Definition/General
Introduction:
Umbilical cord hemangioma is a rare benign vascular tumor occurring in 1 in 5,500 deliveries
It represents the most common primary tumor of the umbilical cord
The lesion consists of proliferating endothelial cells and capillary channels
It may cause significant perinatal complications including fetal death.
Origin:
Originates from abnormal angiogenesis within umbilical cord vessels
Results from endothelial proliferation and capillary formation
May arise from hypoxic stimulation or vascular injury
Hamartomatous rather than truly neoplastic
Associated with altered blood flow patterns.
Classification:
Classified as capillary hemangioma (most common) or cavernous hemangioma
Small lesions (<2 cm) versus large lesions (>2 cm)
Localized versus diffuse involvement
May be associated with angiomatosis.
Epidemiology:
Incidence: 1 in 5,500 deliveries
No gender predilection
No specific maternal age association
Higher mortality with larger lesions
Associated with polyhydramnios in 30% cases.
Clinical Features
Presentation:
Polyhydramnios (30-70% cases)
Preterm delivery (60% cases)
Fetal heart failure
Intrauterine fetal death (10-20%)
Non-immune hydrops fetalis
Cord compression effects.
Symptoms:
Fetal distress during monitoring
Abnormal cardiotocography patterns
Decreased fetal movements
Signs of fetal anemia
Congestive heart failure in fetus
Emergency delivery indication.
Risk Factors:
Advanced maternal age
Previous pregnancy losses
Maternal diabetes
Multiple pregnancy
Vascular malformation syndromes
Family history of hemangiomas.
Screening:
Ultrasound examination may detect larger lesions
Color Doppler shows increased vascularity
Fetal echocardiography for heart failure
Amniotic fluid assessment
Serial monitoring recommended.
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Gross Description
Appearance:
Well-circumscribed mass within umbilical cord
Red to purple color
Soft, spongy texture
Size ranges from 1-10 cm
May cause focal cord enlargement.
Characteristics:
Vascular channels visible on cut surface
Hemorrhage may be present
Thrombosis in some vessels
Surrounding Wharton's jelly compressed
Surface may be ulcerated.
Size Location:
Size: typically 2-5 cm diameter
Location: any segment of cord
Fetal end more common
May involve entire cord circumference
Cord length usually normal.
Multifocality:
Usually solitary lesion
Multiple lesions rare
May be associated with placental hemangiomas
Angiomatosis pattern possible.
Microscopic Description
Histological Features:
Proliferation of capillary-sized vessels and endothelial cells
Well-formed vascular channels
Endothelial cell lining intact
Pericytes and smooth muscle present
Surrounding Wharton's jelly compressed.
Cellular Characteristics:
Plump endothelial cells without atypia
Mitotic activity variable
Pericytes surrounding vessels
Smooth muscle cells in vessel walls
Fibroblasts in stroma.
Architectural Patterns:
Capillary hemangioma pattern most common
Lobular arrangement of vessels
Cavernous spaces may be present
Thrombosis and fibrosis possible
Hemosiderin deposits common.
Grading Criteria:
Classification by vessel size and pattern
Cellular proliferation assessment
Mitotic count evaluation
Thrombosis and fibrosis extent
Size measurement important.
Immunohistochemistry
Positive Markers:
CD31 highlights endothelial cells
CD34 positive in vessels
Factor VIII related antigen positive
GLUT-1 may be positive
Smooth muscle actin in pericytes.
Negative Markers:
Cytokeratin negative in endothelium
EMA typically negative
S-100 negative
Desmin negative in endothelium
Lymphoid markers negative.
Diagnostic Utility:
Confirms vascular nature of lesion
Distinguishes from other tumors
Excludes malignancy
Identifies endothelial proliferation
Differential diagnosis aid.
Molecular Subtypes:
No specific molecular subtypes
Infantile hemangioma markers may be present
GLUT-1 positivity variable
Vascular proliferation markers elevated.
Molecular/Genetic
Genetic Mutations:
No specific genetic mutations identified
Angiogenesis pathway alterations possible
VEGF overexpression may be present
Endothelial growth factor abnormalities
Vascular development gene variants.
Molecular Markers:
VEGF and receptors upregulated
Angiopoietin signaling involved
Endothelial proliferation markers elevated
Hypoxia-inducible factors may be active.
Prognostic Significance:
Size correlates with complications
Large lesions (>4 cm) high risk
Associated with fetal mortality
Polyhydramnios increases risk
Heart failure poor prognostic sign.
Therapeutic Targets:
No specific therapeutic targets
Early delivery may be indicated
Fetal monitoring essential
Antiangiogenic therapy research
Corticosteroids investigational.
Differential Diagnosis
Similar Entities:
Arteriovenous malformation
Cord hematoma
Teratoma
Myxoma
Angiosarcoma (rare).
Distinguishing Features:
Hemangioma: benign vascular proliferation
Hemangioma: no atypia
AVM: abnormal connections
Hematoma: blood products
Teratoma: mixed tissue types
Angiosarcoma: malignant features.
Diagnostic Challenges:
Distinguishing from vascular malformations
Size assessment significance
Thrombosed vessels recognition
Clinical correlation importance
Sampling adequacy.
Rare Variants:
Cavernous hemangioma
Epithelioid hemangioma
Sclerosing hemangioma
Hemangioma with extensive thrombosis
Multiple hemangiomas.
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
Umbilical cord with focal mass measuring [X] cm
Tumor Description
Mass: [size] cm, [location], [color], [consistency]
Microscopic Pattern
Pattern: [capillary/cavernous] hemangioma with [degree] of vascular proliferation
Endothelial Features
Endothelial cells: [normal/plump], [present/absent] atypia, mitoses [count]/10 HPF
Associated Findings
Associated findings: [present/absent] thrombosis, [present/absent] fibrosis
Immunohistochemistry
IHC: CD31 [positive/negative], CD34 [positive/negative], confirming vascular nature
Clinical Significance
Clinical significance: [risk level] for fetal complications based on size [X] cm
Final Diagnosis
Umbilical cord hemangioma, [size] cm