Intracranial growing teratoma syndrome (iGTS): an international case series and review of the literature

George Michaiel, Douglas Strother, Nicholas Gottardo, Ute Bartels, Hallie Coltin, Juliette Hukin, Beverly Wilson, Shayna Zelcer, Jordan R Hansford, Timothy Hassall, Mohamed S AbdelBaki, Kristina A Cole, Lindsey Hoffman, Natasha P Smiley, Amy Smith, Anna Vinitsky, Nicholas A Vitanza, Avery Wright, Kee K Yeo, Lionel M L ChowMagimairajan I Vanan, Girish Dhall, Eric Bouffet, Lucie Lafay-Cousin

Research output: Contribution to journalReview articlepeer-review

15 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)721-730
Number of pages10
JournalJournal of Neuro-Oncology
Issue number3
Publication statusPublished or Issued - May 2020
Externally publishedYes


  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Neoplasms, Germ Cell and Embryonal/complications
  • Pinealoma/complications
  • Retrospective Studies
  • Teratoma/complications
  • Treatment Outcome
  • Young Adult

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