Lymphangioma is a benign tumor composed of dilated lymph vessels that results from malformations of the lymphatic system. They most commonly occur in children under 2 years old as soft, fluid-filled neck masses. Lymphangiomas are classified based on their origin (congenital or acquired), microscopic features (capillary, cavernous, cystic), and size (microcystic or macrocystic). Symptoms include swelling, pain, and breathing issues. Diagnosis involves ultrasound, CT, or MRI scans. Treatment options include surgery, sclerotherapy, radiofrequency ablation, and drug therapies like sirolimus.
2. Introduction
• a benign tumor composed of dilated and newly
formed lymph vessels.
• Lymphangiomas are malformations of the
lymphatic system characterized by lesions that are
thin-walled cysts .
• Lymphatic analogue of hemangioma of blood
vessels.
3. • Represents isolated and sequestered segments of
lymphatic system
Retains the ability to produce lymph
4. • These malformations can occur at any age and may
involve any part of the body, but 90% occur in
children less than 2 years of age and involve the
head and neck.
• Lymphangiomas are commonly diagnosed before
birth using fetal ultrasonography.
6. Acc to origin
• Congenital lymphangiomas are often associated
with chromosomal abnormalities such as Turner
syndrome.
• Acquired lymphangiomas may result from trauma,
inflammation, or lymphatic obstruction.
7. Acc to microscopic characteristic
• Capillary lymphangiomas
• Capillary lymphangiomas are composed of small,
capillary-sized lymphatic vessels and are
characteristically located in the epidermis.
• Cavernous lymphangiomas
• Composed of dilated lymphatic channels,
cavernous lymphangiomas characteristically invade
surrounding tissues.
8. • Cystic hygromas
• Cystic hygromas are large, macrocystic
lymphangiomas filled with straw-colored, protein-
rich fluid.
• Hemangiolymphangioma
• As suggested by their name,
hemangiolymphangiomas are lymphangiomas with
a vascular component.
9. Acc to size
• Microcystic lymphangiomas
• Microcystic lymphangiomas are composed of cysts,
each of which measures less than 2 cm3 in volume.
• Macrocystic lymphangiomas
• Macrocystic lymphangiomas contain cysts measuring
more than 2 cm3 in volume.
• Mixed lymphangiomas
• Lymphangiomas of the mixed type contain both
microcystic and macrocystic components.
10. Staging
• Stage I
• Unilateral infrahyoid.
• Stage II
• Unilateral suprahyoid.
• Stage III
• Unilateral suprahyoid and infrahyoid.
• Stage IV
• Bilateral suprahyoid.
• Stage V
• Bilateral suprahyoid and infrahyoid.
11. Symptoms and complication
• Swelling
• pain
• infection
• breathing problems when swellings in the neck press
on the airway
• difficulty swallowing or speaking
• inflammation or cellulitis
• bleeding
• double vision if the eye socket is affected
12. Diagnosis
• Diagnosis of lymphangiomas is not usually difficult and
can often be done before birth, using a prenatal
ultrasound.
• Once a baby is born, a lymphangioma can be identified
during a physical examination. It will usually appear as
a soft, ill-defined fluid-filled mass that moves around
under the skin when pressure is applied.
• The use of ultrasound, computed tomography (CT), or
magnetic resonance imaging (MRI) scans can also help
identify the mass.
13. Treatment
• Surgery: Surgical removal can be a difficult
procedure if the lymphatic malformation has
traveled into the nerves and muscles.
• Sclerotherapy: A solution is injected into the
swelling to cause it to shrink or collapse.
• Radiofrequency ablation: A high frequency current
delivered via a needle destroys abnormal tissue.
14. • Dermabrasion: A skin resurfacing technique can be
used to treat facial scarring.
• Percutaneous drainage: An incision is made in the
lymphatic malformation, and the fluid is drained.
• Drug treatment: Commonly associated with
treating cancer, the drug sirolimus has been shown
to shrink lymphatic malformations.
15.
16. Differential Diagnosis
• Cavernous Hemangioma
• Lymphangioma can be differentiated from
cavernous hemangioma by the presence of lymphoid
aggregates in the stroma and more irregular lumens
with widely spaced nuclei whereas cavernous
hemangiomas are more common during childhood
and show large dilated blood-filled vessels lined by
flattened endothelial cells. Vessels may be arranged
in roughly lobular arrangement or diffuse haphazard
pattern.