- Department of Medical Oncology, National Cancer Centre, 11 Hospital Drive, Singapore 169610
- Department of Laboratory Medicine, KK Women and Children's Hospital, 100 Bukit Timah Road, Singapore 229899
- Department of Neurosurgery, National Neuroscience Institute, Duke-NUS Graduate Medical School, 11 Jalan Tan Tock Seng, Singapore 308433
- Children's Cancer Centre, KK Women and Children's Hospital, 100 Bukit Timah Road, Singapore 229899
Foo Song Chuan Aaron
Department of Medical Oncology, National Cancer Centre, 11 Hospital Drive, Singapore 169610
DOI:10.4103/2152-7806.119537Copyright: © 2013 Aaron FSC This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Chuan Aaron FS, Q. Dawn CQ, E. Kenneth CT, Hoe NW, Yen SS, Kian TC. Primary human chorionic gonadotropin secreting germinoma of the corpus callosum. Surg Neurol Int 08-Oct-2013;4:137
How to cite this URL: Chuan Aaron FS, Q. Dawn CQ, E. Kenneth CT, Hoe NW, Yen SS, Kian TC. Primary human chorionic gonadotropin secreting germinoma of the corpus callosum. Surg Neurol Int 08-Oct-2013;4:137. Available from: http://sni.wpengine.com/surgicalint_articles/primary-human-chorionic-gonadotropin-secreting-germinoma-of-the-corpus-callosum/
Background:Primary intracranial germinomas are a rare subset of intracranial tumors derived from mis-incorporated germ cells within the folding neural plate during embryogenesis. Though known to arise from midline structures in the central nervous system (CNS), occurrence within the corpus callosum is exceedingly rare.
Case Description:We present a rare case of secreting primary intracranial germinoma with extensive intraventricular metastasis presenting as a multi-cystic butterfly lesion in the genu of the corpus callosum in a young boy.
Conclusion:Intracranial germ cell tumors must be considered for any multi-cystic lesion arising from midline structures in the CNS in the preadult population.
Keywords: Corpus callosum, germinoma, intracranial, secreting
Primary intracranial germinomas are an extremely rare subset of germ cell tumors (GCT) that arise primarily from midline structures in the central nervous system (CNS). Intracranial germinomas account for 0.5-2.0% of all intracranial tumors[
We present a rare case of primary intracranial GCT arising from the corpus callosum in a 15-year-old child treated at our institution.
A 15-year-old Malay boy with no significant past medical history was referred to our institution with a 2-month history of intermittent fever, recurrent headaches, vomiting, significant weight loss, and deterioration in school performance. He did not have any significant drug, family, or developmental history.
On examination, he was found to be disorientated to time, place, and person. He also demonstrated terminal past-pointing bilaterally and left-sided dysdiadochokinesia.
Initial blood investigations revealed a white blood cell count of 6.92×109/L and a C-reactive protein level of <5.0 mg/L. Serum alphafetoprotein (AFP) and human chorionic gonadotropin (b-HCG) were not elevated. Other abnormal serum biochemical parameters included free thyroxine (FT4) of 9.4 pmol/L, thyroid stimulating hormone (TSH) of 3.92 mIU/L, follicular stimulating hormone of <0.1 IU/L, luteinizing hormone of <0.07 IU/L, sodium of 155 mmol/L and serum osmolality of 323 mOSm/kg. Cerebrospinal fluid (CSF) AFP levels were normal but CSF b-HCG levels, however, were markedly elevated at 1837.01 IU/L. Contrasted computer tomographic (CT) imaging revealed a heterogenous 4 cm mass centered in the genu of the corpus callosum, filling the anterior aspect of the third ventricle and spreading along the margins of both frontal horns, causing obstruction of the Foramina of Monro and hydrocephalus. Thickening and enhancement was also noted along the margins of both lateral ventricles and a 1.3×0.9 cm enhancing nodule visualised in the 4th ventricle [
The patient underwent open craniotomy and biopsy of the intraventricular portion of the tumor. Histology revealed a germinoma composed of sheets of large primitive cells with round nuclei bearing prominent nucleoli, and ample clear cytoplasm with discrete cell membranes, interrupted by fibrous septa containing smaller reactive lymphocytes [
The patient underwent four cycles of carboplatin, etoposide, and ifosfamide as per International Society of Pediatric Oncology Central Nervous System Germ Cell Tumor (SIOP CNS GCT) 96 protocol and subsequent craniospinal irradiation (CSI) of 54 Gray (Gy) in 32 fractions, which he tolerated well. Radiologically, his lesions remained stable despite completing treatment [
Lesions of the corpus callosum are often mentioned in association with aggressively infiltrative tumors such as glioblastoma multiforme (GBM) or demyelinating diseases such as multiple sclerosis.[
As the genu of the corpus callosum constitutes the roof of the frontal horns, tumor spread along the CSF pathways probably accounts for the extent of intraventricular dissemination seen in this patient. Tumors of the corpus callosum are known to have progressed extensively before the manifestation of symptoms due to their location's relatively noneloquent nature.[
Raised CSF b-HCG levels coupled with histological confirmation of germinoma suggest the following differential diagnoses: (1) germinoma with syncytiotrophoblastic giant cells (STGC), (2) HCG-secreting germinoma, or (3) mixed GCT with both germinomatous and nongerminomatous components. The latter is especially in the light of a markedly raised tumor markers (b-HCG) as described in our case report.[
Treatment outcomes comparable with that of unifocal germinomas has been shown to be possible for initially disseminated intracranial germinomas (IDIG). A small single-institution series showed excellent outcomes for IDIG treated with intensive platinum-based chemotherapy combined with CSI (median dose 30.0 Gy; range 21.0-36.0 Gy) and focal boosts to both primary and secondary lesions (median dose 30.6 Gy; range 30.6-55.0 Gy) with minimal long-term side effects.[
Remnant lesions are risk factors for recurrence in most pediatric cancers but appear not to be in the case of germinoma. The appearance of a residual lesion after completion of treatment with chemotherapy suggests the possibility of remnant fibrosis/necrosis or a residual nongerminomatous component that was missed on histological sampling in the first place, the most significant of which is a mature teratomatous component. A distinct phenomenon known as the ‘growing teratoma syndrome’ in which the tumor mass is seen to enlarge during or after chemotherapy in the presence of falling serum markers has been reported.[
Intracranial GCT must be considered for a multi-cystic lesion arising from midline structures in the CNS in the preadult population. Histological sampling must be interpreted in the context of serum/CSF biochemical markers to achieve accurate diagnosis. Treatment outcomes of IDIG are excellent if appropriate therapy can be administered.
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