LIMFANGIOMA

Hervina*

*Departement Of Dermatology and venereolgy

Faculty of Medicine Muhammadiyah University of North Sumatera
** Departement Farmacology Faculty of Medicine Muhammadiyah University of
North Sumatera

ABSTRAK

Limfangioma adalah malformasi kongenital yang berasal dari hiperplasia limfatik.

Limfangioma berasal dari jaringan limfe, vaskuler atau gabungan vaskulolimfatik. Klasifikasi
limfangioma yaitu limfangioma kistik atau higroma kistik, limfangioma kavernosus,
limfangioma simpleks atau kapiler dan jarang didapat yaitu hemangiolimfangioma. Limfangioma
juga diklasfikasikan menurut ukuran kista menjadi mikrositik, makrositik dan subtipe campuran.
Limfangioma toraks muncul di intraparenkim dan mediastinum. Limfangioma lebih sering
muncul di rongga mediastinum. Tatalaksana limfangioma adalah dengan multimodalitas yaitu
utama dengan pembedahan, kemoterapi, radioterapi dan skleroterapi. Reseksi komplit diperlukan
untuk mencegah rekurensi.
Kata kunci : limfangioma, limfe, tumor limfe, higroma kistik
ABSTRACK

Lymphangioma is a congenital malformation coming from the lymphatic hyperplasia.

Lymphangioma originates from the lymphatic tissue, vascular or combined lymphovascular.
Lymphangioma is classified into cystic lymphangioma or cystic hygroma, cavernous
lymphangioma, simplex or capillaries lymphangioma and rarely hemangiolymphangioma.
Lymphangioma is also classified according to the size of the cyst to microcytic, macrocytic and
subtypes mix. Thoracic lymphangioma appears in intra-parenchyma and mediastinum.
Lymphangioma mostly appears in the cavity of the mediastinum. Management of lymphangioma
are; multimodality of major surgery, chemotherapy, radiotherapy, and sclerotherapy. Complete
resection is required to prevent recurrence.
Keywords: lymphangioma, lymphatic, lymphatic tumor, cystic hygroma
PRELIMINARY
Lymphangioma is a benign tumor
caused by congenital malformations of the
lymphatic system.4 These tumors usually
occur in the head, neck and armpit, but
sometimes occur in the mediastinum,
retroperitoneum, and thighs. Often also
occurs in the scrotum and perineum.4
Lymphangioma is often found in
newborns, rarely in adults, occurrence in
men and women, not significantly
2 The exact cause of lymphangioma
different. Approximately 50% of these
malformasilimfatik seen in newborns, and
90% appear before the age of 2 years.2
DEFINITION
Lymphangioma is a benign tumor
caused by congenital malformations of the
lymphatic system.4 These tumors usually
occur in the head, neck and armpit, but
sometimes occur in the mediastinum,
retroperitoneum, and thighs. It also often
occurs in the scrotum and perineum.4 The
incidence of lymphatic malformation is
unknown, but it is believed to exceed 6.3%
of all malformations.4 Lymphangiomas
originate from the primitive sac of the
embryo, some lymphatic tissue which loses
contact with the normal lymphatic system,
but still has rapid growth potential initially.4
Pathologically can be divided into: simple
lymphangioma, limfangioma spongiosa,
cystic lymphangioma.6
EPIDEMIOLOGY
This disease is spread throughout the
world. There is no predilection of sex.8
Usually associated with other congenital
anomalies.8 The incidence rate of this
disease is around 1-2 events per 1000 live
births.8 Approximately 50% of these
malformasilimfatik seen in newborns, and
90% appear before age 2 year.8
Lymphangioma is often found in
newborns, rarely in adults, the incidence in
men and women, not significantly different.8
His age ranges from birth to 12 years, but
most (73%) are aged 4 years or younger.8
This cancer is 6 % of all cancers in the
world in 2002 or the fifth most cancer and
an estimated 45,000 cases were diagnosed in
2004.8
ETIOLOGY
formation is unknown, but most cases are
believed to be sporadic.8 The formation of
lymphangiomas may reflect the failure of
the lymph channels to connect with the
venous system during embryogenesis,
abnormal absorption of lymphatic structures,
or both.8 Continuous research has described
several possible vascular growth factors
involved in the formation of lymphatic
malformations such as VEGF-C and FLT-4.
Cases secondary to trauma and infection
have also been reported.8
The cause of lymphangioma is
caused by congenital malformations of the
lymphatic system.3 Genetic factors, tobacco
exposure, alcohol consumption, viruses and
food deficiencies can also be the cause of
lymphangioma. 3
DIAGNOSE
The diagnosis of lymphangioma is
mainly based on clinical history and the
results of conventional physical and light
microscopy.8 Cases of limfangioma are
diagnosed through histological observation.8
In prenatal cases, cystic lymphangiomas are
diagnosed with ultrasound, amniosynthesis
is recommended to examine in relation to
genetic disorders.8
MRI can help determine the level of
involvement and anatomy of all
lymphangioma.8 MRI lesions can help
prevent not expanding, incomplete surgical
resection, because of the relationship with
high recurrence rates.8
Immunohistochemical studies that
are useful in distinguishing lymphangioma
from hemangiomas in difficult cases.8 The
results of testing with VIII antigen are
related to positive factors for hemangiomas
but weak negative or positive in endothelial
lymphangiomas.8
Dermoscopy findings can help in the
diagnosis of skin lymphangioma
circumscriptum.8 These nodules are filled
with a light brown liquid, lakuna surrounded
by a septa palate.8
PATHOPHYSIOLOGY
Lymphangioma sirkumkriptum
shows a history of a small number of typical
vesicles on the skin at birth or immediately
after birth.9 In the following years, vesicles
tend to increase in number, and the affected
skin area continues to grow.9 Other vesicles
or skin disorders may not be noticed until
several years after birth.9 Usually, lesions do
not show symptoms or show no evidence of
disease, but, for the most part, patients may
randomly experience bleeding from ruptured
vesicles.9
Limfagioma cavernosa first appears
in infancy, when nodules are like rubber
without obvious changes in the skin on the
face, body, or extremities.9 These lesions
often grow fast, similar to hemangiomas.9
Cystic hygroma causes subcutaneous
swelling, usually in the armpit, base of the
neck, or groin, and is usually seen
immediately after birth.8 If the lesion is
drained, it will quickly fill with more fluid.8
The lesion will grow and increase to a larger
size if they are not completely eliminated by
surgery.9
Specific mutation patterns in genes
provide information about etiologikanker.2
The p53 gene can be inactivated by various
mutations and is often inactivated in human
cancer.2 Cancer is a complex genetic disease
originating from the accumulation of various
genetic changes.2 including this genetic
change is proto-activation oncogenes and
inactivation of tumor suppressor genes.2
Inactivation of tumor suppressor genes
requires inactivation of all parental alleles,
generally with pointmutation and
2
chromosomal deletions. Molecular models
such as these are paradigms for the
progression of various solid tumors.2
Lymphangiomas originate from congenital
malformations of the lymphatic system .2
THREATMENT
Surgical excision is the treatment of
choice for local lymphangioma if it is
anatomically possible.8 Of the various
surgical techniques that have been explored
for years, the total removal of tumors
without leaving the cystic epithelium has
become the most reliable procedure.8
PROGNOSE
Prognosis has a strong correlation
with the stage at diagnosis.7 In general, the
prognosis is determined by the size of the
tumor, the presence of regional lymph node
metastases and distant metastases, the
greater the tumor prognosis, the worse. and
increase the risk of distant metastases.7
Ranging from 15% of head and neck cancers
will develop into distant metastases.7
CONCLUSION
Lymphangioma is a benign tumor
caused by congenital lymphatic system
malformations.4 In local lymphangiomas,
non-surgical therapy can be given while
supervision is done if lymphphoma does not
affect life function, because some surgeons
believe that more than 15% of these lesions
will shrink by themselves.4 However if the
lesion does not shrink spontaneously at the
age of 5 years, surgical intervention is
needed.8
Complete resection surgery of a local
disease has been shown to be very
effective.8 The recurrence rate is low if
complete removal of the cystic epithelium is
achieved.8 Prognosis has a strong correlation
with the stage at diagnosis.8 In general the
prognosis is determined by tumor size,
presence of regional and metastatic lymph
node metastases far, the greater the tumor's
prognosis the worse.8
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2. Desen W, 2008, Buku Ajar Onkologi
Klinis edisi 2, Fakultas Kedokteran
Universitas Indonesia, Jakarta.
3. Suyatno & Pasaribu,
E.T,2010, Bedah Onkologi
Diagnostik Dan Terapi, CV Sagung
Seto, Jakarta
4. Budimulja, Unandar. 2016. Ilmu
Penyakit Kulit dan Kelamin. Jakarta :
Balai Penerbit FKUI
5. Siregar, R. 2014. Atlas Berwarna
Saripati Penyakit Kulit. Jakarta :
EGC
6. Fitzpatrick, Freedeberg IM, Eisen
AZ, Wolff K, Austen KF, Goldsmith
LA, Katz St. 2007. Dermatology in
General Medicine. Edisi 6. New
York. The Mc Graw-Hill Companies
Inc.
7. Ameh EA, Laberge Louise C,
Laberge Jean M. 2011. Paediatric
Surgery: A Comprehensive Text For
Africa dalam Lymphangioma.
Africa: Global Health Education
Using Low-Cost Publications
8. Schwartz, Robert A. 2016.
Lymphangioma.
http://emedicine.medscape.com/articl
e/1086806-overview
9. Amouri M, Masmoudi A, Boudaya
S, et al. (2007). Acquired
lymphangioma circumscriptum of
the vulva. Dermatology online
journal 13 (4): 10