Cutaneous vascular tumors (overview) L98.9

Common classifications of cutaneous vascular tumors are based on morphologic, clinical, and histologic criteria. Vascular proliferations that appear clinically as "angiomatous tumors" are often unclear as to whether they are:

• autochthonous neoplasms
• neoplasia-like reactive proliferations
• vascular malformations (vascular hamartomas)

are involved. Hemangioma of the infant occupies a special etiopathogenetic and clinical position.

• Hamartomas and malformations:
  • Hamartoma, eccrine, angiomatous
  • Malformations, arteriovenous
  • Lymphangiomas, superficial and deep:
    • Lymphangiectasia, cutaneous
    • Lymphangiokeratoma
    • lymphangioma cavernosum
    • lymphangioma circumscriptum
    • Lymphangiomatosis.
  • Capillary malformations:
    • Naevus flammeus:
      • Naevus flammeus lateralis
      • medial nevus flammeus
      • Angiokeratoma circumscriptum (verrucous hemangioma)
    • Cutis marmorata teleangiectatica congenita.
• dilatations of pre-existing vessels:
  • Naevus araneus
  • Angioma, seniles of the lips (venous lake)
  • Angiokeratomas (with enzymopathy):
    • Angiokeratoma corporis diffusum (Fabry)
    • Fucosidosis.
  • Angiokeratomas (without enzymopathy):
    • Angiokeratoma Mibelli
    • Angiokeratoma scroti et vulvae (Fordyce)
    • Angiokeratome, solitary
    • Angiokeratoma circumscriptum (verrucous hemangioma)
  • Teleangiectasiasand teleangiectatic diseases.
• Cavernous malformations:
  • Hemangioma, cavernous.
• Hemagiomas and reactive proliferations:
  • Blue Rubber Bleb Nevus Syndrome
  • infant hemangioma
  • Hemangioma, tufted
  • Hemangioma, microvenous
  • Gemmangioma
  • Granuloma pyogenicum
  • Angiomatosis, bacillary
  • Bartonelless tickets
  • Acroangiodermatitis
  • Hemangioma, tardives
  • Angiomatosis
  • Angioendotheliomatosis, reactive (benign)
  • Hyperplasia, intravascular, papillary, endothelial
  • Hemangioma, glomeruloid
  • Angiolymphoid hyperplasia with eosinophilia
  • Dermatofibroma, epitheloid cell
  • Hemangioma, targetoid, hemosiderotic
  • Hemangioma, spindle cell hemangioma
  • Angioma serpiginosum.
• borderline and mildly malignant vascular tumors:
  • hemangioendotheliomas:
    • Hemangioendothelioma, Kaposi's form
    • Hemangioendothelioma, retiform
    • Hemangioendothelioma, type Dabska
    • Hemangioendothelioma, epitheloid
  • Kaposi's sarcoma.
• Malignant vascular tumors:
  • angiosarcomas:
    • Idiopathic/classical angiosarcoma in older people
    • lymphedema-associated angiosarcoma
    • Angiosarcoma after radiotherapy
    • Angiosarcoma, epitheloid.
• Angiomas with glomoid, pericyte or smooth muscle differentiation:
  • Glomus tumor (see below glomus angiomatosis)
  • Hemangiopericytoma
  • Haemangiopericytoma, infantiles (myopericytoma, myofibroma)
  • Angioleiomyoma.
• Mesenchymal tumors with dominant vascular component:
  • Angiolipoma
  • Neurofibroma
  • Angiofibroma
  • Giant cell angiofibroma
  • Giant cell angiohistiocytoma.

Structural composition of vascular tumours:

• Vascular orientation (intra- or perivascular)
• Architecture (lobar or nodular/diffuse)
• Endothelial pattern (single layer or papillary multilayer)
• Vascular structure (organoid capillary vessels or bizarre vascular structures).

immunohistology:

• Immunohistologically intravascular angiomas are defined as descendants of blood or lymph vessel endothelia, perivascular angiomas as descendants of smooth muscle cells or pericytes.
• Markers for endothelia are: CD31, ERG, FLI-1, factor VII-related antigen/v. Willebrand factor), CD34, Ulex europaeus.
• Markers for lymphatic endothelia are: D2-D40/Podoplanin, Prox1, LYVE1, VEGFR3 (vascular endothelial growth factor-3)
• In perivascular angiomas, reactivities for alpha smooth muscle actin, desmin, calponin, caldesmon are found.

1. Kutzner H (2003) Vascular tumors of the skin. In: Kerl H et al (Ed.) Histopathology of the skin. Springer Publishing House, Berlin Heidelberg New York, S. 759
2. North PE et al (2003) Vascular neoplasms and neoplastic - like proliferations. In: Bologna J et al (ed.) Dermatology. Mosby, London New York Toronto, S. 1818