title: Bifocal intracranial tumors of nongerminomatous germ cell etiology: diagnostic and therapeutic implications.
abstract: BACKGROUND
  Patients presenting with synchronous bifocal intracranial tumors (masses in the pineal and neurohypophyseal region), detectable human chorionic gonadotropin (hCG) levels (5-100 mIU/mL), and normal alpha feto-protein (AFP) levels (≤10 ng/mL) are often diagnosed empirically with pure germinoma. In such scenarios, pathologic confirmation is often deferred, given that bifocal nongerminomatous germ cell tumors (NGGCTs) are considered rare and because available literature and research protocols support such an approach. We sought to characterize the association between bifocal intracranial tumors and NGGCT histology.
  
  
  METHODS
  Seventy-one patients treated for intracranial germ cell tumors at Massachusetts General Hospital in 1998-2012 were identified. Patients presenting with synchronous bifocal disease were selected for further review.
  
  
  RESULTS
  Of the 71 patients presenting with intracranial germ cell tumors, 14 (19.7%) had synchronous bifocal disease. Of these 14 patients, 7 (50.0%) had germinoma, 3 (21.4%) had NGGCT, and 4 (28.6%) had hCG levels <200 mIU/mL and normal AFP levels and were treated without pathologic confirmation. Of the 3 patients with confirmed bifocal NGGCT, 2 had detectable hCG levels with AFP <10 ng/mL and 1 patient had a detectable hCG level with a modest elevation in AFP.
  
  
  CONCLUSIONS
  NGGCTs should be considered in the differential diagnosis for patients presenting with bifocal intracranial tumors. Given differences in the management of germinomas and NGGCTs, clinicians should strongly consider a biopsy in patients presenting with bifocal masses and normal or modestly elevated biomarkers. Misclassification of such cases as germinomas could result in undertreatment and a possible increased risk for recurrence.
authors: Harvard Radiation Oncology Program, Boston, Massachusetts, USA.
authors: Ayal A
authors: AA
authors: Aizer
authors: Roshan V
authors: RV
authors: Sethi
authors: E Tessa
authors: ET
authors: Hedley-Whyte
authors: David
authors: D
authors: Ebb
authors: Nancy J
authors: NJ
authors: Tarbell
authors: Torunn I
authors: TI
authors: Yock
authors: Shannon M
authors: SM
authors: Macdonald
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D006063:Chorionic Gonadotropin / Q000378:metabolism; D003131:Combined Modality Therapy; D018237:Germinoma; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D009378:Neoplasms, Multiple Primary / Q000473:pathology* / Q000628:therapy; D010870:Pineal Gland / Q000378:metabolism / Q000473:pathology*; D010904:Pituitary Gland, Posterior / Q000378:metabolism / Q000473:pathology*; D011379:Prognosis; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D006063:Chorionic Gonadotropin; D000509:alpha-Fetoproteins
keywords: NGGCT; bifocal; germ cell; multiple midline; nongerminomatous
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