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Urological Science 25 (2014) 112e114

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Urological Science

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Case report

Adrenal cystic lymphangioma: A case report and review of theliterature

Ting-Po Lin a, Marcelo Chen a, Chi-Kuan Chen b, Jong-Ming Hsu a, Wun-Rong Lin a,*

aDepartment of Urology, Mackay Memorial Hospital, Taipei, Taiwan, ROCbDepartment of Pathology, Mackay Memorial Hospital, Taipei, Taiwan, ROC

Open access under CC BY-NC-ND license.

a r t i c l e i n f o

Article history:Received 5 July 2013Received in revised form25 September 2013Accepted 12 November 2013Available online 12 February 2014

Keywords:adrenal cystcystic lymphangiomapathology

* Corresponding author. Department of Urology,Number 92, Section 2, Jhongshan North Road, JhongsTaiwan, ROC.

E-mail address: cedric305@yahoo.com.tw (W.-R. L

1879-5226 Copyright � 2014, Taiwan Urological Assohttp://dx.doi.org/10.1016/j.urols.2013.11.006

a b s t r a c t

A 37-year-old female was being evaluated for fever when a huge cystic lesion of about 9 � 10.5 cm2 insize in the right adrenal fossa with internal septal structures containing spots of calcification was foundincidentally on computed tomography. A right adrenal cyst was suspected, and right adrenalectomy andsurgical resection of the lesion were then performed. The pathology showed multilocular spaces lined byflat cells, compatible with adrenal cystic lymphangioma. Immunohistochemically, the tumor wasstrongly positive for D2-40 and CD31. She received regular imaging follow-up, and no metastatic diseasehas been found until now. The radiological and pathological features of adrenal cysts and the treatmentstrategy for adrenal lymphangiomas are discussed.Copyright � 2014, Taiwan Urological Association. Published by Elsevier Taiwan LLC.

1. Introduction

Lymphangiomas are benign vascular lesions that are known tooccur throughout the whole body. Adrenal lymphangiomas (alsoknown as cystic adrenal lymphangiomas) are extremely rare. Theywere first reported in 1965, and currently less than 50 cases havebeen reported in the English literature.1 They are most often foundincidentally as a large cysticmass, and surgical removal is necessaryto rule out other types of adrenal neoplasms. Herein, we report apatient with a right adrenal cystic lymphangioma and review theradiological and pathological features of the tumor.

2. Case report

A 37-year-old Taiwanese female presented with fever off and onfor 3 days. A physical examination showed RUQ (right upperquadrant) knocking tenderness, and abdominal echo revealed ahuge cystic lesion with septation and a honeycomb appearance(Fig. 1). Abdominal computed tomography (CT) demonstrated ahuge cystic lesion of about 9 � 10.5 cm2 in size in the region be-tween the right adrenal gland and the liver. Subtle internal septalstructures with spots of calcification were seen (Fig. 2). Blood

Mackay Memorial Hospital,han District, Taipei City 104,

in).

ciation. Published by Elsevier Taiw

tests were all in the normal range, including a cortisol level of10.87 mg/dL (normal range 5e25 mg/dL), ACTH (adrenocorticotropichormone) 20.30 pg/mL (normal range 5e77 pg/mL), plasma reninactivity 0.13 ng/mL/h (normal range 0.15e2.23 ng/mL/h), aldoste-rone 3.91 ng/dL (normal range 0.75e15.00 ng/dL), 24-hour urinaryexcretion of vanillylmandelic acid 2.7 mg/d (normal range 1.0e7.5mg/d), 24-hour urinary excretion of epinephrine<3 mg/d (normalrange <22.4 mg/d), 24-hour urinary excretion of normetanephrine20.5 mg/d (normal range 11.1e85.5 mg/d), and 24-hour urinaryexcretion of dopamine 168.6 mg/d (normal range 50e450 mg/d).

The patient received right adrenalectomy and tumor excision inJuly 2011. On pathological examination, the tumor was grossly amulticystic tumor measuring 10 � 9.8 � 7 cm3 in size (Fig. 3). Theinner surface was smooth and glistening, and the cysts had a serousfluid or colloid content with yellowish-orange spots in the cysticwalls. There were also foci of hemorrhage and fibrosis. Micro-scopically, sections of the tumor revealed the features of a cysticlymphangioma composed of multilocular spaces lined by flat cells(Fig. 4). Immunohistochemically, the tumor was strongly positivefor D2-40 and CD31 (Fig. 5), and the histopathological diagnosiswas a cystic lymphangioma.

The patient was discharged from the hospital after 10 dayswithout perioperative complications. Follow-up CT scans at 6 and 9months showed a 2.9 � 2.4 cm2 nonenhanced cystic lesion in theright adrenal fossa (Fig. 6). A subsequent CT scan 2 years aftersurgery revealed that this lesion had decreased in size to2.2 � 1.3 cm2. Postoperative seroma formation was favored, and nometastatic disease was found.

an LLC. Open access under CC BY-NC-ND license.

Fig. 1. Abdominal echo: a huge cystic lesion with septation.

Fig. 3. Surgical specimen: a multicystic tumor measuring 10 � 9.8 � 7 cm in size.

Fig. 4. Sections show adrenal gland with dilated cystic spaces, lined by flat cells, andcontaining amorphus hypocellular fluid.

T.-P. Lin et al. / Urological Science 25 (2014) 112e114 113

3. Discussion

Lymphangiomas are benign malformations of vessels. They areknown to occur throughout the whole body, although most of thelesions are located in the neck, axilla, and mediastinum.2 Occa-sionally, they are found in the abdominal cavity (5%), including themesentery of the small intestine, omentum, mesocolon, or retro-peritoneum.1 The tumors can occur at all ages, and have a peakincidence from the third to sixth decades of life.3 The pathogenesisof adrenal lymphangiomas is still unclear; however, the mostfavored theory is abnormal development and/or ectasia oflymphatic vessels.1

Adrenal cysts are uncommon, with a reported incidence of0.06e0.18%.1 Barron and Emanuel4 categorized adrenal cystic le-sions into pseudo-, epithelial, endothelial, and parasitic cysts. CTfindings of cystic lesions vary. Pseudocysts are generally suprarenalmasses with mixed density, central necrosis, or heterogeneousenhancement. Simple endothelial cysts such as lymphangiomasappear as thin-walled, fluid-density lesions on CT; they are usuallyhypodense and nonenhancing lesions. However, the radiologicalfeatures cannot fully distinguish lymphangiomas from malignantor functional lesions such as adrenal cortical carcinoma orpheochromocytoma.

Histologically, adrenal lymphangiomas are characterized bymultiloculated cystic cavities.5 The cysts are lined by a single layer

Fig. 2. Computed tomography scan of the abdomen: cystic mass in the right-sideretroperitoneum. Fig. 5. The flat lining cells are strongly positive for D2-40.

Fig. 6. Computed tomography scan of the abdomen 6 months after surgery shows a2.9 � 2.4 cm, non-enhanced cystic lesion at the right adrenal fossa.

T.-P. Lin et al. / Urological Science 25 (2014) 112e114114

of flattened endothelial cells, and endothelial atypia or degenera-tive changes are uncommon. Previous reports have not shown anyevidence of associated adrenal hyperplasia, adrenal adenoma, ad-renal cortical adenoma, or pheochromocytoma.1 Immunohis-tochemically, cells with an endothelial liningmostly stain positivelyfor CD31, factor VIII, CD34, and D2-40. CD31, factor VIII, and CD34label both the vascular and the lymphatic endothelium. D2-40 is acommercially available monoclonal antibody directed against hu-man podoplanin, a transmembrane mucoprotein that is expressedin lymphatic endothelial cells.6 It has emerged as an immunore-agent expressed by a variety of different neoplasms such as lym-phangiomas, Kaposi sarcomas, hemangioendotheliomas, adrenalcortical carcinomas, epithelioid mesotheliomas, and seminomas.6

In the current case, it served as a specific marker to distinguishan angiomatous from a lymphangiomatous cyst.

Adrenal lymphangiomas are usually asymptomatic; however,nonspecific symptoms related to mass effect such as fever,

abdominal pain, or palpable mass may occur. Functional lesions inendothelial cysts are not common.1,7 The management strategy isinfluenced by three factors: (1) functional status of the cyst; (2) thepossibility of incidental malignancy; and (3) potential complica-tions such as hemorrhage or infection.8 Neri and Nance9 reported a7% incidence of malignancy in their review of over 600 cases ofadrenal cysts from the literature. Thus, surgical removal is generallynecessary to rule out other types of adrenal neoplasms.1 Fine-needle aspiration of fluid can be an alternative therapy if the cystis symptomatic and nonfunctional, and raises a very low suspicionof malignancy.9,10

Conflicts of interest

The authors declare that they have no financial or non-financialconflicts of interest related to the subject matter or materials dis-cussed in the manuscript.

Sources of funding

No funding was received for the work described in this article.

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