Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0043346 (
xeroderma pigmentosum
)
2,924
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
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.
...
PMID:Malignant fibrous histiocytoma of the skin and the conjunctiva in xeroderma pigmentosum. 165 10
Malignant fibrous histiocytoma
(
MFH
) of the conjunctiva is an extremely rare tumour, and only three previous cases have been reported. We describe two patients with
MFH
of the conjunctiva: a 58-year-old white male with epibulbar tumour who had exenteration and is alive after five years' follow-up, and a 3 1/2-year-old African girl with
xeroderma pigmentosum
and an
MFH
of her right eye conjunctiva, the first reported case of this association. The characteristics and the methods of diagnosis of
MFH
are discussed.
...
PMID:Malignant fibrous histiocytoma of the conjunctiva. 170 95
