Hemangioma pathophysiology

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

Overview

Development of hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis. Hemangioma may be associated with POEMS syndrome and Castleman disease. On gross pathology, spongy with vascular compartments of various sizes separated by fibrous tissue are findings of hemangioma. On microscopic histopathological analysis, channels lined by benign endothelium containing red blood cells are findings of hemangioma.

Pathophysiology

The pathogenesis of hemangiomas has not been elucidated, but there are two competing theories;

First theory

Second theory

  • The second theory is that the presence of liver hemangiomas involves a genetic background of mutations.[1]
  • Genetic errors in growth factor receptors have also been shown to affect development of hemangiomas.
  • Metalloproteinases can accumulate in the endoplasmic reticulum of the tumor cells causing:
    • Self-digestion
    • Vacuole formation
  • Cavernous hemangioma cell can downregulate Derlin-1.
    • Derlin-1 is a protein that when overexpressed induces the dilated endoplasmic reticulum to return to its normal size.

Third theory

  • The third theory suggests that hemangioma endothelial cells arise from disrupted placental tissue imbedded in fetal soft tissues during gestation or birth.[2]
  • Markers of hemangiomas have been shown to coincide with those found in placental tissue.
  • This is further supported by the fact that they are found more commonly in infants following:[2]

Growth Pattern

  • Hemangiomas follow a predictable course with three distinct developmental phases:[2]
Proliferation phase
  • In most hemangiomas, eighty percent of proliferation occurs by three months of life but may last longer.[2][3]
  • During proliferation, rapid growth can lead to exhaustion of blood supply with resulting ischemia, necrosis, ulceration, and bleeding.
Quiescence phase
  • Following proliferation, hemangiomas enter a slower or no growth phase, known as quiescence.[2]
  • This phase typically lasts from nine to twelve months of age.
Involution phase
  • The final and unique phase of the hemangioma lifecycle is involution.[2]
  • This phase is marked by graying of the overlying skin and shrinking of the deeper components.
  • At the final stages of involution, a fibrofatty protuberance may remain.

Associated Conditions

Hemangioma may be associated with:

Gross Pathology

  • Grossly hemangiomas are described as “spongy” with vascular compartments of various sizes separated by fibrous tissue.[1]
  • Thrombi may be present and are well separated from the normal liver parenchyma despite the absence of a fibrous capsule.

Microscopic Pathology

On microscopic histopathological analysis channels lined by benign endothelium containing red blood cells are findings of hemangioma.[3]

Gallery

Immunohistochemistry

Hemangioma is demonstrated by positivity to:[3]

  • CD31 positive
  • D2-40 negative

References

  1. 1.0 1.1 1.2 1.3 Papafragkakis, Haris; Moehlen, Martin; Garcia-Buitrago, Monica T.; Madrazo, Beatrice; Island, Eddie; Martin, Paul (2011). "A Case of a Ruptured Sclerosing Liver Hemangioma". International Journal of Hepatology. 2011: 1–5. doi:10.4061/2011/942360. ISSN 2090-3456.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Richter, Gresham T.; Friedman, Adva B. (2012). "Hemangiomas and Vascular Malformations: Current Theory and Management". International Journal of Pediatrics. 2012: 1–10. doi:10.1155/2012/645678. ISSN 1687-9740.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Microscopic features of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015

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