Vascular tumours

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This article covers soft tissue vascular tumours. Vascular malformations are covered in the vascular malformations article.

Normal histology

Normal blood vessel histology is dealt with in the vascular disease article.

Mimics

Distinct entities

Hemangioma

General

  • May be found in the liver.
    • Classically subcapsular.
      • May rupture and be life-threatening.[1]

Hemangiomas to remember - if you're only going remember a few:

  • Glomeruloid, infantile, caverous, capillary, arteriovenous, venous and intramuscular.

Childhood

Common childhood hemangiomas:[2]

  • Tufted - small clusters of blood vessels.
  • Microvenular hemangioma.
  • Glomeruloid hemangioma - associated with POEMS syndrome, Castleman disease.[3][4]
  • Epithelioid hemangioma.
  • Targetoid hemosideric hemangioma.
  • Infantile hemangioma (AKA juvenile hemangioma[5]) - these tumours are GLUT-1 +ve. They tumours grow and then spontaneously regress.[6]

Soft tissue

Several types are seen in soft tissue:[7]

  • Capillary.
  • Cavernous.
  • Arteriovenous.
  • Venous.
  • Intramuscular.
  • Synovial.

Microscopic

Features:

  • Channels lined by endothelium containing RBCs.

DDx:

Images:

IHC

  • CD31 +ve.
  • D2-40 -ve.[8]

Juvenile hemangioma:[5]

  • GLUT-1 +ve.

Lymphangioma

General

Treatment:

  • Surgical excision.

Microscopic

Features:[10][9]

  • Thin-walled channels lined by endothelium.
  • +/-Eosinophilic intraluminal material.
  • +/-Clusters of intraluminal lymphocytes.
  • +/-Occasional RBCs.

DDx:

Images:

IHC

Features:[11][8]

  • D2-40 +ve.

Kaposi sarcoma

  • Abbreviated KS.

General

  • Caused by Human herpesvirus-8 (HHV-8).
  • In the North American context, it is often associated with immunodeficiency, e.g. HIV/AIDS.

Interesting note:

  • It has been said that KS is not really a sarcoma.[12]

Stages

It is seen in different stages:[13][14]

  1. Patch stage.
  2. Plaque stage.
  3. Nodular stage.
  4. Exophytic stage.
  5. Infiltrative stage.
  6. Lymphadenopathic stage.

Note:

  • The first three are the classic ones.

Type or form

Classically divided into four types:[15][16][17]

  • Classic = old men Mediterranean or Ashkenazi Jew.
  • Endemic = African infants and young males.
  • Immunosuppression-associated or transplant-associated - iatrogenic.
  • AIDS-associated.

Microscopic

Features:[18]

  • Vascular lesion (abundant RBCs) with:
    • +/-"Promontory sign" - small vessel protruding into an abnormal vascular space.[19]
      • Not pathognomonic for KS.[20]
    • +/-Spindle cells with minimal nuclear atypia.
    • RBC extravasation - very useful - important feature.[21]
  • +/-Intracytoplasmic hyaline globules - uncommon - one usu. needs to search for 'em.[22]
    • Pale pink globs (that are paler than RBCs) - important feature.
  • +/-Hemosiderin deposits.
  • +/-Plasma cells.[23]

Notes:

DDx:

  • Angiosarcoma - have many mitoses, nuclear atypia, RBC extravasation not common.
  • Masson's hemangioma - AKA intravascular papillary endothelial hyperplasia.
  • Benign lymphangioendothelioma.[24]
    • Histologically very similar.[25]

Images:

Stains

  • PAS +ve -- hyaline globules.

IHC

  • CD31 +ve.
  • CD34 +ve.
  • HHV-8 +ve.

Masson hemangioma

  • AKA intravascular papillary endothelial hyperplasia.[26]
  • AKA Masson tumor.[27]

General

  • Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
  • Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.[26]

Microscopic

Features:

  • Well-circumscribed - key (low power) feature.
  • Abundant small vascular channels with benign endothelium.
  • +/-Papillary formation with a fibrotic core covered by benign endothelium.[28]

Notes:

Images:

Angiosarcoma

General

  • Malignant tumour - with a horrible prognosis.[29]
  • Classically on the scalp or head & neck.

Epidemiology:

  • May arise secondary to therapeutic radiation or chronic lymphoedema related to breast carcinoma.
  • Liver angiosarcomas are assoc. with vinyl chloride exposure.[30]

Microscopic

Features:

  • Very many small capillaries of irregular shape lined with:
    • Pleomorphic nuclei.
      • May have hobnail morphology.
  • Mitoses.
  • Cytoplasmic vacuoles.
    • Cells trying to form lumina - embryologic.

Notes:

Images:

IHC

  • CD34 +ve.
  • CD31 +ve.
  • FLI-1 +ve.[31]
  • D2-40 +ve/-ve.[8]

Kaposiform hemangioendothelioma

General

  • Locally aggressive.[32]
  • Childhood tumour.[33]
  • Approximately half have Kasabach–Merritt phenomenon[33] = vascular tumour --> coagulopathy.

Microscopic

Features:[34]

  • Spindle cells lesions in sheets or nodules.
  • +/-Round tumour nodules - "cannon ball" appearance.

DDx:

IHC

Features:[34]

  • Vimentin +ve.
  • C31 +ve.
  • CD34 +ve.
  • UEA-1 lectin +ve.

Epithelioid hemangioendothelioma

General

  • Malignant.[32]
  • Adults - wide age range.
  • Associated with oral contraceptives, vinyl chloride.[35]

Microscopic

Features:[35]

  • Large epithelioid perivascular cells with:
    • Abundant pale eosinophilic cytoplasm.
    • Cytoplasmic vacuolation (some cells) - AKA "blister cells" - key feature.
      • May form lumen and have RBC within.
    • Vesicular nucleus with prominent nucleolus in some cells.
  • Tuft-like projections into capillaries.
  • Tumour cells may be in well-circumscribed paucicellular nodules or more cellular poorly formed aggregates.

DDx:

Images:

IHC

Features:[35]

  • CD31 +ve.
  • CD34 +ve.
  • Factor VIII +ve.

See also

References

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  2. Prieto VG, Shea CR (July 1999). "Selected cutaneous vascular neoplasms. A review". Dermatol Clin 17 (3): 507–20, viii. PMID 10410855.
  3. Uthup S, Balachandran K, Ammal VA, et al. (August 2006). "Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma". Am. J. Kidney Dis. 48 (2): e17–24. doi:10.1053/j.ajkd.2006.04.089. PMID 16860182.
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  20. Fernandez-Flores A, Rodriguez R (June 2010). "Promontory Sign in a Reactive Benign Vascular Proliferation". Am J Dermatopathol. doi:10.1097/DAD.0b013e3181cf0ae5. PMID 20577080.
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