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Titolo:
LHERMITTE-DUCLOS DISEASE AND COWDENS-SYNDROME IN AN ADOLESCENT PATIENT - CASE-REPORT
Autore:
WELLS GB; LASNER TM; YOUSEM DM; ZAGER EL;
Indirizzi:
HOSP UNIV PENN,DEPT PATHOL & LAB MED,DIV NEUROPATHOL,3400 SPRUCE ST,6FOUNDERS PAVIL PHILADELPHIA PA 19104 HOSP UNIV PENN,DEPT RADIOL,NEURORADIOL SECT PHILADELPHIA PA 19104
Titolo Testata:
Journal of neurosurgery
fascicolo: 1, volume: 81, anno: 1994,
pagine: 133 - 136
SICI:
0022-3085(1994)81:1<133:LDACIA>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
MULTIPLE HAMARTOMA; DYSPLASTIC GANGLIOCYTOMA; ASSOCIATION; CEREBELLUM;
Keywords:
LHERMITTE-DUCLOS DISEASE; COWDENS SYNDROME; CEREBELLUM; MULTIPLE HAMARTOMA SYNDROME;
Tipo documento:
Note
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
22
Recensione:
Indirizzi per estratti:
Citazione:
G.B. Wells et al., "LHERMITTE-DUCLOS DISEASE AND COWDENS-SYNDROME IN AN ADOLESCENT PATIENT - CASE-REPORT", Journal of neurosurgery, 81(1), 1994, pp. 133-136

Abstract

Recent reports of seven cases of Lhermitte-Duclos disease occurring in adult patients with Cowden's syndrome (multiple hamartoma syndrome) strongly suggest that Lhermitte-Duclos disease is one of the types of neoplasia that characterize this syndrome. A case of Lhermitte-Duclos disease is reported in a 16-year-old girl with craniomegaly, choroidalhamartoma, and conjunctival papilloma of the right eye, and a historyof bilateral multinodular adenomatous goiter and cystic hygroma. These findings strongly suggest a diagnosis of Cowden's syndrome. Althoughthe syndrome traditionally has been defined by mucocutaneous criteria, it typically also involves hamartomas and neoplasia of internal organs, most commonly in the thyroid, breast, and female genitourinary tract. Because the mucocutaneous features may develop several decades after birth, the present case both supports the previously reported association between Lhermitte-Duclos disease and Cowden's syndrome and highlights the need for long-term follow-up monitoring of a pediatric patient with Lhermitte-Duclos disease because of the risk of malignancies associated with Cowden's syndrome.

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Documento generato il 02/12/20 alle ore 15:14:28