10.14735/amko201319 OR Pineal Germ Cell Tumors Review Dotaz Zobrazit nápovědu
BACKGROUND: Primary intracranial germ cell tumors represent a rare category of neoplasms, which occur in children and young adults. The WHO classification divides intracranial tumors into germinomas and non-germinomas. The most frequent locality of these tumors is pineal and suprasellar region. Clinical signs and symptoms depend on the localization of the tumour - they most commonly include signs of increased intracranial pressure, Parinauds syndrome, bitemporal hemianopsy and signs of endocrine deficiency. Gadolinium enhanced MRI scan of the brain is the imagining examination of choice in the diagnostic strategy of intracranial germ cell tumors. However, the imagining studies do not provide sufficient information about histological type; therefore, biopsy is necessary. The exception represents cases with characteristically increased levels of tumor markers (AFP and β-HCG) measured in the serum and cere-brospinal fluid. CASE: A pineal germ cell tumor was observed in a 26-year-old male with presentation of an eye-sight disorder with focusing difficulty and photophobia, accompanied by intensive fatigue and sleepiness, nausea with occasional vomiting, intermittent headaches and Parinauds syndrome. MRI examination of the brain showed tumor expansion in the pineal region and in the right part of the mesencephalon. Radical extirpation of the tumor in the pineal region was performed. The follow-up MRI scan of the brain revealed relapse of the disease. The patient underwent craniospinal radiation therapy with subsequent postoperative chemotherapy (regimen cisplatin and etoposide), three cycles in total. Currently, the patient is 30 months after finishing of oncological treatment in clinical remission of the disease. CONCLUSION: The treatment and prognosis of this neoplasm differ between particular categories. Germinomas have better survival rates than non-germinomas. A 5-year survival rate of germinoma patients after application of radiotherapy alone was > 90% of cases. The addition of chemotherapy lead to a decrease of the dose and minimalization of the irradiated area, with achievement of fewer side effects without a decrease of the curability. Non-germinomas are less radiosensitive than germinomas, but after the application of the adjuvant chemotherapy, survival benefit was achieved. However, the optimal management of these tumors remains controversial.
- MeSH
- dospělí MeSH
- germinální a embryonální nádory * klasifikace diagnóza terapie MeSH
- lidé MeSH
- pinealom * klasifikace diagnóza terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
INTRODUCTION: The pineal region is a deep-seated part of the brain surrounded by highly eloquent structures. Differential diagnosis of space-occupying lesions in this region encompasses pineal gland cysts, pineal gland tumours, metastases, germ cell tumours, meningiomas, gliomas, hemangioblastomas and neuroectodermal tumours. A treatment strategy is based mainly on tumour anatomical characteristics and histological type. Except germinatous tumours, a surgical excision is the treatment of choice. METHODS: Microsurgical approaches: The microsurgical supracerebellar-infratentorial approach is an essential approach to the pineal region. Despite certain risks, it allows a straightforward and completely extracerebral approach with a minimal cerebellar retraction. The other basic approach is the microsurgical occipital-transtentorial approach that is advantageous in patients with a supratentorial tumour extension or a steep tentorium. The interhemispheric-transcallosal approach and the transcortical-transventricular approach are possible options in selected cases.Endoscopic approaches: The neuroendoscopy provides a minimally invasive method to perform a tumour biopsy and to treat hydrocephalus in one session. Stereotactic biopsy: The stereotactic needle biopsy represents an alternative to the endoscopic biopsy in patients without hydrocephalus and in patients with dorsally located lesions inaccessible from the third ventricle. CONCLUSION: Modern neurosurgery offers a rich variety of surgical approaches to the pineal region. The complexity of space-occupying lesions in this region requires an individualised treatment, a prudent preoperative planning and a meticulous surgical technique.
- Klíčová slova
- pineal gland pineal tumours neurosurgical procedures - craniotomy neuroendoscopy.,
- MeSH
- biopsie metody MeSH
- diferenciální diagnóza MeSH
- epifýza mozková * patologie chirurgie MeSH
- lidé MeSH
- mikrochirurgie metody MeSH
- nádory mozku * diagnóza patologie chirurgie MeSH
- neurochirurgické výkony * škodlivé účinky metody MeSH
- neuroendoskopie metody MeSH
- pinealom * diagnóza patologie chirurgie MeSH
- plánování péče o pacienty MeSH
- stereotaktické techniky * MeSH
- zohlednění rizika MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH