Definition/General

Introduction:
-Colorectal hemangioma is a benign vascular tumor composed of proliferating endothelial cells forming vascular channels
-It represents <1% of all colorectal tumors
-Most commonly occurs in the rectosigmoid region
-Shows excellent prognosis with complete excision.
Origin:
-Arises from endothelial cells lining blood vessels
-Can originate from submucosal vessels
-May arise from deeper vascular plexuses
-Shows benign proliferation of vascular elements
-Demonstrates hamartomatous nature in some cases.
Classification:
-Classified as capillary hemangioma (small vessels)
-Cavernous hemangioma (large vessels)
-Mixed type hemangioma
-Epithelioid hemangioma (rare)
-Pyogenic granuloma (lobular capillary hemangioma)
-WHO classification: Benign vascular tumor.
Epidemiology:
-Peak incidence in 5th-6th decades
-No gender predilection
-More common in rectosigmoid region (70%)
-Rare entity in colorectum
-Often asymptomatic
-May present as incidental finding on colonoscopy.

Clinical Features

Presentation:
-Rectal bleeding (most common symptom)
-Asymptomatic polyp
-Change in bowel habits
-Abdominal pain (uncommon)
-Iron deficiency anemia
-Tenesmus in rectal location
-Palpable mass on examination.
Symptoms:
-Painless rectal bleeding (60-70% cases)
-Hematochezia
-Chronic blood loss leading to anemia
-Mucus discharge
-Feeling of incomplete evacuation
-Rarely massive bleeding
-Constitutional symptoms rare.
Risk Factors:
-No specific risk factors identified
-Congenital predisposition possible
-Trauma may trigger growth
-Hormonal changes (pregnancy)
-Hereditary hemorrhagic telangiectasia (rare association)
-Blue rubber bleb nevus syndrome.
Screening:
-No specific screening required
-Colonoscopy for symptomatic patients
-CT angiography for large lesions
-MRI for extent evaluation
-Endoscopic ultrasound for submucosal lesions
-Biopsy for histological confirmation.

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Gross Description

Appearance:
-Well-circumscribed vascular mass
-Red to blue coloration
-Soft, compressible consistency
-Sponge-like cut surface
-Blood-filled spaces visible
-Smooth surface if submucosal.
Characteristics:
-Polypoid or sessile morphology
-Hemorrhage may be present
-Thrombosis in some vessels
-Calcification (phleboliths) rare
-Intact overlying mucosa
-Well-demarcated from surrounding tissue.
Size Location:
-Size ranges from 0.5-5 cm typically
-Rectosigmoid region most common (70%)
-Left-sided predominance
-Cecum second most common
-Submucosal location predominant
-Intramural extension possible.
Multifocality:
-Usually solitary (90% cases)
-Multiple lesions in syndrome cases
-Blue rubber bleb nevus syndrome - multiple GI hemangiomas
-Hereditary hemorrhagic telangiectasia - multiple sites
-Isolated colorectal involvement most common.

Microscopic Description

Histological Features:
-Proliferation of endothelial cells forming vascular spaces
-Capillary-type vessels or cavernous spaces
-Single layer of endothelium
-Basement membrane present
-Supporting stroma minimal
-No atypia in endothelial cells.
Cellular Characteristics:
-Bland endothelial cells lining vascular spaces
-Flattened nuclei
-Minimal cytoplasm
-No pleomorphism
-Rare mitoses
-Pericytes around larger vessels
-Smooth muscle in vessel walls.
Architectural Patterns:
-Capillary hemangioma: small, uniform vessels
-Cavernous hemangioma: large, dilated spaces
-Lobular architecture in some cases
-Feeder vessels at periphery
-Fibrous septa between vascular spaces
-Thrombosis may organize.
Grading Criteria:
-No grading system for benign hemangiomas
-Cellularity assessment helps exclude angiosarcoma
-Mitotic activity minimal (<1/10 HPF)
-Nuclear atypia absent
-Endothelial multilayering absent.

Immunohistochemistry

Positive Markers:
-CD31 positive (endothelial marker)
-CD34 positive
-Factor VIII positive
-ERG positive (nuclear)
-FLI-1 positive (nuclear)
-Vimentin positive.
Negative Markers:
-Smooth muscle actin negative (except pericytes)
-Desmin negative
-Cytokeratins negative
-S-100 negative
-c-KIT negative
-EMA negative.
Diagnostic Utility:
-Confirms vascular nature
-CD31/CD34 most reliable endothelial markers
-ERG nuclear staining specific for endothelium
-Rules out other tumors
-Differentiates from GIST
-Excludes epithelial tumors.
Molecular Subtypes:
-No specific molecular subtypes
-Ki-67 low proliferation index (<5%)
-GLUT1 may be positive (infantile type)
-D2-40 positive in lymphatic type
-Prox1 in lymphatic endothelium.

Molecular/Genetic

Genetic Mutations:
-No specific mutations identified consistently
-Somatic mutations rare
-PIK3CA mutations in some cases
-AKT1 mutations rarely
-Different from cutaneous hemangiomas genetically
-Clonal vs reactive nature debated.
Molecular Markers:
-VEGF expression variable
-Angiopoietin-1/Tie-2 pathway involvement
-bFGF expression in some cases
-PDGF in supporting cells
-Low proliferation markers
-Apoptosis markers low.
Prognostic Significance:
-Excellent prognosis with complete excision
-No malignant potential
-Rare recurrence after excision
-Bleeding risk main concern
-Large size increases bleeding risk
-Complete excision curative.
Therapeutic Targets:
-Complete surgical excision
-Endoscopic resection for small lesions
-Segmental resection for large lesions
-Sclerotherapy for selected cases
-Anti-angiogenic therapy not indicated
-Surveillance after incomplete excision.

Differential Diagnosis

Similar Entities:
-Angiosarcoma - malignant, atypical endothelial cells, high mitoses
-Kaposi sarcoma - spindle cells, HHV-8 positive
-Arteriovenous malformation - thick-walled vessels, no tumor
-Pyogenic granuloma - lobular pattern, surface ulceration
-Angiolipoma - mixed vascular and adipose elements.
Distinguishing Features:
-Angiosarcoma: pleomorphic cells, multilayering, high Ki-67
-Kaposi sarcoma: spindle morphology, slit-like spaces, HHV-8+
-AVM: non-neoplastic, thick muscular walls
-Pyogenic granuloma: surface lobular pattern, granulation tissue
-Angiolipoma: mature fat admixed with vessels.
Diagnostic Challenges:
-Small biopsy samples may be inadequate
-Crush artifact in endoscopic biopsies
-Distinguishing from AVM
-Reactive vs neoplastic nature
-Excluding angiosarcoma in cellular areas.
Rare Variants:
-Epithelioid hemangioma - epithelioid endothelial cells
-Spindle cell hemangioma - spindle cell areas
-Microvenular hemangioma - small venular channels
-Hobnail hemangioma - hobnail endothelium
-Anastomosing hemangioma - anastomosing pattern.

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

Colorectal biopsy/resection containing a [size] cm vascular lesion

Diagnosis

Colorectal Hemangioma

Classification

Benign vascular tumor - [capillary/cavernous/mixed] type

Histological Features

Shows proliferation of bland endothelial cells forming vascular channels. No atypia or increased mitotic activity.

Size and Extent

Size: [X] cm, limited to [submucosa/muscularis propria]

Margins

Margins: [involved/uninvolved]

Immunohistochemistry

CD31: positive, CD34: positive, ERG: positive (nuclear), Ki-67: low

Special Studies

Immunohistochemistry confirms vascular differentiation

No molecular testing required

Endothelial markers positive

Final Diagnosis

Colorectal hemangioma, [capillary/cavernous] type