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RICH

Congenital hemangiomas

RICH and NICH had many similarities, such as appearance, location, size, and sex distribution. The obvious differences in behavior served to differentiate RICH, NICH, and common GLUT1+ infantile hemangioma.

Magnetic resonance imaging (MRI) of the three tumors is quite similar, but some RICH also had areas of inhomogeneity and larger flow voids on MRI and arterial aneurysms on angiography. The histologic appearance of RICH differed from NICH and common GLUT1+ infantile hemangioma, but some overlap was noted among the three lesions.

Synopsis

- small-to-large lobules of capillaries
- moderately plump endothelial cells and pericytes
- lobules surrounded by abundant fibrous tissue.
- central involuting zone(s) characterized by lobular loss, fibrous tissue, with large and abnormal draining channels (50%)
- hemosiderin thrombosis
- cyst formation
- focal calcification
- extramedullary hematopoiesis

Immunochemistry

- With rare exceptions, endothelial cells in RICH (as in NICH) did not express glucose transporter-1 protein (GLUT1)

Variants

- multifocal RICHs (17937433)

Associations

- chorangioma (17937433)
- infantile hemangioma (17937433)

See also

- vascular tumors

  • congenital hemangioma
    • NICH (non-involuting congenital hemangioma)

References

- Mulliken JB, Bischoff J, Kozakewich HP. Multifocal rapidly involuting congenital hemangioma: A link to chorangioma. Am J Med Genet A. 2007 Oct 15; PMID: 17937433

- Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol. 2003 Nov-Dec;6(6):495-510. PMID: 15018449

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