming symptomatic.4 ese
tumors rarely undergo spontaneous resolution and therefore
treatment is usually recommended.5 Although rare in incid-
ence, these lesions have been shown to be accurately diagnos-
ed with endoscopic ultrasound guided neneedle aspiration
Clin Endosc 2013;46:595-597
Copyright
©
2013 Korean Society of Gastrointestinal Endoscopy 595
(EUS-FNA).6
CASE REPORT
A 66-year-old female presented to her primary care physi-
cian for evaluation of 3 months of abdominal pain. Her pain
was associated with heartburn, bloating, and indigestion and
had been unresponsive to acid suppressing therapy. She de-
nied any nausea or vomiting but did note occasional diarrhea.
Further evaluation was performed with an abdominal ultra-
sound which revealed a 5 cm hypoechoic mass in the region
of the tail of the pancreas. She underwent a magnetic reson-
ance imaging (MRI) which conrmed the mass and noted it
to be cystic in nature with multiple thin septations (Fig. 1). e
patient was referred to gastroenterology and underwent EUS
with FNA of the cystic lesion. EUS identied a 4.7×3.3 cm cy-
stic lesion with internal septations adjacent to the tail of the
pancreas, but not within the pancreas itself (Fig. 2). White co-
lored uid was aspirated using a 19 gauge Cook Echotip FNA
needle and initial uid analysis was notable for lymphocytes.
A fluid triglyceride level was noted to be elevated at 8,243
mg/dL. e patient was referred to surgery and underwent
laparoscopic removal of the cystic lesion. Post resection, surgi-
cal pathology specimens were consistent with a cystic lymph-
angioma (Figs. 3, 4). At her 1 month postoperative visit, the
patient noted signicant improvement in her abdominal pain.
CASE REPORT
Retroperitoneal Cystic Lymphangioma Diagnosed by Endoscopic
Ultrasound-Guided Fine Needle Aspiration
Tyler Black1, Cynthia D. Guy2 and Rebecca A. Burbridge3
Departments of 1Internal Medicine, 2Pathology, and 3Gastroenterology, Duke University Medical Center, Durham, NC, USA
Retroperitoneal cystic lymphangiomas are rare tumors of the lymphatic system. ese tumors usually present in childhood and are oen
diagnosed incidentally with imaging procedures. Although benign, they can grow to large sizes and become symptomatic due to their
compressive eects. ey can cause diagnostic dilemmas with other retroperitoneal cystic tumors including those arising from the liver,
kidney, and pancreas. Endoscopic ultrasound (EUS) has become an invaluable tool in the assessment of cystic lesions in the region of the
pancreas. is case describes a 66-year-old female who presented with 3 months of abdominal pain. Radiographic imaging was suggestive
of a cystic lesion in the region of the pancreas. EUS was performed conrming a cystic lesion adjacent to the tail of the pancreas with sub-
sequent ne needle aspiration uid analysis consistent with a cystic lymphangioma.
Key Words:
Endosonography; Biopsy, ne-needle; Lymphangioma
Open Access
Received: December 3, 2012 Revised: January 21, 2013
Accepted: January 23, 2013
Correspondence: Tyler Black
Department of Internal Medicine, Duke University Medical Center, 2301 Er-
win Rd, Durham, NC 27710, USA
Tel : +1-334-714-4850, Fax: +1-919-681-6448, E-mail: tylerpblack@gmail.com
cc is is an Open Access article distributed under the terms of the Creative
Commons Attribution Non-Commercial License (http://creativecommons.org/
licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution,
and reproduction in any medium, provided the original work is properly cited.
Print ISSN 2234-2400 / On-line ISSN 2234-2443
http://dx.doi.org/10.5946/ce.2013.46.5.595

596 Clin Endosc 2013;46:595-597
Retroperitoneal Cystic Lymphangioma
DISCUSSION
e dierential diagnosis for a cystic lesion of the retrope-
ritoneum is broad and includes both benign and malignant
tumors including cystic mesothelioma, teratoma, undieren-
tiated sarcoma, malignant mesenchymoma, pancreatic pseu-
docyst, and lymphangioma.2 Retroperitoneal cystic lymphan-
giomas are rare tumors that are thought to arise due to an ab-
normal connection between the iliac and retroperitoneal ly-
mphatic sacs and the venous system, leading to lymphatic
stasis in the sacs.2 Retroperitoneal lymphangiomas are oen
classied into cystic and cavernous types.7 e cavernous type
is usually patent to adjacent lymph ow and therefore com-
pressible; whereas, cystic lymphangiomas are noncompress-
ible and may be uniloculated or multiloculated.4 An additional
third type, capillary lymphangioma, is rarely seen in retroperi-
toneal lymphangiomas. Preliminary imaging with ultrasound
can be useful given that it can demonstrate the cystic nature
of a lesion. Further imaging with computed tomography or
MRI can help further classify cysts as unilocular or multilocu-
lar, assess the relationship of the lymphangiomas to adjacent
organs, and further delineate the boundaries of the cyst.2 Most
retroperitoneal lymphangiomas are diagnosed incidentally
in asymptomatic patients, but complications such as severe ab-
dominal pain, hemorrhage, infection, torsion, rupture, and
obstruction can occur.8 A uid that appears grossly chylous
with a high triglyceride level is essentially diagnostic of a cystic
lymphangioma.8 Surgical excision is considered to be the tr-
eatment of choice for cystic lymphangiomas given their po-
tential to grow and develop complications.9 Alternative treat-
ments in the form of aspiration, radiotherapy, and scleroth-
erapy have been reported with variable results.4
e role of EUS-FNA is less dened with respect to rare cy-
stic diseases but has been evolving over the last decade. Just
as EUS has become invaluable in pancreatic lesions, it is also
benecial in nonpancreatic tumors of the retroperitoneum.
Imaging modalities can provide useful diagnostic informa-
tion but cannot determine whether a lesion is benign or ma-
lignant. e ability for cell sampling with EUS-FNA makes it
an important diagnostic modality in this regard and can help
Fig. 1. Magnetic resonance imaging abdomen showing cystic le-
sion in region of pancreas.
Fig. 2. Endoscopic ultrasound showing cystic lesion adjacent to
pancreas.
Fig. 3. H&E stain at ×100 magnication shows variably sized, di-
lated endothelial-lined spaces with a hypocellular, brovascular
connective tissue stroma, and collections of lymphocytes.
Fig. 4. Immunohistochemistry for D240, a marker for lymphatic
endothelium (×100).

Black T et al.
597
further guide subsequent therapeutic strategy. e perform-
ance characteristics of EUS-FNA have been shown to be quite
good with a specicity of 100% and accuracy of 86% previ-
ously reported in the literature.10 The risks associated with
EUS-FNA of these lesions are small, mainly bleeding, and in-
fection. Tumor seeding or leakage of lymphatic fluid with
EUS-FNA has been sparsely reported in the literature and sub-
stantial evidence for this complication is still lacking.11 Given
the low risk nature, diagnostic accuracy, and ability for cell
sampling, EUS-FNA should be considered a rst line modal-
ity in the evaluation of these cystic lesions of the retroperito-
neum.
Conflicts of Interest
e authors have no nancial conicts of interest.
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