Definition/General

Introduction:
-Hepatic hemangioma is the most common benign liver tumor
-It represents 80-90% of benign hepatic lesions
-FNAC is typically contraindicated due to bleeding risk
-When performed, it shows characteristic vascular cytomorphology with minimal cellularity.
Origin:
-Arises from hepatic blood vessels (capillaries and venules)
-Represents a developmental vascular malformation rather than true neoplasm
-Composed of endothelial-lined vascular channels
-Supported by fibrous stroma
-May enlarge during pregnancy due to hormonal stimulation.
Classification:
-Classified as cavernous hemangioma (most common type)
-Capillary hemangioma (rare in adults)
-Sclerosed hemangioma (involuted form)
-Size classification: small (<4 cm) vs large (>4 cm)
-Giant hemangiomas (>10 cm)
-Atypical hemangiomas with unusual imaging features.
Epidemiology:
-Most common benign liver tumor (prevalence 1-20%)
-Female predominance (F:M = 3-5:1)
-Peak detection in 4th-5th decades
-Often incidental finding on imaging
-Congenital lesions may present in infancy
-Association with pregnancy and estrogen exposure
-Indian population: increasing detection with widespread imaging.

Clinical Features

Presentation:
-Usually asymptomatic (>80% cases)
-Incidental finding on imaging studies
-Large hemangiomas may cause abdominal discomfort
-Right upper quadrant pain (stretching liver capsule)
-Hepatomegaly (giant hemangiomas)
-Rarely: consumptive coagulopathy (Kasabach-Merritt syndrome).
Symptoms:
-Most patients completely asymptomatic
-Vague abdominal fullness (large lesions)
-Nausea (rare, large tumors)
-Early satiety (mass effect)
-Abdominal pain (atypical, controversial)
-Thrombocytopenia (giant hemangiomas, rare)
-No constitutional symptoms typically.
Risk Factors:
-Female gender (hormonal influence)
-Pregnancy (estrogen stimulation causes growth)
-Oral contraceptive use
-Hormone replacement therapy
-Multiple pregnancies
-Age >40 years (increased detection)
-Familial clustering (rare cases)
-No known environmental risk factors.
Screening:
-Usually detected incidentally on imaging
-Ultrasound: echogenic lesion with posterior enhancement
-CT triphasic: peripheral nodular enhancement, centripetal fill-in
-MRI (gold standard): T2 hyperintense, characteristic enhancement
-Technetium-99m RBC scan: persistent uptake
-FNAC generally avoided.

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Gross Description

Appearance:
-FNAC (when performed) yields hemorrhagic aspirate
-Paucicellular specimen with blood predominance
-May show fibrous tissue fragments
-Rare endothelial cell clusters
-Hemosiderin-laden macrophages may be present
-Background shows extensive red blood cells.
Characteristics:
-Bloody aspirate with minimal cellular material
-Thin consistency due to blood dilution
-May contain fibrous connective tissue
-Rare smooth muscle fragments
-Calcifications in sclerosed areas
-Cholesterol clefts (rare)
-Background predominantly hemorrhagic.
Size Location:
-Variable size: from few mm to >20 cm
-Most common location: right hepatic lobe
-Subcapsular location frequent
-Multiple hemangiomas in 10-15% cases
-Giant hemangiomas may occupy entire lobe
-Pedunculated forms rarely seen.
Multifocality:
-Solitary lesion in 85-90% cases
-Multiple hemangiomas (hemangiomatosis) in 10-15%
-Bilobar involvement in multiple cases
-Associated with cutaneous hemangiomas (rare)
-Extrahepatic hemangiomas (spleen, kidney)
-Familial multiple hemangiomas (very rare).

Microscopic Description

Histological Features:
-Paucicellular aspirate with predominant blood
-Rare endothelial cells lining vascular spaces
-Flat, bland endothelial cells with oval nuclei
-Fibrous tissue fragments with spindle cells
-Smooth muscle cells from vessel walls
-Hemosiderin deposits in chronic lesions.
Cellular Characteristics:
-Endothelial cells: flat, elongated with bland nuclei
-Smooth muscle cells: spindle-shaped, eosinophilic cytoplasm
-Fibroblasts: spindle cells in fibrous areas
-No cellular atypia or pleomorphism
-Minimal mitotic activity
-Background inflammatory cells rare.
Architectural Patterns:
-Vascular pattern with blood-filled spaces
-Cavernous pattern: large vascular channels
-Capillary pattern: small vessel proliferation (rare)
-Sclerotic pattern: fibrous replacement
-Thrombosed areas with organization
-Calcified areas in old lesions.
Grading Criteria:
-No grading system for hemangiomas
-Assessment based on vascular pattern
-Cavernous type: large blood-filled spaces
-Capillary type: small proliferating vessels
-Sclerosed type: fibrous replacement
-Thrombosed areas: organized thrombi
-Calcification degree in chronic lesions.

Immunohistochemistry

Positive Markers:
-CD31 (endothelial marker, highlights vascular lining)
-CD34 (endothelial and vascular marker)
-ERG (endothelial nuclear marker)
-Factor VIII (endothelial marker, less specific)
-D2-40 (lymphatic endothelium, if present)
-Smooth muscle actin (vessel wall smooth muscle).
Negative Markers:
-Cytokeratins (negative, excludes carcinoma)
-Hepatocyte marker (negative)
-CD68 (excludes Kupffer cell tumor)
-Desmin (negative in endothelium)
-S-100 (negative, excludes neural tumors)
-Chromogranin/synaptophysin (negative).
Diagnostic Utility:
-Limited utility in FNAC setting due to paucicellular nature
-CD31/CD34 can confirm vascular nature
-Helps distinguish from other vascular lesions
-Excludes malignancy (negative epithelial markers)
-Not routinely performed due to bleeding risk
-Histology preferred for definitive diagnosis.
Molecular Subtypes:
-No specific molecular subtypes recognized
-Hormonal receptors (estrogen, progesterone) may be present
-VEGF expression in growing lesions
-No specific genetic alterations identified
-Familial cases: possible genetic predisposition
-Research focus on angiogenesis factors.

Molecular/Genetic

Genetic Mutations:
-No specific somatic mutations identified
-Developmental origin suggests germline factors
-Hormonal influence: estrogen receptor pathway
-Angiogenesis-related gene expression
-Familial clustering: possible genetic predisposition
-No tumor suppressor gene involvement.
Molecular Markers:
-VEGF (vascular endothelial growth factor) expression
-Angiopoietin pathway activation
-Endothelial markers: CD31, CD34, ERG expression
-Hormonal receptors: ER, PR in some cases
-Basic FGF (fibroblast growth factor)
-Thrombospondin (anti-angiogenic factor).
Prognostic Significance:
-Excellent prognosis (benign lesion)
-Size correlation: larger lesions more symptomatic
-Pregnancy effect: may enlarge during pregnancy
-Malignant transformation extremely rare (not reported)
-Complications rare except in giant hemangiomas
-Spontaneous regression may occur.
Therapeutic Targets:
-Observation for asymptomatic lesions
-Propranolol (beta-blocker) for infantile hemangiomas
-Corticosteroids (historical use)
-Anti-angiogenic therapy (experimental)
-Hormonal manipulation (avoiding estrogen)
-Surgical resection for symptomatic giant lesions.

Differential Diagnosis

Similar Entities:
-Hepatocellular carcinoma (hypervascular HCC)
-Focal nodular hyperplasia (FNH with central scar)
-Hepatic adenoma (hormonal association)
-Metastatic hypervascular tumors (neuroendocrine, RCC)
-Angiosarcoma (malignant vascular tumor)
-Arteriovenous malformation.
Distinguishing Features:
-Hemangioma: centripetal enhancement, T2 hyperintense on MRI
-HCC: arterial enhancement with washout, AFP elevation
-FNH: central scar, Kupffer cell uptake on sulfur colloid
-Adenoma: fat content, oral contraceptive history
-Angiosarcoma: rapid growth, irregular enhancement
-Metastases: multiple lesions, known primary.
Diagnostic Challenges:
-Distinguishing from hypervascular HCC on imaging
-Atypical hemangiomas with unusual enhancement
-Small hemangiomas vs micrometastases
-Flash-filling hemangiomas vs other hypervascular lesions
-FNAC interpretation (when performed) challenging
-Sclerosed hemangiomas vs fibrotic lesions.
Rare Variants:
-Capillary hemangioma (more common in children)
-Sclerosed/hyalinized hemangioma
-Thrombosed hemangioma with calcification
-Giant hemangioma with mass effect
-Flash-filling hemangioma
-Hemangiomatosis (multiple lesions)
-Pedunculated hemangioma.

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.

Clinical Information

Patient with liver lesion on imaging, [clinical presentation]

Procedure Note

FNAC generally contraindicated for suspected hemangioma due to bleeding risk

Specimen Adequacy

Paucicellular specimen, predominantly hemorrhagic

Cytomorphological Features

Shows [blood, rare endothelial cells, fibrous fragments]

Imaging Correlation

Imaging shows [characteristic hemangioma features]

Differential Diagnosis

Differential includes [other vascular lesions, hypervascular tumors]

Final Cytological Diagnosis

Paucicellular vascular lesion, consistent with hemangioma (imaging correlation recommended)

Recommendations

Recommend imaging-based diagnosis, avoid repeat FNAC