Simultaneous suprasellar and pineal germ cell tumors in five late stage adolescents: endocrinological studies and prolonged follow-up.
Primary germ cell tumors (PGCT) of the central nervous system usually develop in the third ventricle area, and most frequently in the pineal region. The suprasellar region is the second preferential site for development of these tumors which are rarely simultaneously present in these two sites. We report five new cases of PGCT with pineal and suprasellar localizations, which appeared in late puberty in four boys and one girl aged 17-19 years. The clinical picture associated signs of intracranial hypertension, convergence and verticality palsies, diabetes insipidus and pituitary deficiency. Encephalic MRI revealed a double localization. Endocrine tests revealed a particular pattern associating central diabetes insipidus and a hypothalamic-pituitary disconnection syndrome. Following identification of the pathological type of lesions via a neurosurgical approach, treatment was based on a combined method using chemotherapy, radiotherapy and hormone replacement. Based on this treatment, prolonged remissions were obtained with a good quality of life.
Vuillermet
P
P
Department of Endocrinology, University Hospital, Rouen, France.
Cauliez
B
B
Fréger
P
P
Vannier
J P
JP
Pellerin
A
A
Kuhn
J M
JM
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D001932:Brain Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D003131:Combined Modality Therapy; D004358:Drug Therapy; D005260:Female; D005500:Follow-Up Studies; D012739:Gonadal Steroid Hormones / Q000097:blood; D013006:Growth Hormone / Q000097:blood; D020249:Hormone Replacement Therapy; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007030:Hypothalamo-Hypophyseal System / Q000378:metabolism*; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D009378:Neoplasms, Multiple Primary / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D010870:Pineal Gland / Q000473:pathology; D010871:Pinealoma / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D010911:Pituitary Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D011379:Prognosis; D011878:Radiotherapy; D013972:Thyrotropin / Q000097:blood; D055815:Young Adult
D012739:Gonadal Steroid Hormones; D000324:Adrenocorticotropic Hormone; D013972:Thyrotropin; D013006:Growth Hormone; D006854:Hydrocortisone