[Long-term follow-up of patients with suprasellar germinomas].
BACKGROUND
Suprasellar germinomas are rare intracranial neoplasms, which mainly occur in children and adolescents and manifest with endocrine symptoms and/or compression syndromes.


METHODS
The clinical, hormonal and morphological findings as well as treatment and complications were investigated in seven patients (six male, one female) with germinomas.


RESULTS
Mean age at diagnosis was 19.7 years (range 15-32 years). First disease-related symptoms were diabetes insipidus (three patients), loss of libido (two patients), pseudopubertas praecox (one patient), and dwarfism (one patient). However, decisive symptoms leading to final diagnosis were visual disturbances (five patients), pubertas tarda (one patient), and hypogonadism (one patient). All patients were treated by transcranial radiation with a dose of 40-54 Gy. One patient received additional chemotherapy with cisplatin, etoposide, and ifosfamide (PEI). Patients were followed up for 14.6 years (range 7-27 years). Intracranial and pulmonary relapses were observed in two patients. Panhypopituitarism and diabetes insipidus were seen in all patients after treatment. Two patients suffered from loss of vision, two further patients from unilateral amaurosis. One patient developed epilepsy and persistent cognitive impairment. Long-term follow-up shows that two patients died from recurrent disease and decompensated liver cirrhosis, respectively. The other patients are long-term survivors. Full social integration with employment was possible in one case.


CONCLUSIONS
Suprasellar germinomas cause endocrine symptoms during early tumor stages, however, diagnosis is generally established when ocular symptoms related to tumor compression are already present. Long-term survival is characterized by panhypopituitarism, diabetes insipidus and, partly, ocular or cerebral defects.
Bauditz
Juergen
J
Medizinische Klinik und Poliklinik mit Schwerpunkt Gastroenterologie, Hepatologie und Endokrinologie, Universitätsklinikum Charité, Campus Mitte, Charitéplatz 1, 10117, Berlin. Juergen.Bauditz@charite.de
Lochs
Herbert
H
Ventz
Manfred
M
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D017024:Chemotherapy, Adjuvant; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003919:Diabetes Insipidus / Q000209:etiology; D018450:Disease Progression; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D018237:Germinoma / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy; D006801:Humans; D007018:Hypopituitarism / Q000209:etiology; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000209:etiology; D009384:Paraneoplastic Endocrine Syndromes / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy; D010910:Pituitary Irradiation; D010911:Pituitary Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy; D015996:Survival Rate
D005047:Etoposide; D002945:Cisplatin; D007069:Ifosfamide