Malignant fibrous histiocytoma of the skin and the conjunctiva in xeroderma pigmentosum.
Patients with xeroderma pigmentosum (XP) experience cutaneous and ocular abnormalities, including neoplasia of epithelial and melanocytic cellular origin. Black patients with XP display more severe ocular abnormalities and neoplasia. We report the clinicopathologic findings in two black young children with XP who presented with malignant fibrous histiocytoma (one of the conjunctiva and the other one of the skin of the face). These appearances of malignant fibrous histiocytoma at an unusually early age and in rare locations are, to our knowledge, the first to be reported in XP and go along with the special characteristics of XP: frequent occurrence of tumors on exposed parts of the body at a very early age.
Pe'er
J
J
Department of Ophthalmology, Hadassah University Hospital, Jerusalem, Israel.
Levinger
S
S
Chirambo
M
M
Ron
N
N
Okon
E
E
D002675:Child, Preschool; D003230:Conjunctival Neoplasms / Q000473:pathology*; D005154:Facial Nerve / Q000473:pathology*; D005260:Female; D018219:Histiocytoma, Benign Fibrous / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010858:Pigmentation; D012878:Skin Neoplasms / Q000473:pathology*; D014983:Xeroderma Pigmentosum / Q000473:pathology*