Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Qiao J. Venous hemangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuevenoushemangioma.html. Accessed December 16th, 2024.
Definition / general
- Also called venous malformation (VM)
- Rare slow growing benign tumor of aberrant and ectatic venous connections
- Vascular anomalies, including venous hemangiomas, are congenital lesions of abnormal vascular development
Essential features
- Mainly present in adults
- Tumor present in the subcutaneous or deep soft tissues with predilection for the limbs
- Also present as a long standing slow growing tumor
- Rapid growth may occur during puberty, pregnancy or traumatic injury
- Calcifications can be seen in these tumors due to phleboliths (calcified thrombi)
- Deep seated tumor is difficult to excise and can recur locally but subcutaneous tumors do not show a tendency to recur
Terminology
- Venous hemangioma / venous malformation
Epidemiology
- Incidence ~ 1 in 10,000
- Most occur sporadically
- Both men and women are affected
Sites
- Mostly in adult limbs
Pathophysiology
- Aberrant venous connections lead to venous congestion, thrombosis, phlebolith formation and gradual expansion of these lesions
Etiology
- Vascular / venous malformation
- Inherited forms of venous hemangiomas are autosomal dominant disorders and have been localized to chromosome 9p
- Mutation of angiopoetin receptor gene TIE2 / TEK was recently discovered in multiple sporadic venous hemangiomas
Clinical features
- Visible at birth but presents as a deep mass
- Overlying skin may appear normal or possess a bluish discoloration
- With more cutaneous involvement, the lesions appear darker blue or purple
Diagnosis
- Calcifications can be seen in the radiological examinations due to phleboliths (calcified thrombi) within venous hemangiomas
- MRI is the imaging modality of choice for diagnosing venous hemangioma; it offers superior delineation of the disease, which is necessary for treatment planning
Laboratory
- D dimer may be elevated and a marker of disease
Radiology description
- Radiographic studies, angiogram and MRI
Prognostic factors
- Deep seated tumor is difficult to excise and can recur locally
- Subcutaneous tumors do not show a tendency to recur
Treatment
- Different treatment modalities based on size, location and patient age, including periodic observation, embolization, sclerotherapy, radiofrequency ablation and wide surgical excision
Gross description
- Hemorrhagic cutaneous or deep soft tissue mass with ill defined and dilated vessels
Microscopic (histologic) description
- Typically consists of closely packed thick walled vessels, which are variably dilated and commonly display thrombosis with occasionally formation of phleboliths (calcified thrombi)
- Vein valves are present
- Occasional hemosiderin interposition can be seen in vessel walls
Microscopic (histologic) images
Additional references
Board review style question #1
Which statements are true about venous hemangioma?
- It can grow rapidly during pregnancy
- The deep soft tissue tumor can be entirely excised without recurrence
- The tumor can be found in subcutaneous tissue or deep soft tissue
- Venous hemangioma is a slow growing tumor
- (1,3,4)
- (1,4)
- (2,3)
- (1,2,3,4)
Board review style answer #1
A. (All but 2 are correct; 2 is wrong, since deep seated tumors are difficult to excise and can recur locally)
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Reference: Venous hemangioma
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Reference: Venous hemangioma