Intracranial growing teratoma syndrome: clinical characteristics and treatment strategy.
This study evaluated the clinical and radiological characteristics, as well as the treatment outcomes, for the rare phenomenon known as intracranial growing teratoma syndrome (iGTS). One hundred seventy patients diagnosed with intracranial germ cell tumours (GCT) between 1997 and 2008 were enrolled in this retrospective analysis. Thorough reviews of medical records, brain magnetic resonance images (MRI), pathological findings and tumour markers [alpha-fetoprotein (αFP) and beta-human chorionic gonadotropin (βHCG)] were performed to identify the incidence of iGTS cases and to clarify their clinical characteristics. Eleven out of the 170 intracranial GCT patients (6.5%) were identified as having iGTS. All instances of iGTS originated from non-germinomatous GCT (NGGCT), with incidence rate of 21% (11/52). Six iGTS cases developed from mixed GCTs, four from immature teratomas (ITs) and one from yolk sac tumour. All 11 iGTS patients showed honeycomb-shaped multi-cystic growth patterns on MRI, which is a typical characteristic of iGTS. Surgical excision was performed in all patients, and complete excision was possible in nine patients. Eight of them are alive with no evidence of recurrence; however, two patients who had residual masses died owing to progression of iGTS. Early recognition and suspicion of iGTS, during or after adjuvant therapy of NGGCT, is crucial to ensure that surgical intervention can be performed in a timely manner. Early radical excision may be the treatment of choice for better iGTS prognosis.
Department of Neurosurgery, Clinical Neuro-Science Center, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seoul, South Korea.
Chae-Yong
CY
Kim
Jung-Won
JW
Choi
Ji Yeon
JY
Lee
Seung-Ki
SK
Kim
Kyu-Chang
KC
Wang
Sung-Hye
SH
Park
Gheeyoung
G
Choe
Hyo Seop
HS
Ahn
Il-Han
IH
Kim
Byung-Kyu
BK
Cho
D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000453:epidemiology* / Q000473:pathology*; D017024:Chemotherapy, Adjuvant / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D018997:Chorionic Gonadotropin, beta Subunit, Human / Q000097:blood; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013577:Syndrome; D013724:Teratoma / Q000453:epidemiology* / Q000473:pathology*; D016896:Treatment Outcome; D055815:Young Adult; D000509:alpha-Fetoproteins / Q000032:analysis
D014408:Biomarkers, Tumor; D018997:Chorionic Gonadotropin, beta Subunit, Human; D000509:alpha-Fetoproteins