Pediatric conjunctival neoplasms

2024-12-06   Paola Dal Cin, PhD 

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)

Keywords
Epibulbar, epibulbar osseus, phakomatous choristomas, conjunctival nevus, inflamed juvenile conjunctival naevus

Classification

Definition

Conjunctival tumors in children are nearly always benign. and are found in white children more than in African American or Asian children. 1 Hamartomas of the conjunctiva are not neoplastic, but they form tumors, e.g.  epibulbar, epibulbar osseus and phakomatous choristomas. These lesions represent normal tissue(s) in an abnormal location. They can be a solitary lesion or can be associated with Goldenhar’s syndrome. 2
Junctional, subepithelial (stromal), or compound types, are benign localized clonal proliferations of melanocytic nevus cells. So far, a single genetic investigation by immunohistochemistry techniques reported mutually exclusive mutations in BRAF and NRAS, as seen similar in skin nevi. 3 Evolution of conjunctival nevus into malignant melanoma is extremely low (<1%).
Inflamed juvenile conjunctival naevus (IJCN) is a distinctive compound naevus, occurring in patients aged < 18 year exhibiting more NRAS p.Q61R mutations than BRAF p.V600E mutations (by immunohistochemistry). 3 A single case with BRAF p.V600E mutation was identified. 4

Article Bibliography

Reference NumberPubmed IDLast YearTitleAuthors
1281257592017Clinical Features Differentiating Benign From Malignant Conjunctival Tumors in Children.Shields CL et al
2302065522018Goldenhar Syndrome - ophthalmologist's perspective.Schmitzer S et al
3293321232018BRAF, NRAS, and GNAQ Mutations in Conjunctival Melanocytic Nevi.Francis JH et al
4161233972005T1799A BRAF mutations in conjunctival melanocytic lesions.Goldenberg-Cohen N et al

Citation

Paola Dal Cin, PhD

Pediatric conjunctival neoplasms

Atlas Genet Cytogenet Oncol Haematol. 2024-12-06

Online version: http://atlasgeneticsoncology.org/solid-tumor/209301/pediatric-conjunctival-neoplasms