Intracranial endodermal sinus tumors associated with growth hormone replacement therapy in a girl

Case report

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The primary intracranial endodermal sinus tumor (EST) is regarded as a rare histological subtype that is often associated with components of other germ cell tumors, and there are no reports on the onset of intracranial ESTs after growth hormone (GH) replacement therapy. The authors report an extremely rare case of pure primary EST associated with GH replacement therapy. A 15-year-old girl with GH deficiency experienced headache, nausea, and vomiting after GH replacement therapy for a 17-month period. Magnetic resonance imaging showed 2 tumor masses located in the pineal region and frontal horn of the right lateral ventricle, respectively. Before surgery, the authors administered 1 cycle of neoadjuvant chemotherapy, which shrank the tumor and facilitated surgical intervention. The larger mass located in the pineal region was removed via a right occipital transtentorial approach, and postoperative histopathological analysis revealed a pure EST. While there is a clear association between the initiation of GH replacement therapy and the development of the EST in this case, the causal effect cannot be specified. Nevertheless, this case demonstrates that GH replacement therapy must be used cautiously.

Abbreviations used in this paper: AFP = α-fetoprotein; EST = endodermal sinus tumor; GH = growth hormone; IFG-I = insulin-like growth factor–I.

Article Information

Address correspondence to: Xiang'en Shi, M.D., Ph.D., Department of Neurosurgery, Fu Xing Hospital, Capital Medical University, No. 20 Fu Xing Menwai Street, Xicheng District, Beijing 100038, People's Republic of China. email: shixen@sina.com.

Please include this information when citing this paper: DOI: 10.3171/2011.10.PEDS1195.

© AANS, except where prohibited by US copyright law.

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Figures

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    Sagittal (a and b) and coronal (c and d) MR images obtained before the GH replacement therapy when the patient was at the age of 13 years. The images show that the pituitary is thin, and there are no tumors in pineal region and lateral ventricle. The images in b and d show contrast-enhanced studies of those in a and c, respectively.

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    Plain CT scan revealing obstructive hydrocephalus caused by the mass lesions located in the pineal region and frontal horn of the right lateral ventricle.

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    Transaxial (a–c) and sagittal (d) MR images acquired before neoadjuvant chemotherapy. a: A T1-weighted image showing hypointensity of a large mass located in the pineal region and a small mass with a cyst in the frontal horn of the right lateral ventricle. b: A T2-weighted image demonstrating hyperintensity of the masses with mild edema in right frontal lobe. c and d: Gadolinium-enhanced T1-weighted images revealing solid masses with strong enhancement, except the central areas suggesting the necrosis or intratumoral hemorrhage.

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    Transaxial (a–c) and sagittal (d) MR images all showing regression of the masses after neoadjuvant chemotherapy.

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    Photomicrographs. a: Papillary structures consisting of columnar tumor cells. No Schiller-Duval bodies were noted. H & E stain, original magnification × 200. b: Hyaline droplets. PAS stain, original magnification × 400. c: Immunohistochemical study showing a strong positive reaction AFP. Original magnification × 400. Scale bars = 50 μm.

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