Introduction
Lymphangiomas are rare benign tumors. They are preferentially located in the head,
neck, and axilla in children.[1]
However, lymphangiomas in the peritoneal cavity are extremely rare, particularly in
adults. In the abdomen, lymphangiomas occur most commonly in the mesentery, followed
by the omentum, mesocolon, and retroperitoneum. The etiology is unclear, but they
are considered primarily to congenital in origin. [2]
Preoperative diagnosis is often difficult due to the frequent silent clinical course.
Radiological investigations are a useful diagnostic tool, but definitive diagnosis
is confirmed by histopathology after a complete surgical resection. [3]
The report describes five cases of adult patients with mesenteric lymphangioma.
Case report N.1
A 67-year-old female presented with a dull abdominal pain in the right hypochondrium
and flank of a 1-month duration and presence of constipation.
An abdominal examination revealed distension, hyperactive bowel sounds and tenderness
during palpation in the central abdominal quadrant and right flank.
An ultrasonographic abdominal scan revealed the presence of a cystic mass (max diameter
32 mm) in the retrocecal adipose tissue.
A computed tomography (CT) abdomen scan confirmed the presence of the cystic lesion
and its dissociability from the cecum, appendix and ileum. In addition, the CT scan
revealed the presence of multiple ipodense formations in the body and tail of pancreas.
Neoplastic markers (AFP, CEA, CA 19-9, CA 15-3, CA125, TPS) were negative.
During the hospital stay an abdominal MR was performed. The exam revealed in the retrocecal
adipose tissue the presence of a fluid-filled formation with a diameter of 35 mm that
appeared indissociable from the cecum for the presence of a tissutal connection (Figure
1 A – B). Other cystic lesions (communicating with pancreatic duct) were present in
the whole pancreas and in the right kidney.
Figure 1
MRI of the abdomen. Axial (a) and coronal (b) T2-weighted TSE images showed the presence
of a fluid-filled cystic lesion with a diameter of 35 mm in the retrocecal adipose
tissue.
The patient underwent exploratory surgery: at laparotomy a yellowish ovalar formation
with a diameter of 6 cm was found arising from the mesentery of ileum with partial
adhesion of the appendix. The lesion had a cystic aspect and was fluid-filled. We
performed a total excision of the lesion and appendicectomy. Histopathological examination
was consistent with the diagnosis of mesenteric lymphangioma. The patient has been
followed-up for 6 months and no recurrence occurred.
Case report N.2
A 68-year-old male patient presented with a painless abdominal swelling of two years
duration. A clinical examination revealed the presence of an abdominal mass extending
from the right hypocondrium to the right iliac fossa.
Abdominal ultrasonography and CT scans revealed the presence of an ipodense and fluid
– filled formation, 10 cm in diameter which extended from the right hepatic lobe to
the ascending colon. Posteriorly the mass bordered the anterior aspect of the right
kidney. In addition, a left kidney agenesia was present.
An abdomen MR was also performed: presence of a 10-cm fuid – filled mass with internal
septa that extends from the hepatic hilum to the right colon, displacing medially
the second duodenal portion and anteriorly the right colonic flexure. At MR this formation
presented a strict contiguity with the common bile duct (Figure 2 A – B).
Figure 2
MRI of the abdomen. Axial (a) and coronal (b) T2-weighted TSE images showed the presence
of a huge fluid-filled mass with internal septa located in the hepato-renal space
extending from the hepatic hilum to the right colon and displacing medially the second
duodenal segment and anteriorly the hepatic flexure of the colon.
In consideration of the clinical symptoms, we performed a laparotomic exploration.
At laparotomy we found an ovalar formation of cystic aspect, 10 cm in diameter, which
was closely adherent to the biliary tree. The dissection was conducted closely the
CBD and the mass appeared originating from the cranial extremity of the common hepatic
duct. The complete excision determined a microperforation of the biliary tree that
was repaired with a reabsorbable suture. An abdominal drainage was positioned and
it was removed in the 7 th postoperative day.
A citologic examination was conducted on the fluid aspirated from the cyst and it
revealed the presence of chronic inflammatory cells. The histopathological examination
showed the presence of dilated lymphatic vessels and endothelial proliferation: this
pattern was consistent with the diagnosis of cystic emolymphangioma.
Case report N.3
A 80 – year – old male patient complained of diffuse abdominal pain. A clinical examination
revealed an occlusive state. We performed an abdominal ultrasonography and CT that
revealed in left hypocondrium the presence of an anechogenic ovalar formation of 8,6
x 4 x 5 cm, with internal septa and a thin wall, with polycyclic edge . This formation
appeared adherent to the gastric body and the jejunal loops.
Neoplastic markers (AFP, CEA, CA 19-9, CA 15-3, CA125, TPS) were negative.
The patient underwent a laparotomic exploration and the cystic formation was completely
excised. (Figure 3)
Figure 3
Cystic lymphangioma with polycyclic edge and internal septa (intraoperative specimen)
The histopathological examination was consistent with the diagnosis of mesenteric
cystic lymphangioma. (Figure 4)
Figure 4
Numerous dilated lymphatic spaces, sometimes filled with lymph (EE 5X). Inset: focally
lymphatic spaces are covered by plump endothelial cells (EE 20X)
The patient has been followed-up for 4 months and no recurrence occurred.
Case report N.4
A 77 – year – old male patient complained of a dull abdominal pain of one year duration
localized in the periumbilical region. This pain was associated to abdominal swelling
and early satiety after meals.
An abdominal examination revealed the presence of a solid mass that occupied the epigastric
and mid-epigastric region. An abdomen CT showed a huge mass composed by multiple confluent
cystic lesions displacing inferiorly the small bowel. (Figure 5 A - B)
Figure 5
CT of the abdomen. Axial (a) and MPR coronal (b) CT images showed a huge mass composed
by multiple confluent cystic lesions occupying the peritoneal spaces and displacing
inferiorly the small bowel.
At laparotomic exploration, the mass appeared composed by multiple cysts containing
a citrine fluid. A careful dissection was conducted to excise the mass without spillage
of fluid in peritoneal cavity.
The histopathological examination confirmed the diagnosis of mesenteric cystic lymphangioma.
The post-operative course showed no complications. The patient has been followed up
for 1 year and no recurrence was observed.
Case report N.5
A 65-year-old male patient was diagnosed ten years before the presence of an intra-abdominal
limphangyoma. The mass was followed-up with annual abdominal – MR and ecotomography.
At abdominal – RM the formation was fluid- filled with internal septa and measured
18x12 cm : it was placed between the posterior aspect of the stomach and the pancreas
with lateral extension in the hepatorenal space.(Figure 6) He had no clinical symptoms
but due the growing of the mass in the last years (12x8 cm in 2008) we performed a
laparotomic resection of the tumor. A citologic examination was conducted on the fluid
aspirated from the cyst and it revealed the presence of chronic inflammatory cells.
The histopathological examination was consistent with the diagnosis of cystic lymphangioma.
Figure 6
MRI of the abdomen. Fluid - filled mass (18x12 cm) with internal septa located between
the stomach and the pancreas with lateral extension in the hepatorenal space.
Discussion
Intra-abdominal mesenteric lymphangioma is an uncommon tumor that is usually manifesting
in early adulthood. This tumor has an incidence of approximately 1/20,000 and 1/250,000
hospital admissions, and it is more frequent in men than women with a M/F ratio of
3:1. [4]
Intra-abdominal lymphangioma are almost always found in the retroperitoneum, followed
by mesentery. In intraperitoneal sites, the small bowel mesentery (70%) is the commonest
site, with 50–60% of all cysts located in the ileal mesentery. [5]
The etiology of mesenteric lymphangioma is considered to be congenital, with abnormal
embryonic development of the lymphatic system causing sequestration of lymphatic tissue.
However, other possible causes have been suggested such as: abdominal trauma, lymphatic
obstruction, inflammatory process, surgery, and/or radiation therapy. [6]
Data relative to our case series are listed in Table 1: we performed the surgical
excision of a mesenteric lymphangioma in five patients (4 male and 1 female) with
a median age of 71.4 years (65 –80).
Table 1
Patient characteristics
Patient n. 1
Patient n. 2
Patient n. 3
Patient n. 4
Patient n. 5
Sex
F
M
M
M
M
Age
67
68
80
77
65
Symptoms
Abdominal pain
Abdominal swelling
Intestinal obstruction
Abdominal pain and early satiety
Asymptomatic
Diameter (cm)
3,5
7
8,6
15
18
Congenital abnormalities
Multiple pancreatic and renal cysts
Left kidney agenesia
NO
NO
NO
Histopathology
cystic lymphangioma
cystic emolymphangioma
cystic lymphangioma
cystic lymphangioma
cystic lymphangioma
Follow – up
6 months
2 years
4 months
1 year
1 year
Recurrence
No
No
No
No
No
In two patients of our series other congenital abnormalities were associated: in patient
n.1 also other cystic lesions (kidney and pancreas) were present and in case report
n.2 a left kidney agenesia was associated (Table 1).
In case n.2 the histopatological examination revealed the presence of a cystic emolymphangioma
(Table 1) that is very rare entity in intra – abdominal localization. This tumor is
considered a congenital malformation of the vascular system whose formation may be
explained by obstruction of the venolymphatic communication between dysembrioplastic
vascular tissue and the systemic circulation.[7]
In our series, clinical manifestations are variable among patients, with no characteristic
signs and symptoms. More common manifestations are a painless abdominal distension
or an abdominal mass, with the latter being often detected incidentally. There can
be an acute abdomen presentations if the lymphangioma becomes complicated due to infection,
hemorrhage or bowel obstruction ( as in case n.3) (Table 1). [8]
The ultrasonographic presentation of a mesenteric lymphangioma is described as a cystic
lesion with multiple thin septa (honeycomb or cobweb pattern). Ultrasound-guided diagnostic
aspiration usually yields a chylous aspirate. On CT imaging, mesenteric lymphangiomas
appear as a uni- or multilocular masses with enhancement of the wall and septum by
contrast medium [9]. However these studies help to determine, if the tumor is cystic,
its size and location, but they are insufficient to establish an accurate preoperative
diagnosis. Magnetic resonance imaging is the most useful preoperative radiological
tool for diagnosis and in surgical planning. In differentiating the mesenteric cyst
from the lymphangioma, magnetic resonance imaging is suggestive because it allows
a good differentiation of cystic and septal structures. Mesenteric cystic lymphangiomas
lack demonstrable fat content by chemical shift and fat saturation, as clearly seen
in the magnetic resonance imaging of dermoid cyst [10].
However, the definitive diagnosis of lymphangioma is based on histopathology and immunochemistry
: the lining mesothelial cells are immunoreactive for cytokeratin and negative for
factor VIIIIs. Double staining with Prox1 and CD31 is the most reliable method for
characterizing lymphangioma endothelial cells.[11]
In our patients we performed a complete surgical excision of the mass, that is the
treatment of choice for cystic lymphangioma, even if asymptomatic. Surgical enucleation
without damage to the blood supply of the bowel is also suggested, with the prognosis
being generally favorable, but with increasing tumor size radical resection becomes
more difficult and local recurrence more probable [12,13]. Infiltration of the intestine
or involvement of the main branch of mesenteric arteries or adjacent organs necessitate
segmental resection of the intestine [14] .
The prognosis after adequate excision of the cystic tumors of the mesentery is considered
to be excellent.
Drainage has been suggested as a modality of treatment in high – risk patients but
is often unsuccessful because of recurrence and for the risk of perforation of the
mesentery during the drainage of the lymphangioma [15]. Instillation sclerotherapy
with alcohol is being used for ablation, but this method can be destructive to normal
tisuue. In cases of failed percutaneous sclerotherapy using alcohol, acetic acid has
been used with good success in intra-abdominal lymphangioma [16].
Adjuvant treatment with OK-432, a biological response modifier with antitumor effects,
has been shown to prevent further enlargement of small localized remnant cysts after
surgery [17].
In our experience, we follow up the patients with a clinical examination and an abdominal
ultrasound at 3 , 6 and 12 months after the operation , then annually. During a median
follow up period of 11,5 months (4 – 48 months) no recurrence occurred (Table 1).
Conclusion
Mesenteric lymphangioma in adult is a rare disease: it may present as an abdominal
swelling or acute intestinal obstruction. Preoperative diagnostic tools are ultrasound
and abdomen CT or MR. Complete surgical resection is the ideal modality of treatment
for mesenteric lymphangioma. Definitive diagnosis is confirmed by histopathology.