Lymphangioma (Mesenteric Cyst)

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 0 (0 votes)

This article have been viewed 2219 times

 Can arise from or involve virtually any structure

• Circumscribed cystic mass with variable density

image Typically water density (near 0 HU) or chylous (< -20 HU), and only rarely hemorrhagic
image No internal enhancement
image Can be multiloculated (± septations) with “feathery” appearance
image Soft lesions without mass effect: Easily indented by surrounding structures, such as mesenteric vessels/bowel
image Usually hypointense on T1WI MR and hyperintense on T2WI

TOP DIFFERENTIAL DIAGNOSES

• Loculated ascites
• Gastrointestinal duplication cyst
• Pancreatic pseudocyst
• Peritoneal inclusion cyst
• Cyst or cystic tumor arising from visceral organ

CLINICAL ISSUES

• Symptoms are rare (particularly in adults)

image Rare symptoms due to mass effect, superinfection, or internal hemorrhage
• Surgery is treatment of choice when necessary

DIAGNOSTIC CHECKLIST

• Differentiate from other primary cystic lesions or tumors of visceral organs
image
(Left) Axial CECT shows a complex cystic mass in the mesentery sandwiching a small bowel segment image. The mass is near water density and has small foci of calcification in its septa and peripheral walls image. The soft nature of the mass is indicated by the absence of bowel obstruction.

image
(Right) Axial CECT shows a complex water-density mass in the mesentery, immediately adjacent to the pancreas and duodenum. The mass is divided by multiple septa image, which, like the peripheral walls, are thin.
image
(Left) Axial CECT in a female patient shows a large, cystic mass with multiple septations image filling much of the lower abdomen. Note the calcifications image in the septa and the peripheral walls.

image
(Right) Axial CECT in a female patient shows a cystic retroperitoneal mass image with subtle septa and a small focus of calcification image. The mass was resected and found to contain chylous fluid (typical of a lymphangioma) and an epithelial lining, features that help account for the variety of names for this tumor.

TERMINOLOGY

Synonyms

• Lymphoepithelial cyst, cystic lymphangioma, mesenteric cyst, lymphatic malformation

Definitions

• Congenital benign malformation of lymphatic system arising due to failure of embryologic lymphatic development
• Generic descriptive term for benign congenital cystic mass arising in mesentery or omentum

IMAGING

General Features

• Best diagnostic clue

image Cystic mass (without mass effect) in mesentery or retroperitoneum
• Location

image Majority of lymphangiomas arise in head, neck, or axillae
image Lymphangiomas of abdomen rare (7% of all lymphangiomas)

– Can involve multiple compartments of peritoneum or retroperitoneum
– Can arise from or involve virtually any structure
image Lymphangiomatosis: Widespread lymphangiomas (usually liver, spleen, mediastinum, lungs, mesentery)

– Usually presents in infants and young children
• Size

image Few mm to 40 cm in diameter
• Morphology

CT Findings

• Circumscribed cystic mass with variable density

image Typically water density (near 0 HU) or chylous (< -20 HU), with lesions rarely demonstrating hemorrhagic contents
image No internal enhancement
• Can be multiloculated (± septations) with “feathery” appearance

image ± fine calcifications along cyst wall
• Soft lesions without mass effect that are indented by surrounding structures (e.g., mesenteric vessels or bowel)

Ultrasonographic Findings

• Fluid-filled cystic structure with thin internal septa

image ± internal echoes due to debris, hemorrhage, or infection

MR Findings

• Multiloculated cyst, usually hypointense on T1WI and hyperintense on T2WI

image Can be T1 hyperintense due to internal fat/chyle

DIFFERENTIAL DIAGNOSIS

Loculated Ascites

• May appear similar to lymphangioma, but there is typically a known underlying cause for ascites (e.g., cirrhosis)

Gastrointestinal Duplication Cyst

• Cystic mass with thick wall abutting bowel

Pancreatic Pseudocyst

• Cyst with visible wall in patient with history of pancreatitis
• Cyst often associated with stranding of surrounding fat

Cyst or Cystic Tumor Arising From Visceral Organ

• Mesenteric cysts can abut visceral organs (such as pancreas or kidneys) and mimic cystic lesion arising from organ (e.g., exophytic renal cyst)

Peritoneal Inclusion Cyst

• Cystic mass in reproductive-age female after surgery
• Loculated cystic lesion conforming to shape of pelvis and often surrounding ovary

PATHOLOGY

General Features

• Etiology

image Failure of normal embryologic development with lymphatic tissue not communicating with rest of lymphatic system

Gross Pathologic & Surgical Features

• Thin walled and multiseptated with serous, serosanguineous, or chylous fluid contents

Microscopic Features

• Cuboidal or columnar cells lining cyst ± smooth muscle, lymphatics, and blood vessels within walls

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Usually asymptomatic (particularly in adults)
image Rare symptoms (abdominal distention, pain) due to size (particularly in neonates) or superinfection
image Theoretical risk of pseudomyxoma peritonei

Demographics

• Epidemiology

image Can occur at any age, but 75% discovered age < 5 years 

– 90% continue to grow until 2 years of age
image M > F
image Rare (1/40,000 persons)

Natural History & Prognosis

Treatment

• Follow asymptomatic patients with repeat imaging
• Aspiration and sclerosing agents are usually ineffective
• Open or laparoscopic surgical resection if lesion is symptomatic or cannot be differentiated from malignancy

image Good prognosis after surgery: 0-13.6% recurrence rate

DIAGNOSTIC CHECKLIST

Consider

• Differentiate from other primary cystic lesions or tumors of visceral organs

image Look for claw sign of cystic mass arising from visceral organ

image
(Left) Axial CECT demonstrates innumerable low density, cystic masses image throughout the retroperitoneum. The lesions surround major vasculature without mass effect or narrowing.
image
(Right) Axial CECT more caudal in the same patient demonstrates similar-appearing cystic lesions image along the iliac chains in the pelvis. Patients with lymphangiomatosis, as in this case, can have innumerable lymphangiomas anywhere in the body.
image
(Left) T2WI MR demonstrates a lymphangioma image in the left pelvic retroperitoneum. As in this case, most lymphangiomas are uniformly T2 hyperintense, although intralesional hemorrhage, debris, or fat can result in a more intermediate T2 signal.

image
(Right) Axial T1 C+ MR in the same patient demonstrates an absence of internal enhancement or nodularity within the cystic lesion image, features that would cast doubt on the diagnosis of a lymphangioma.
image
(Left) Axial CECT demonstrates a cystic mass image in the retroperitoneum. Notice the lack of any internal enhancing soft tissue or mural nodularity.

image
(Right) Coronal VR CECT better illustrates how the mass image envelops vasculature image without appreciable mass effect or narrowing (characteristic of lymphangiomas).

image
(Left) Axial CECT demonstrates a cystic mass image in the left retroperitoneum. The lesion abuts the pancreatic tail and left colon without appreciable mass effect.
image
(Right) Axial CECT in the same patient nicely demonstrates the multiloculated, “feathery” morphology of the lesion image. This appearance is quite common with lymphangiomas, which frequently appear to have multiple internal discrete components or locules.
image
(Left) Coronal volume-rendered CECT demonstrates a multiloculated cystic lesion image in the left retroperitoneum. The lesion envelops multiple arteries and veins image, which do not appear deviated or narrowed.

image
(Right) Coronal VR CECT again nicely demonstrates the morphology of the lymphangioma image, which has several discrete cystic components and appears multiloculated. These lesions are characteristically soft, and while they abut adjacent structures, there is typically no mass effect.
image
(Left) Axial CECT shows a thin-walled mass with water density, indented by a mesenteric vessel image in this patient with cystic lymphangioma.

image
(Right) Axial CECT shows another view of the water-density, thin-walled mass being indented by a mesenteric vessel image.
image
Axial CECT shows cystic lymphangioma as a thin-walled water-density mesenteric mass with scattered calcifications in septa.

image
Coronal T2WI MR shows large, multiloculated cystic lymphangioma with water intensity.

Share this: