BACKGROUND: Pineal region tumors represent a rare and histologically diverse group of lesions. Few studies are available to guide management and the outcomes after stereotactic radiosurgery (SRS). METHODS: Patients who underwent SRS for a pineal region tumor and for whom at least 6 months of imaging follow-up was available were retrospectively assessed in 5 centers. Data were collected from the medical record and histology level analyses were performed, including actuarial tumor control and survival analyses. RESULTS: A total of 70 patients were treated between 1989 and 2014 with a median follow-up of 47 months. Diagnoses were pineocytoma (37%), pineoblastoma (19%), pineal parenchymal tumor of intermediate differentiation (10%), papillary tumor of the pineal region (9%), germinoma (7%), teratoma (3%), embryonal carcinoma (1%), and unknown (14%). Median prescription dose was 15 Gy at the 50% isodose line. Actuarial local control and survival rates were 81% and 76% at 20 years for pineocytoma, 50% and 56% at 5 years for pineal parenchymal tumor of intermediate differentiation, 27% and 48% at 5 years for pineoblastoma, 33% and 100% at 5 years for papillary tumor of the pineal region, 80% and 80% at 20 years for germinoma, and 61% and 67% at 5 years for tumors of unknown histology. New focal neurological deficit, Parinaud syndrome, and hydrocephalus occurred in 9%, 7%, and 3% of cases, respectively. CONCLUSIONS: SRS is a safe modality for the management of pineal region tumors. Its specific role is highly dependent on tumor histology. As such, all efforts should be made to obtain a reliable histologic diagnosis.

• Klíčová slova
• Gamma knife radiosurgery, Pineal region tumors, Survival, Tumor control,
• MeSH
• dítě MeSH
• dospělí MeSH
• epifýza mozková patologie   chirurgie   MeSH
• internacionalita * MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru mortalita   patologie   chirurgie   MeSH
• míra přežití trendy   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádory mozku mortalita   patologie   chirurgie   MeSH
• následné studie MeSH
• pinealom mortalita   patologie   chirurgie   MeSH
• předškolní dítě MeSH
• radiochirurgie mortalita   trendy   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• výzkumná zpráva MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

BACKGROUND: Papillary tumor of the pineal region (PTPR) is a recently defined tumor entity. Its clinical course is characterized by frequent local recurrence, and patients may experience the burden of symptoms due to the anatomical location of the growing mass. Guidelines for treatment protocols, and the role of radiotherapy are still being investigated. CASE: We report the case of a 27-year old woman who was referred to our department after she was diagnosed with PTPR and had undergone multiple surgical interventions. We delivered adjuvant conformal radiotherapy on the gross residual tumor to a total dose of 59.4 Gy (33 × 1.8 Gy). DISCUSSION: After a follow-up period of 41 months, we obtained a complete response to the treatment, according to the Response evaluation criteria in solid tumors criteria (RECIST). Radiation treatment was well tolerated, and the patient did not develop acute and late side effects. The neurological symptoms, which were documented at the diagnosis and after the surgical procedure, have not been recorded at last follow-up. CONCLUSIONS: Formal consensus for managing patients with a diagnosis of PTPR are nonexistent. Despite surgery, this tumor has a tendency to recur. Radiotherapy could have a role in the adjuvant setting and needs to be investigated in a multicenter setting with a long follow-up.Key words: radiotherapy - neurosurgery - magnetic resonance - pineal region - brain tumor.

• MeSH
• adjuvantní radioterapie metody   MeSH
• dospělí MeSH
• epifýza mozková patologie   MeSH
• lidé MeSH
• lokální recidiva nádoru radioterapie   MeSH
• nádory mozku patologie   radioterapie   chirurgie   MeSH
• papilom radioterapie   chirurgie   MeSH
• pinealom radioterapie   chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUND: Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors. PATIENTS AND METHODS: Original prospective treatment and outcome data from international trial groups were pooled. Cox regression models were developed considering treatment elements as time-dependent covariates. RESULTS: Data on 135 patients with pineoblastoma aged 0.01-20.7 (median 4.9) years were analyzed. Median observation time was 7.3 years. Favorable prognostic factors were age ≥4 years (hazard ratio [HR] for progression-free survival [PFS] 0.270, P < .001) and administration of radiotherapy (HR for PFS 0.282, P < .001). Metastatic disease (HR for PFS 2.015, P = .006), but not postoperative residual tumor, was associated with unfavorable prognosis. In 57 patients <4 years old, 5-year PFS/overall survival (OS) were 11 ± 4%/12 ± 4%. Two patients survived after chemotherapy only, while 3 of 16 treated with craniospinal irradiation (CSI) with boost, and 3 of 5 treated with high-dose chemotherapy (HDCT) and local radiotherapy survived. In 78 patients aged ≥4 years, PFS/OS were 72 ± 7%/73 ± 7% for patients without metastases, and 50 ± 10%/55 ± 10% with metastases. Seventy-three patients received radiotherapy (48 conventionally fractionated CSI, median dose 35.0 [18.0-45.0] Gy, 19 hyperfractionated CSI, 6 local radiotherapy), with (n = 68) or without (n = 6) chemotherapy. The treatment sequence had no impact; application of HDCT had weak impact on survival in older patients. CONCLUSION: Survival is poor in young children treated without radiotherapy. In these patients, combination of HDCT and local radiotherapy may warrant further evaluation in the absence of more specific or targeted treatments. CSI combined with chemotherapy is effective for older non-metastatic patients.

• Klíčová slova
• high-dose chemotherapy, pediatric, pineoblastoma, radiotherapy, treatment,
• MeSH
• antitumorózní látky terapeutické užití   MeSH
• databáze faktografické MeSH
• dítě MeSH
• dospělí MeSH
• epifýza mozková účinky léků   patologie   účinky záření   MeSH
• kojenec MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádory mozku farmakoterapie   mortalita   radioterapie   terapie   MeSH
• pinealom farmakoterapie   mortalita   radioterapie   terapie   MeSH
• předškolní dítě MeSH
• přežití po terapii bez příznaků nemoci MeSH
• prospektivní studie MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH
• Spojené státy americké MeSH
• Názvy látek
• antitumorózní látky 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

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

BACKGROUND: Papillary pineocytoma is an extremely rare tumor usually with a poor outcome. CASE REPORT: We report a case of a 10-year-old-girl with pineal gland tumor and obstructive hydrocephalus diagnosed using MRI. The child was successful treated by insertion of a ventriculoperitoneal shunt and consecutive tumor resection by supracerebellar-infratentorial approach. Histopathological examination showed a papillary structure of the pineocytoma. As such, tumors are considered to be aggressive the child was subjected to radio- and chemotherapy. CONCLUSION: At six year follow-up after surgery, the patient is symptom-free and the MRI shows no tumor recurrence.

• MeSH
• dítě MeSH
• epifýza mozková * MeSH
• lidé MeSH
• nádory mozku * diagnóza   terapie   MeSH
• pinealom * diagnóza   terapie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• choristom diagnóza   MeSH
• chromofobní adenom diagnóza   MeSH
• dítě MeSH
• dospělí MeSH
• epifýza mozková * MeSH
• lidé MeSH
• nádory hypofýzy diagnóza   MeSH
• nádory mozku diagnóza   MeSH
• pinealom diagnóza   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• endokrinní žlázy patofyziologie   MeSH
• hypothalamus * MeSH
• lidé MeSH
• metody MeSH
• mladiství MeSH
• nádory mozku diagnóza   MeSH
• pinealom diagnóza   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• cerebelární ataxie diagnóza   MeSH
• dítě MeSH
• lidé MeSH
• nemoci mozečku diagnóza   MeSH
• pinealom diagnóza   MeSH
• předškolní dítě MeSH
• Schilderova difuzní cerebroskleróza diagnóza   MeSH
• syndrom MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• předškolní dítě MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH

• Klíčová slova
• ANGIOMA, SCLEROSING *, BRAIN NEOPLASMS *, CENTRAL NERVOUS SYSTEM *, CHOLESTEATOMA *, CRANIOPHARYNGIOMA *, GLIOMA *, MEDULLOBLASTOMA *, MENINGIOMA *, NEOPLASM STATISTICS *, NEURILEMMOMA *, PINEALOMA *, PITUITARY NEOPLASMS *, TERATOID TUMOR *,
• MeSH
• benigní fibrózní histiocytom * MeSH
• centrální nervový systém * MeSH
• cholesteatom * MeSH
• gliom * MeSH
• hemangiom * MeSH
• kraniofaryngeom * MeSH
• lidé MeSH
• meduloblastom * MeSH
• meningeální nádory * MeSH
• meningeom * MeSH
• nádory hypofýzy * MeSH
• nádory mozečku * MeSH
• nádory mozku * MeSH
• nádory epidemiologie   MeSH
• neurilemom * MeSH
• pinealom * MeSH
• teratom * MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH