Intracranial germ-cell tumors in children

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✓ All patients with confirmed intracranial germ-cell tumors treated at the Hospital of Sick Children during the period January, 1952, to December, 1989, were reviewed. Of the 51 tumors reviewed, 16 were located in the suprasellar region, 32 in the pineal region, and three in both the pineal and the suprasellar regions. Forty-nine patients underwent surgical resection which was total in seven and partial in 20, and consisted of a biopsy in 22. Two patients were managed on the basis of serum and cerebrospinal fluid markers. Surgical tools such as the operating microscope, the ultrasonic surgical aspirator, and the laser beam allowed safe debulking and removal of the deep-seated tumors in the pineal region. There were no operative deaths in the 36 patients treated since 1972, who included 23 with pineal tumors. Twenty-five patients with germinomas received radiotherapy and had a 5-year survival rate of 85.1%. Thirteen patients with non-germinoma germ-cell tumors received radiotherapy and had a 5-year survival rate of 45.5%. On the basis of this review, the authors recommend resection of pineal and suprasellar germ-cell tumors in order to firmly establish an accurate histological diagnosis to guide the extent of adjuvant therapy. In the case of a pure germinoma without evidence of dissemination, adjuvant therapy consists only of local radiotherapy. On the other hand, for malignant non-germinoma germ-cell tumors, adjuvant therapy must include chemotherapy as well as craniospinal axis radiotherapy.

Article Information

Address reprint requests to: Harold J. Hoffman, M.D., Division of Neurosurgery, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.

© AANS, except where prohibited by US copyright law.

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Figures

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    Computerized tomography (CT) scans of a patient with pineal germinoma. Left: Unenhanced CT scan showing a hyperdense calcified mass. Right: Enhanced CT scan showing bright enhancement of the tumor.

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    T1-weighted magnetic resonance images, anteroposterior view (left) and lateral view (right), of a patient with mixed germinoma, endodermal sinus tumor, and teratoma in the pineal region. Note the mixed densities seen in this tumor.

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    T1-weighted magnetic resonance image showing a thickened pituitary stalk as the only evidence of suprasellar germinoma in a child who presented with diabetes insipidus.

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    Photograph of the roof of the mouth of a patient showing invading malignant teratoma which had arisen in the suprasellar region.

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    Computerized tomography (CT) scans of a patient with a large mixed non-germinoma germ-cell tumor. Left: Preoperative CT scan showing the tumor. Right: Postoperative CT scan showing total resection of the tumor.

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