II. Definitions

  1. Lymphangioma
    1. Benign tumor developing as a Congenital Abnormality of the lymph system
    2. Results in dilated lymph channels

III. Epidemiology

  1. Onset under age 2 years (typically as a young infant)

IV. Pathophysiology

  1. Congenital lymphatic malformation
    1. Lymphatic vessel fails to connect and drain
    2. Results in large multiloculated cyst
  2. Distribution
    1. Neck (60%)
    2. Other locations
      1. Chest Wall
      2. Mediastinum
      3. Axilla
    3. Rare locations
      1. Inguinal region
      2. Retroperitoneal region

V. Associated Conditions

  1. Turner's Syndrome
  2. Noonan's Syndrome
  3. Down Syndrome

VI. Symptoms

  1. Initial
    1. Asymptomatic
    2. Painless lesion unless infected or bleeding
  2. Progression: Continues to increases in size
    1. 90% symptomatic by age 2 years

VII. Signs

  1. Fluctuant mass usually in neck
  2. Mass transilluminates

VIII. Complications

  1. Infection
  2. Bleeding
  3. Airway compromise (due to compression)

IX. Differential Diagnosis

X. Imaging

  1. Chest XRay
    1. Identify mediastinal extension
    2. Pleural Effusion

XI. Management

  1. Deliver where NICU available if diagnosed prenatally
    1. Risk of airway compromise
  2. Surgical excision

XII. Prognosis

  1. Recurrence rate after surgical excision: 10-15%

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Related Studies

Ontology: Lymphangioma (C0024221)

Definition (MSH) A benign tumor resulting from a congenital malformation of the lymphatic system. Lymphangioendothelioma is a type of lymphangioma in which endothelial cells are the dominant component.
Definition (NCI_CDISC) A benign neoplasm arising from the lymphatics.
Definition (NCI) A benign neoplasm arising from the lymphatics. The tumor is composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.
Concepts Neoplastic Process (T191)
MSH D008202
ICD9 228.1
ICD10 D18.1 , M9170/0
SnomedCT 69044001, 189200000, 154625006, 189875000, 93168006, 269646001, 400178008, 254836000, 253057002
English Lymphangioma, Lymphangiomas, Lymphangioma, any site, LYMPHANGIOMA, Lymphangioma NOS, [M]Lymphangioma NOS, benign lymphangioma, benign lymphangioma (diagnosis), Lymphangioma, no ICD-O subtype, Lymphangioma [Disease/Finding], lymphangiomas, congenital lymphangioma, lymphangioma, [M]Lymphangioma NOS (morphologic abnormality), Lymphangioma of unspecified site (disorder), Lymphangioma of unspecified site, Lymphangioma, no International Classification of Diseases for Oncology subtype (morphologic abnormality), Lymphangioma, no International Classification of Diseases for Oncology subtype, Lymphangioma, no ICD-O subtype (morphologic abnormality), LYMPHANGIOMA, BENIGN, Congenital lymphangioma, Lymphangioma morphology, Benign lymphangioma, Congenital lymphangioma (disorder), Lymphangioma (disorder), Lymphangioma, site unspecified, Benign lymphangioma (morphologic abnormality), Lymphangioma, NOS
French LYMPHANGIOME, Lymphangiome, site non précisé, Lymphangiome
Portuguese LINFANGIOMA, Linfangioma de qualquer localização, Linfangioma
Spanish LINFANGIOMA, morfología: linfangioma, no clasificado como subtipo en CIE - O, Linfangioma de cualquier localización, [M]linfangioma, SAI, linfangioma de localización no especificada (trastorno), [M]linfangioma, SAI (anomalía morfológica), linfangioma (trastorno), linfangioma benigno (anomalía morfológica), linfangioma benigno, linfangioma congénito (trastorno), linfangioma congénito, linfangioma de localización no especificada, linfangioma, SAI, linfangioma, morfología de linfangioma, morfología: linfangioma, no clasificado como subtipo en CIE - O (anomalía morfológica), Linfangioma
German LYMPHANGIOM, Lymphangiom, beliebige Stelle, Lymphangiom, jede Lokalisation, Lymphangiom
Dutch lymfangioom, elke plaats, Lymfangioom, elke lokalisatie, lymfangioom, Angioom, lymf-, Lymfangioom
Italian Linfangioma, ogni sede, Linfangioma
Japanese リンパ管腫、部位を限定しない, リンパカンシュ, リンパカンシュブイヲゲンテイシナイ, リンパ管腫-海綿状, リンパ管内皮腫, リンパ管腫, 海綿状リンパ管腫
Swedish Lymfangiom
Czech lymfangiom, Lymfangiom, Lymfangiom kterékoliv lokalizace
Finnish Lymfangiooma
Korean 모든 부위의 림프관종
Polish Śródbłoniak limfatyczny, Naczyniak limfatyczny
Hungarian Lymphangioma, bármely hely, Lymphangioma
Norwegian Lymfangiom

Ontology: Cavernous lymphangioma (C0205828)

Definition (NCI) A lymphangioma characterized by the presence of thin-walled cavernous lymphatic spaces.
Concepts Neoplastic Process (T191)
MSH D008202
ICD10 M9172/0
SnomedCT 89056007
English Cavernous Lymphangiomas, Lymphangiomas, Cavernous, Cavernous Lymphangioma, Cavernous lymphangioma, Cavernous lymphangioma (morphologic abnormality), cavernous; lymphangioma, lymphangioma; cavernous, Lymphangioma, Cavernous
Portuguese Linfangioma Cavernoso
Spanish Linfangioma Cavernoso, linfangioma cavernoso (anomalía morfológica), linfangioma cavernoso
French Lymphangiome caverneux
German Lymphangiom, kavernöses
Italian Linfangioma cavernoso
Norwegian Kavernøst lymfangiom
Dutch caverneus; lymfangioom, lymfangioom; caverneus, Lymfangioom, caverneus

Ontology: Lymphangioma, Cystic (C0206620)

Definition (NCI) A benign lymphatic neoplasm usually arising from the neck and characterized by cystic dilation of the lymphatic vessels.
Definition (MSH) A cystic growth originating from lymphatic tissue. It is usually found in the neck, axilla, or groin.
Concepts Neoplastic Process (T191)
MSH D018191
ICD10 M9173/0
SnomedCT 423984004, 40225001, 399882002
English Hygroma cystic, Hygroma, Hygroma, Cystic, Lymphangioma, Cystic, Cystic Hygromas, Cystic Lymphangiomas, Hygromas, Hygromas, Cystic, Lymphangiomas, Cystic, Cystic Hygroma, Cystic Lymphangioma, Non-neoplastic hygroma (disorder), Non-neoplastic hygroma, HYGROMA CYSTIC, Colli, Cystic Hygroma, Hygroma Colli, Cystic, Cystic Hygroma Colli, Lymphangioma, Cystic [Disease/Finding], cystic hygroma, cystic lymphangioma, cystic lymphangiomas, hygromas, cystic hygromas, Cystic lymphangioma, Cystic hygroma, Cystic hygroma (disorder), Cystic lymphangioma (morphologic abnormality), cystic; lymphangioma, hygroma, lymphangioma; cystic, Hygroma, NOS, Lymphangioma;cystic
Dutch cystisch hygroom, hygroom cystisch, cysteus; lymfangioom, lymfangioom; cysteus, cystisch lymfangioom, Cystisch lymfangioom, Hygroom, Hygroom, cystisch, Lymfangioom, cystisch
German zystisches Hygrom, Hygrom, zystisch, HYGROMA CYSTICUM, zystisches Lymphangiom, Hygrom, zystisches, Hygrom, Lymphangiom, zystisches
Portuguese Higroma quístico, HIGROMA QUISTICO, Linfangioma cístico, Higroma Cístico, Higroma, Linfangioma Cístico
Spanish Higroma quístico, higroma no neoplásico, higroma no neoplásico (trastorno), higroma quístico (trastorno), higroma quístico, higroma, linfangioma quístico (anomalía morfológica), linfangioma quístico, Linfangioma cístico, Higroma Quístico, Higroma, Linfangioma Quístico
Japanese 水滑性嚢腫, ノウホウセイリンパカンシュ, ノウホウセイヒグローマ, スイカツセイノウシュ, リンパ管腫-嚢胞性, 嚢腫性リンパ管腫, のう胞性リンパ管腫, ヒグローマ, 嚢胞性ヒグローマ, 嚢胞性リンパ管腫, ヒグローマ-嚢胞性
Swedish Lymfangiom, cystiskt
Czech lymfangiom cystický, Cystický lymfangiom, Hygroma cysticum, Cystický hygrom
Finnish Imusuonirakko
French HYGROMA KYSTIQUE, Hygrome kystique, Lymphangiome kystique, Hygroma kystique
Italian Igroma cistico, Igroma, Linfangioma cistico
Polish Wodniak torbielowaty, Hygroma, Naczyniak limfatyczny torbielowaty
Hungarian Hygroma cystikus, Cystikus lymphangioma, Cystikus hygroma
Norwegian Cystisk hygrom, Cystisk lymfangiom