Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Thursday, March 30, 2006

Retroperitoneal Lymphangioma

Martin Cherk, Mehrdad Nikfarjam and Christopher Christophi, Department of Surgery, University of Melbourne,
Austin Hospital, Victoria, Australia.

Lymphangiomas are rare cystic tumours that may present as cystic masses in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. Confusion with other cystic tumours of the retroperitoneum including those arising from the liver, kidney and pancreas is common. A case of a retroperitoneal cystic lymphangioma occurring in a 41-year-old woman raising interesting diagnostic and
management issues is reported. The patient presented with vague abdominal pain and persistent nausea. Radiological imaging demonstrated a large multiloculated thin-walled cyst involving the upper retroperitoneum. Surgical resection was complete, revealing a benign cavernous lymphangioma. The patient made a complete recovery and was disease-free 30 months postoperatively. [Asian J Surg 2006;29(1):51–4]

Key Words: cystic tumour, lymphangioma, retroperitoneum

Address correspondence and reprint requests to Professor Christopher Christophi, Department of Surgery, University of Melbourne, Austin Hospital, Lance Townsend Building Level 8, Studley Road, Heidelberg, Victoria 3084, Australia.


• Date of acceptance: 18 August 2004


An interesting and rare differential diagnosis for a retroperitoneal
cystic mass is cystic lymphangioma. A case of a patient
presenting with a multicystic mass in the retroperitoneum
that was identified as a lymphangioma is reported. These
tumours are commonly confused with other cystic masses in
the retroperitoneum. The particular diagnostic features and
treatment outcomes relating to retroperitoneal lymphangiomas
are discussed.

Case report

A 41-year-old woman presented with a 3-month history of
vague upper abdominal pain and persistent nausea. She had
no previous illness episodes. Colonoscopy and gastroscopy
were normal. Full blood count and liver function tests were
within normal limits. An ultrasound scan revealed a large
cystic mass (8 × 9 × 7 cm) in the region of the porta hepatis
(Figure 1A), which was thought to be extrahepatic. Computed
tomography (CT) showed a multiloculated cyst possibly arising
from outside the liver, compressing the adjacent liver and
extending medial to the second part of the duodenum towards
the pancreas (Figure 1B). Magnetic resonance imaging (MRI)
confirmed a cystic non-enhancing extrahepatic lesion (Figures
1C and D). Carcinoembryonic antigen and CA19-9 were not
elevated and hydatid serology was negative.

Preoperative diagnosis based on imaging investigations
could not be made with any certainty and a percutaneous
biopsy of the lesion was deemed too high-risk due to the
location and the possibility of malignant seeding if the lesion
was neoplastic.

A laparotomy was ultimately performed. A large retroperitoneal
multicystic tumour attached to the uncinate process
of the pancreas and coeliac axis and extending to the porta
hepatis between the inferior vena cava and portal vein was
identified. The cyst was excised intact, with a small portion of
attached pancreatic capsule and tissue.

The operation was made technically difficult by the retroperitoneal
location of the mass and its intricate relationship
with the pancreas and coeliac axis and its tributaries. The thinwalled
nature of the cyst rendered it liable to rupture and
required handling with extreme care.


Multiple cystic locules containing thin opaque fluid were
noted. The cyst lining comprised trabeculated white tissue
with a scattering of firm white and grey nodules within the
cyst wall. Histopathology revealed a mass with a central
cystic space and a multiloculated appearance, in part lined
by a flattened endothelium, with underlying small nodular
proliferations of smooth muscle. Large adjoining lymphoid
collections were identified (Figure 2). The cyst was adherent
in part to a small portion of pancreas tissue. Findings were
consistent with a retroperitoneal cavernous lymphangioma.


Recovery was uncomplicated. The patient was symptom-free
30 months postoperatively with no evidence of recurrence on
subsequent abdominal imaging.


The differential diagnosis of cystic tumour in the retroperitoneum
raises several possibilities. These include both malignant
and benign tumours. Malignant causes include necrotic
neoplasms, germ cell tumours (teratoma), undifferentiated
sarcoma, cystic metastases (gastric/ovarian), malignant
mesenchymoma, biliary cystadenoma/carcinoma and cystic
mesothelioma.1 Benign cystic lesions of the retroperitoneum
include lymphangioma, microcystic pancreatic adenoma and
cysts of urothelial and foregut origin. Cysts of foregut origin
are subdivided into bronchogenic cysts, which contain cartilage
or seromucinous respiratory glands, oesophageal cysts,
that are composed of well-developed layers of smooth muscle
without cartilage, and simple foregut cysts, which have none
of these distinguishing features.2

In the case presented, the unusual location of the cyst and
its intimate relationship with the liver contributes a myriad
of other diagnostic possibilities including simple liver cyst,
choledochal cyst, biliary cystadenoma/carcinoma, cystic hepatic
metastases, hamartomas and parasitic infections such
as hydatid disease.3

Lymphangiomas are extremely rare benign neoplasms of
the lymphatic system with no reports of incidence due to their
rarity. They can occur in any location in which lymphatics are
normally found. Head and neck lymphangiomas (cystic
hygromas) account for 75% of reported cases.4 The remainder
occur in the axillary region (20%) or in other less common
locations (5%). Retroperitoneal lymphangiomas account
for approximately 1% of all lymphangiomas, with 185 cases
identified in a review of international literature.5

The aetiology of these benign neoplasms is uncertain, with
most authors postulating a combination of inflammatory
and fibrotic processes or genetic predisposition. Other factors
implicated include mechanical pressure and retention, traumatic
factors, degeneration of lymph nodes and disorders
of endothelial lymphatic vascular secretion or permeability.

The frequent development within areas where primitive lymph
sacs occur suggests that lymphangiomas are malformations
arising from sequestrations of lymphatic tissue that fail to
communicate normally with the lymphatic system.6 This results
in the formation of unicystic or multicystic tumours
whose cavity is covered with a layer of endothelium and filled
with chylous or serous material.

Intra-abdominal lymphangiomas may arise from the
mesentery, greater omentum or retroperitoneum and tend to
grow slowly within the abdomen. The majority are asymptomatic
and are discovered incidentally on abdominal imaging
for other conditions. Symptoms are usually related to tumour
size. Approximately 40% of cases present with symptoms of
obstruction.7 Other symptoms include ascites and intermittent
fever. Rarely, bleeding or rupture of the tumour will cause
an acute abdomen.5 Clotting disorders have also been described
in patients with lymphangiomas.8 Acute symptoms
are more common in children than adults. The average time
from occurrence of symptoms to operation is more than 7

In contrast to mesenteric cysts, lymphangiomas not
only have the typical characteristics of the lymphatic system,
but also have smooth-muscle cells in their walls. Histologically,
lymphangiomas are classified as capillary, cavernous or cystic,
with only the latter two reported to occur in the retroperitoneal

Preoperative diagnosis of lymphangiomas is rare prior to
laparotomy or laparoscopy.5 Abdominal X-rays generally
show nonspecific expansion and, in some cases, foci of calcification
may be seen. Lymphangiography is seldom used but
is sometimes useful in preoperative diagnosis. CT and MRI
features of lymphangiomas have been described. The diagnosis
of lymphangioma based on these modalities is generally
one of many potential differentials for a multiloculated cystic
mass arising retroperitoneally. One of the main features of
retroperitoneal lymphangioma is that the mass is generally of
water density on CT or MRI.10 Guided biopsy of the lesion is
often difficult and rarely attempted due to the location of
tumours and concerns of potential dissemination of
malignancy. When fine-needle biopsy is performed, characteristic
abundant lymphocytes may be apparent.10 The likelihood
of preoperative diagnosis is greatest when imaging is
combined with biopsy.11

Outcomes following complete resection of retroperitoneal
lymphangiomas are generally good. Surgery is often required
for symptom control or diagnosis.12 Recurrence of
symptoms with incomplete excision is possible. Dissemination
in the retroperitoneum is very rare but potentially a
fatal complication.13 Injection of sclerosants such as alcohol
and bleomycin into lymphangiomas has been described
in the literature in nonsurgical candidates. However,
induration of the cyst and infection often complicate these

In conclusion, the differential diagnosis of a retroperitoneal
cystic lesion raises several possibilities. Cystic lymphangioma
should be considered. Despite being rare, these tumours
have an excellent prognosis, with symptom relief and cure
achieved with complete surgical excision.


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Asian Journal of Surgery