Malignant schwannoma associated with xeroderma pigmentosum in a patient belonging to complementation group D.
A 43-year-old man with xeroderma pigmentosum, XP97TO, was allocated to complementation group D. He had had moderate photosensitivity at age 1 year and freckles by age 6 but no neurologic abnormalities. Nevertheless, his fibroblasts in culture had the XP-D phenotype. They showed a sevenfold hypersensitivity to killing by 254 nm ultraviolet radiation and a diminished level (29%) of unscheduled DNA synthesis. Phototesting revealed delayed maximum erythema at 72 hours after UVB exposure and a lowered minimal erythema dose. Lentigo maligna developed on the patient's face, and a rapidly growing malignant schwannoma was found on the left trigeminal nerve. This may be the first case of a peripheral nervous tissue neoplasm in xeroderma pigmentosum.
Nakamura
T
T
Department of Dermatology, Kumamoto University Medical School, Japan.
Ono
T
T
Yoshimura
K
K
Arao
T
T
Kondo
S
S
Ichihashi
M
M
Matsumoto
A
A
Fujiwara
Y
Y
D000328:Adult; D003390:Cranial Nerve Neoplasms; D005148:Facial Dermatoses / Q000145:classification / Q000473:pathology; D005153:Facial Neoplasms / Q000473:pathology; D006801:Humans; D008297:Male; D008545:Melanoma / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000473:pathology; D009442:Neurilemmoma / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology; D014276:Trigeminal Nerve; D014983:Xeroderma Pigmentosum / Q000145:classification / Q000473:pathology