Lymphangioma and Superficial Lymphangioma


Large, Deep Lymphangioma
Clinical photograph shows a large, deep lymphangioma (cystic hygroma) on the lateral neck of a child.



image
Superficial Lymphangioma
This example of a superficial lymphangioma shows widely dilated spaces in the superficial dermis. The lumina show several papillary projections image with slightly fibrinous cores lined by small, hyperchromatic-staining endothelial cells.

image
Deep Lymphangioma
Deep lymphangioma shows large vascular channels filled with proteinaceous material, a few lymphocytes, and scattered erythrocytes image.

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Deep Lymphangioma
This example of a deep lymphangioma shows multiple dilated endothelial-lined channels with small collections of lymphocytes image within the lumina, along with fluid and erythrocytes.


TERMINOLOGY


Synonyms




• Lymphangioma circumscriptum (LC) (superficial cutaneous lymphangioma)

• Lymphangiomatosis (LS) (generalized lymphangioma, systemic angiomatosis)

• Cystic lymphangioma (cystic hygroma)

• Deep lymphangioma (cavernous lymphangioma)

• Lymphatic malformation


Definitions




• Proliferation of lymphatic vessels
image Superficial (lymphangioma circumscription)

image Deep (cavernous lymphangioma)

image Diffusely involve most organ systems (LS)


ETIOLOGY/PATHOGENESIS


Developmental Anomaly




• Most cases are considered developmental or congenital malformations/hamartomas, not true neoplasms
image Maldevelopment during embryonic lymphangiogenesis most likely etiology

– Leads to sequestered lymphatics that fail to communicate with normal lymphovascular system

image May be due to maternal infections or substance abuse

image LS considered congenital in most cases


Genetics




• Associated with genetic syndromes, including Turner syndrome (cystic hygroma), Noonan syndrome, Maffucci syndrome, trisomies 13, 18, 21

• Mutations in  VEGFR-C, VEGFR3, PROX1, FOXC2, and SOX18 genes implicated


Acquired




• Rare acquired cases occur in adults
image Likely associated with infection or trauma


CLINICAL ISSUES


Epidemiology




• Incidence
image More common in children: Estimated 6% of benign childhood tumors

• Age
image Often present at birth or within first 2 years of life (~ 90% of cases)

image LS usually presents within first 2 decades of life

• Sex
image Intraabdominal lymphangiomas have slight male predominance

image LS has no gender predilection


Site




• Head and neck most common site for cystic lymphangiomas
image Usually posterior triangle but can occur in anterior triangle

image Also occur in axillae, abdomen, and internal organs

• Cavernous type more frequent in oral cavity, upper trunk, limbs, and abdominal sites
image Intraabdominal lymphangiomas occur in mesentery, omentum, and retroperitoneum

• LC: Axillary folds, neck, and trunk are most common sites

• LS: Can affect any organ system but often involves bones, soft tissues, and skin


Presentation




• Cystic mass lesion; may be superficial or deep
image Typically presents as large, slow-growing, painless mass (deep lymphangioma) or as multiple small, grouped, superficial vesicular lesions (LC)

image LS presents with numerous cystic lesions, both superficial and deep

• Soft and fluctuant swellings on palpation

• Intraabdominal cases may present with abdominal distension, mass on palpation
image May also develop abdominal obstruction, volvulus, and infarction

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Lymphangioma and Superficial Lymphangioma
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