Lymphangiom des Ösophagus

Esophageal

lymphangioma: a case report and review of literature. Endoscopic and histopathological findings of the esophageal lymphangioma. a sessile polypoid soft tumor with a lustrous surface was found endoscopically in the upper-mid esophagus at 20–22 cm from the incisor. b Under endoscopic ultrasonography, the tumor showed a heterogeneous hypoechoic pattern and involved the lamina propria and submucosal space, but not the muscularis propria. c Microscopically, the tumor was composed of dilated lymphatic vessels in various sizes underneath the normal esophageal squamous epithelium. d The lymphatic vessels were lined by flat benign endothelial cells with lymphocytic aggregates in the fibrous stroma. e Flat endothelial lining cells of this esophageal lymphangioma were immunoreactive to D2–40, a classical biomarker for lymphatic endothelial cells, confirming the diagnosis of lymphangioma

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Lymphatic malformations are benign lesions of vascular origin that show lymphatic differentiation. Specifically, they are vascular malformations and not vascular tumors as per the 2018 ISSVA classification of vascular anomalies .

This article focuses on the general features of lymphatic malformations. For a specific discussion in other locations, please refer to:

Terminology

These malformations were formerly called lymphangiomas. This expression is out-of-date according to the 2018 ISSVA classification .

Epidemiology

They can present at any age but most often occur in the pediatric population (~90% in those less than 2 years old ). The worldwide incidence of lymphangiomas is 1:6000-16000 live births. Males and females are equally affected.

Clinical presentation

Generally, the presentation may be with symptoms related to local mass effect and/or hemorrhage.

For example, a lymphatic malformation within the orbit may present with progressive proptosis with acute deterioration in symptoms, the mass effect resulting in compressive optic neuropathy, diplopia/ocular muscle weakness, and orbital bruising.

The clinical examination may reveal soft, non-tender masses on palpation with a doughy consistency.

Pathology

Typically comprised of thin-walled cystic masses and can be classified according to the size of the cystic lesions :

macrocystic lymphatic malformation
- previously known as cystic hygroma / cystic lymphangioma, although a term still commonly used when large in the cervical region
- mean diameter of cystic lesions >1 cm
microcystic lymphatic malformation
- previously known as cavernous lymphangioma
- mean diameter of cystic lesions <1 cm
mixed type lymphatic malformation: both macroscopic and microscopic features

Their wall consists of connective tissue, smooth muscle, fat, blood vessels, nerve, and/or lymphatic tissue.

Location

They can occur at almost any location:

marked predilection in the head and neck: 95% in the neck and axillary regions
- cystic hygroma (cystic or nuchal lymphangioma)
mesentery, retroperitoneum, abdominal viscera, lung, and mediastinum: ~5%

Associations

Radiographic features

Lymphatic malformations may cross more than one compartment. For instance, in the head and neck region, larger lesions tend to occupy more than one deep space, sandwiching between normal structures.

Ultrasound

multilocular cystic masses
internal septa of varying thickness
cystic contents: usually anechoic, hyperechoic if debris, high lipid concentration, infection or hemorrhage
wide variations exist: solid areas, or mostly solid with cystic foci
color Doppler: +/- arterial or venous flow in the septa

CT

Most lymphatic malformations appear homogeneous and cystic on CT, but some appear inhomogeneous because of the presence of proteinaceous, fluid, blood, or fat components within the lesion. It is rare for CT to demonstrate intrinsic septations. There is only minimal or no displacement/compression of adjacent structures.

MRI

Fluid-fluid levels may be seen if complicated by hemorrhage. Signal characteristics include:

T1: can be variable especially dependent on protein content
T2: usually high signal

Treatment and prognosis

Surgical excision or interventional sclerotherapy (with interferon, OK-432, or bleomycin) is often necessary . Other possible treatment methods include steroid therapy, laser treatment, aspiration, radiofrequency ablation, or cautery.