Suprasellar germinomas in childhood and adolescence: diagnostic pitfalls

Détails

ID Serval
serval:BIB_64C60E3FFD86
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Suprasellar germinomas in childhood and adolescence: diagnostic pitfalls
Périodique
Journal of Pediatric Endocrinology and Metabolism
Auteur⸱e⸱s
Ramelli  G. P., von der Weid  N., Stanga  Z., Mullis  P. E., Buergi  U.
ISSN
0334-018X (Print)
Statut éditorial
Publié
Date de publication
12/1998
Volume
11
Numéro
6
Pages
693-7
Notes
Journal Article --- Old month value: Nov-Dec
Résumé
The clinical and neuro-endocrine data of seven young male patients with suprasellar germinomas seen between 1984 and 1992 are reported. The most common initial symptom was 'idiopathic' central diabetes insipidus (DI), which occurred in all seven patients. The time interval between the appearance of this first clinical sign and the definitive diagnosis of a suprasellar germinoma ranged from 3 to 66 months. Raised prolactin levels and growth hormone deficiency were indicators of a process located in the hypothalamic-pituitary region. An increased beta-HCG level in the serum or the CSF confirmed the diagnostic suspicion of a germinoma and was helpful as a tumor marker in follow-up. Neuro-radiologic studies (CT or MRI) were also disappointing in the early stage when patients presented only with DI. Later on, as patients developed additional symptoms or signs related to the tumor, imaging studies were positive. Given the variable rate of tumor progression, the nonspecific early signs of hypothalamic-pituitary dysfunction (DI) as well as the often negative early imaging studies, the diagnosis of suprasellar germinoma is difficult but should always be considered in the presence of so-called 'idiopathic' central DI. Repeated brain MRIs are mandatory in young patients with idiopathic DI in order not to miss an underlying suprasellar germinoma.
Mots-clé
Adolescent Adult Brain Neoplasms/complications/*diagnosis/physiopathology Child Chorionic Gonadotropin, beta Subunit, Human/blood/cerebrospinal fluid Diabetes Insipidus/diagnosis/etiology Diagnosis, Differential Germinoma/complications/*diagnosis/physiopathology Humans Magnetic Resonance Imaging Male Prolactin/blood *Sella Turcica Tomography, X-Ray Computed
Pubmed
Web of science
Création de la notice
25/01/2008 10:35
Dernière modification de la notice
20/08/2019 14:21
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