TY - JOUR
T1 - Intracranial growing teratoma syndrome (iGTS)
T2 - an international case series and review of the literature
AU - Michaiel, George
AU - Strother, Douglas
AU - Gottardo, Nicholas
AU - Bartels, Ute
AU - Coltin, Hallie
AU - Hukin, Juliette
AU - Wilson, Beverly
AU - Zelcer, Shayna
AU - Hansford, Jordan R.
AU - Hassall, Timothy
AU - AbdelBaki, Mohamed S.
AU - Cole, Kristina A.
AU - Hoffman, Lindsey
AU - Smiley, Natasha P.
AU - Smith, Amy
AU - Vinitsky, Anna
AU - Vitanza, Nicholas A.
AU - Wright, Avery
AU - Yeo, Kee K.
AU - Chow, Lionel M.L.
AU - Vanan, Magimairajan I.
AU - Dhall, Girish
AU - Bouffet, Eric
AU - Lafay-Cousin, Lucie
N1 - Publisher Copyright:
© 2020, Springer Science+Business Media, LLC, part of Springer Nature.
PY - 2020/5/1
Y1 - 2020/5/1
N2 - Purpose: Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon of paradoxical growth of a germ cell tumor (GCT) during treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of iGTS in Western countries. Methods: Pediatric patients from 22 North American and Australian institutions diagnosed with iGTS between 2000 and 2017 were retrospectively evaluated. Results: From a total of 777 cases of central nervous system (CNS) GCT, 39 cases of iGTS were identified for an overall frequency of 5%. Pineal region was a more frequent location for iGTS as compared to cases of GCT without iGTS (p < 0.00001). In patients with an initial tissue diagnosis of GCT, immature teratoma was present in 50%. Serum AFP or ßhCG was detectable in 87% of patients (median values 66 ng/mL and 44 IU/L, respectively). iGTS occurred at a median of 2 months (range 0.5–32) from diagnosis, in the majority of patients. All patients underwent surgical resection, leading to gross total resection in 79%. Following surgery, all patients resumed adjuvant therapy or post treatment follow-up for GCT. At a median follow-up of 5.3 years (range 0.2–11.8), 37 (95%) of patients are alive, including 5 with stable residual mass. Conclusion: iGTS occurs in 5% of patients with GCT in Western countries. Tumors of the pineal region and GCT containing immature teratoma appear to be associated with a higher risk of developing iGTS. Complete surgical resection is the mainstay of treatment. Overall survival of patients developing iGTS remains favorable.
AB - Purpose: Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon of paradoxical growth of a germ cell tumor (GCT) during treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of iGTS in Western countries. Methods: Pediatric patients from 22 North American and Australian institutions diagnosed with iGTS between 2000 and 2017 were retrospectively evaluated. Results: From a total of 777 cases of central nervous system (CNS) GCT, 39 cases of iGTS were identified for an overall frequency of 5%. Pineal region was a more frequent location for iGTS as compared to cases of GCT without iGTS (p < 0.00001). In patients with an initial tissue diagnosis of GCT, immature teratoma was present in 50%. Serum AFP or ßhCG was detectable in 87% of patients (median values 66 ng/mL and 44 IU/L, respectively). iGTS occurred at a median of 2 months (range 0.5–32) from diagnosis, in the majority of patients. All patients underwent surgical resection, leading to gross total resection in 79%. Following surgery, all patients resumed adjuvant therapy or post treatment follow-up for GCT. At a median follow-up of 5.3 years (range 0.2–11.8), 37 (95%) of patients are alive, including 5 with stable residual mass. Conclusion: iGTS occurs in 5% of patients with GCT in Western countries. Tumors of the pineal region and GCT containing immature teratoma appear to be associated with a higher risk of developing iGTS. Complete surgical resection is the mainstay of treatment. Overall survival of patients developing iGTS remains favorable.
KW - CNS
KW - Germ cell tumor
KW - Growing teratoma syndrome
KW - Intracranial
UR - http://www.scopus.com/inward/record.url?scp=85083527510&partnerID=8YFLogxK
U2 - 10.1007/s11060-020-03486-9
DO - 10.1007/s11060-020-03486-9
M3 - Article
C2 - 32297094
AN - SCOPUS:85083527510
SN - 0167-594X
VL - 147
SP - 721
EP - 730
JO - Journal of Neuro-Oncology
JF - Journal of Neuro-Oncology
IS - 3
ER -