Benign vascular tumor

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For information about classification of vascular tumors, click here.

Vascular Tumor

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Overview

Classification

Benign Vascular tumor
Locally aggressive or borderline vascular tumors
Malignant vascular tumors

Benign Vascular Tumor Home Page

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]


Overview

Benign vascular tumors, are benign growths formed from blood vessels; such as, hemangioma, hemangioendothelioma, Kaposi sarcoma. They exhibit a wide range of clinical manifestations, and may occur as isolated lesions or may occur as manifestation of multi-system syndromes and diseases. Their diagnosis and management depends on their clinical manifestations and coexistent anomalies. International Society for the Study of Vascular Anomalies (ISSVA) has classified these lesions into benign vascular tumors and related lesions.

Benign Vascular Tumors

Infantile hemangioma / Hemangioma of infancy

Pattern Different types
  • Focal
  • Multifocal
  • Segmental
  • Indeterminate
  • Superficial
  • Deep
  • Mixed (superficial + deep)
  • Reticular / abortive / minimal growth
  • Others
Adapted from International Society for the Study of Vascular Anomalies[1]
Association with other lesions
PHACE association /

syndrome

Posterior fossa malformations, Hemangioma, Arterial

anomalies, Cardiovascular anomalies, Eye anomalies ,

sternal clefting and ⁄ or supraumbilical raphe

LUMBAR (SACRAL,

PELVIS) association /

syndrome

Lower body hemangioma, Urogenital anomalies,

Ulceration, Myelopathy, Bony deformities, Anorectal

malformations, Arterial anomalies, and Renal anomalies

Adapted from International Society for the Study of Vascular Anomalies[1]

Congenital hemangioma

  • Rare tumor that arises in utero and presents as fully developed lesion at birth. Following birth they can regress completely, partially or not at all. So they can be classified as rapidly involuting (RICH), non-involuting (NICH), partially involuting (PICH).[17][18]
    1. Rapidly involuting (RICH)
    2. Non-involuting (NICH)
      • Fast flow tumor that presents as well defined, plaque like lesion with pink to purple color, telangiectasias and pale borders.
      • Typically remains stable but there have been some reports of growth and expansion.[18]
    3. Partially involuting (PICH)
      • These lesions start involution as RICH but become stable over time and persist as NICH.[20]
  • Somatic mutations in GNAQ/GNA11 are thought to cause the congenital hemangioma. GNAQ and its paralogue GNA11 function in intracellular signaling pathways as Gq alpha subunit.[18][21]
  • Diagnosis is usually clinical but imaging techniques such as MRI, CT scan, contrast-enhanced ultrasound and later biopsy can be considered if required. Surgical excision should be considered in case of complications, NICH and PICH.[22][23]

Tufted angioma

For more information on tufted angioma, click here

Spindle-cell hemangioma

  • Rare benign tumor that manifests as solitary or multiple nodules confined to dermis and subcutaneous tissues in almost all of the cases. Histopathologically, it appears as solid areas that are cellular and consist of spindle cells seen attached to vessel walls, and cavernous spaces that can be thrombosed. Size may increase over time and patient usually complains of swelling and pain. The nodules or masses can be mobile and elastic or can be firm and immobile.[36][37][38]
  • Somatic mutations in IDH1 and IDH2 have been found to be present in 70% of spindle-cell hemangiomas. IDH1 and IDH2 are important enzymes in cell energy cycles (α-ketoglutarate and NADPH generation).[39][40]
  • Diagnosis often requires biopsy and imaging studies such as MRI to ascertain the extent of the tumor. Local excision is the treatment modality of choice with excellent prognosis in majority of the cases although recurrence is very common.[36][37][38][41][42]

Epithelioid hemangioma

For more information on epithelioid hemangioma, click here

Pyogenic granuloma

For more information on Pyogenic granuloma, click here.

Hobnail hemangioma

Microvenular hemangioma

  • Rare lesion that most often manifests as single asymptomatic nodule, plaque or papule with color varying from red to bluish-red. Majority of the lesions are located on trunk and limbs. Histologically, the tumor consists of irregular and branching venous structures with inconspicuous lumina. Endothelial cells display absence of atypia and mitotic figures. Some lesions may be painful and/or tender.[68][69][70][71]
  • Etiology and pathogenesis have not been well-understood but a recent study may associate progesterone with microvenular hemangioma.[72]
  • Diagnosis requires biopsy because of rarity of this tumor. Treatment is through surgical excision.[68][69][70][71]

Anastomosing hemangioma

Glomeruloid hemangioma

Papillary hemangioma

Intravascular papillary endothelial hyperplasia

Cutaneous epithelioid angiomatous nodule

Acquired elastotic hemangioma

Littoral cell hemangioma of the spleen

Related lesions

Eccrine angiomatous hamartoma

Reactive angioendotheliomatosis

Bacillary angiomatosis

For more information on bacillary angiomatosis, click here.

References

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