Journal of Surgical Case Reports, 2018;5, 1–3

doi: 10.1093/jscr/rjy106
Case Report

CASE REPORT

Symptomatic lymphangioma of the adrenal gland: a

case report
Rémy Liechti1,*, Lana Fourie1, Stefan Fischli2, and Jürg Metzger1
1
Department of General and Visceral Surgery, Cantonal Hospital of Lucerne, Lucerne, Switzerland and
2
Department of Endocrinology, Cantonal Hospital of Lucerne, Lucerne, Switzerland
*Correspondence address. Department of General and Visceral Surgery, Cantonal Hospital of Lucerne, Lucerne, Switzerland. Tel: +41-79-824-9303;
E-mail: [email protected]

Abstract
Lymphangiomas (LAs) are rare benign tumors of the lymphatic vessels. In total, 95% of all reported LAs are located in the
head, neck and the mediastinum. LAs of the adrenal gland are very rare and currently, only ~54 cases have been reported in
literature. We present a case of a big left-sided adrenal LA. Abdominal imaging revealed a big cystic lesion in the left upper
abdomen of unknown origin. For diagnostic and therapeutic reasons we performed explorative midline laparotomy. The left
adrenal gland was found to be the origin of the cystic tumor. Hence, the patient underwent adrenalectomy in order to
remove the intact cystic lesion. Diagnosis was then confirmed by histological examination. With adrenal LAs being a very
rare entity, diagnosis is challenging and only little evidence exists on treatment options. We discuss diagnostic, therapeutic
and surgical approaches concerning such cases and provide an overview of the current literature.

INTRODUCTION CASE REPORT

Lymphangiomas (LAs) are rare benign tumors of the lymphatic A 32-year-old female patient was referred to our emergency
vessels. The etiology of LAs has not yet been clearly established. department complaining of progressive, colicky abdominal
They are believed to result from malformations of sequestered pain accompanied by intermittent nausea and vomiting during
lymphatic tissue. These lesions can be present at birth or occur at a period of more than 3 weeks. The pain was localized in the
any age. The most common sites of occurrence are head, neck epigastric region and radiated through to the back. In addition,
and the mediastinum accounting for 95% of all reported LAs [1]. the patient complained of generalized paresthesia as well as
The remaining 5% are located in the abdominal cavitiy, involving episodes of hot flashes and palpitations. There was no alter-
the mesentery of the small intestine, mesocolon, omentum, pan- ation in stool consistency or frequency. Her past medical his-
creas or adrenal gland. LAs of the adrenal gland are very rare and tory revealed no long-term medication or previous abdominal
currently, only approximately 54 cases have been reported in lit- operations.
erature [2, 3]. These lesions are usually detected as asymptomatic, On physical examination, the patient had normal vital signs.
incidental finding in patients undergoing an abdominal imaging Her abdomen was soft, non-tender and non-distended. Laboratory
procedure for other reasons. The diagnosis is very challenging investigations showed a slightly elevated white blood cell
and to date, the histological tissue analysis remains the only reli- count as well as C-reactive protein level. Beta-HCG test was
able tool to confirm the diagnosis of an adrenal LA. negative. Computed tomography scanning of the pulmonary
We present a case of a big left-sided adrenal LA causing arteries was performed in order to rule out pulmonary embol-
non-specific abdominal pain and symptoms. ism. The simultaneous display of the upper abdomen revealed

Received: February 15, 2018. Accepted: May 7, 2018

Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018.
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 2 | R. Liechti et al.

Figure 1: (A, B) Coronal and sagittal T2 weighted, fat suppressed MR image of the abdomen showing displacement of the spleen as well as the left kidney by the
adrenal tumor. (C) Intra-operative finding showing close proximity of the cystic lesion to the left colonic flexure. (D) Surgically excised cystic tumor together with the
left adrenal gland.

a big cystic lesion, which seemed to be attached to the left kid- At the 3-month follow-up, the patient presented with sleep
ney. Biochemical analysis and hormone testing showed no disorder, nausea and weight loss. The ACTH stimulation test
signs of a hyper functioning mass. Abdominal MRI revealed a showed a relative adrenocortical insufficiency while DHEA-S
maximum diameter of 12.5 cm. The origin of the cystic lesion levels remained normal. Consecutively the patient was treated
was suspected either in the mesentery or the omentum majus with hydrocortisone, as needed during stressful situations. The
(Fig. 1A and B). Considering the abdominal symptoms as well as further course remained uneventful (Fig. 1).
the fact that neither origin nor dignity could be determined in
this patient, a total surgical removal followed by histological
examination was indicated.
DISCUSSION
We performed an upper midline laparotomy. To ensure a
good anatomical overview, mobilization of the left colonic Among all of the reported cases, no single imaging modality
flexure was necessary and the omental bursa was entered. was able to correctly diagnose an adrenal LA. Similar to our
The distal part of the pancreas showed close proximity to the case, five adrenal LAs have even been characterized as a non-
cystic lesion and needed careful separation. After adhesiolysis adrenal lesion prior to the operation [3]. A recent review of the
between the splenic surface, the posterior gastric wall as well literature concerning the general management of LAs con-
as the parietal peritoneum, the left adrenal gland with its cluded that treatment options should be individualized
associated vein, was found to be the origin of the cystic tumor. depending on size, symptoms and anatomic localization of the
Hence total adrenalectomy was performed in order to remove tumor [4]. With adrenal LAs being a very rare entity, the utiliza-
the intact cystic lesion (Fig. 1C and D). Following a short period tion of treatment guidelines for adrenal incidentalomas seems
of postoperative nausea and vomiting, gradual return to a nor- to be appropriate. If the cystic adrenal lesion is clinically and
mal diet was possible. The patient was discharged on the sixth biologically inactive and its appearance remains unchanged
postoperative day. over a period of 18 months time, conservative management is
The histological examination of the specimen revealed an justified [5]. There is no evidence of reliable diagnostic value of
adrenal lymphangioma located in a normal structured adrenal preoperative biopsy or fine-needle aspiration for adrenal neo-
gland. plasms [6].

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 Symptomatic lymphangioma of the adrenal gland | 3

Adrenal masses with either suspicious imaging aspects or DISCLAIMER

size larger than 4 cm should be considered for resection due to
The views expressed in the submitted article are not an official
a substantial risk of malignancy [7].
position of the institution or funder.
Up until now, only one adrenal LA has been resected using a
retroperitoneoscopic approach and only three adrenal LAs have
been treated via laparoscopic adrenalectomy [2, 3]. Taking in REFERENCES
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The authors have no conflict of interest. 1013–8.

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