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Titolo:
PHEOCHROMOCYTOMA AS THE FIRST MANIFESTATION OF VONHIPPEL-LINDAU-DISEASE
Autore:
RICHARD S; BEIGELMAN C; DUCLOS JM; FENDLER JP; PLAUCHU H; PLOUIN PF; RESCHE F; SCHLUMBERGER M; VERMESSE B; PROYE C;
Indirizzi:
HOP LA PITIE SALPETRIERE,EPHE,NEUROHISTOL LAB,PAVILLON MONTYON F-75641 PARIS FRANCE HOP INTERCOMMUNAL POISSY FRANCE HOP ST JOSEPH F-75674 PARIS FRANCE HOP HOTEL DIEU F-69288 LYON FRANCE HOP LAENNEC NANTES FRANCE HOP BROUSSAIS F-75674 PARIS FRANCE INST GUSTAVE ROUSSY VILLEJUIF FRANCE HOP CLAUDE HURIEZ LILLE FRANCE
Titolo Testata:
Surgery
fascicolo: 6, volume: 116, anno: 1994,
pagine: 1076 - 1081
SICI:
0039-6060(1994)116:6<1076:PATFMO>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
VONHIPPELLINDAU DISEASE; MEMBERS; LOCUS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
23
Recensione:
Indirizzi per estratti:
Citazione:
S. Richard et al., "PHEOCHROMOCYTOMA AS THE FIRST MANIFESTATION OF VONHIPPEL-LINDAU-DISEASE", Surgery, 116(6), 1994, pp. 1076-1081

Abstract

Background. von Hippel-Lindau disease is an autosomal dominant disorder characterized by the development of hemangioblastomas in the cerebellum, spinal cord, and retina, renal cell carcinoma and cysts, pancreatic cysts, and pheochromocytoma. Methods. We have studied a series of 36 French patients affected with von Hippel-Lindau disease pheochromocytoma. Thirty (83%) of them were diagnosed as having von Hippel-Lindaudisease because the disease occurred in a familial von. Hippel-Lindaudisease setting; six (17%) were diagnosed as having von Hippel-Lindaudisease because they displayed another characteristic manifestation of that disease. Results. The mean age at pheochromocytoma diagnosis was 29 +/- 14 years (5 to 62 years). Bilateral tumors were documented in15 (42%) cases, paraganglioma was associated with adrenal pheochromocytoma in four cases, and malignant pheochromocytoma occurred in three cases. Prevalence of pheochromocytoma revealing von Hippel-Lindau disease was 20 (53%) out of 36. In six cases pheochromocytoma was the onlymanifestation of the disease. Conclusions. In the interest of the patients themselves and of family members who are at risk, search for vonHippel-Lindau disease must be systematic in the presence of pheochromocytoma. Basic checkup may be completed with familial inquiry, ophthalmoscopy, cerebral magnetic resonance imaging, abdominal ultrasonography, and computed tomography-scan for detection of latent lesions. In the future, after characterization of von Hippel-Lindau disease gene mutations, molecular diagnosis is going to be possible in individual patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/11/20 alle ore 06:42:53