Unfortunately, the germ cell tumors of the brain arise in deepseated locations that, even with modern day neurosurgical and imaging techniques, can lead to significant damage if operated upon by inexperienced hands. The rarity of these tumors means that only those neurosurgeons – and largely pediatric neurosurgeons – who work in major medical centers, have sufficient experience and expertise to tackle these tumors. In general, a biopsy (a small sample of tumor tissue) is essential if the MRI scan shows a tumor in the pineal or suprasellar regions, and the tumor markers in both serum and CSF are not elevated. Patients with pineal region tumors usually present with hydrocephalus that needs to be corrected by the placement of a shunt; accordingly, many skilled neurosurgeons today will combine the placement of a third ventriculostomy (a totally internalized shunt) with a tiny biopsy of the tumor, performed through a much less invasive endoscopic procedure. However, the tumor tissue sample is often extremely small by this approach, and may not be representative of the entire tumor, thereby failing to recognize an additional component of a mixed germ cell tumor. Attempts to remove most of the tumor by radical surgical resection have NOT been shown to improve the cure rate of patients with germ cell tumors of the brain, and are associated with higher complication rates, some of which may be very serious and irreversible. However, in the case of tumors consisting of mature or immature teratoma components, these components are not so responsive to irradiation or chemotherapy, and are indeed best eradicated by surgical resection. The best time to undertake such a surgical resection is not at initial diagnosis, but through delayed surgical resection, after initial chemotherapy (or in some cases initial radiation therapy) have shrunk down the other elements of the germ cell tumor, and a residual tumor mass remains indicative most likely of mature or immature teratoma. Sometimes, the mature or immature teratoma component of the tumor is so substantial, that the tumor actually increases in size with initial chemotherapy. This uncommon but well-recognized scenario is known as “The Growing Teratoma Syndrome”, and when this happens, it is critical that the treating physicians recognize it for what it is, a situation requiring prompt surgical resection. It is NOT an indication that chemotherapy has failed, and all hope for cure is lost unless immediate high dose radiation therapy or – even worse – far more intensive chemotherapy – is implemented. Resection of the teratoma should be undertaken, and then the original plan of treatment continued. |