BACKGROUND AND OBJECTIVES: Pineocytomas are grade 1 tumors arising from the pineal parenchyma. Gross total resection can potentially cure these benign lesions but can be associated with morbidity. This study was designed to provide multi-institutional data to evaluate the results of stereotactic radiosurgery (SRS) for pineocytomas. METHODS: Centers participating in the International Radiosurgery Research Foundation were asked to review their database and provide data for patients who had SRS for histology confirmed grade 1 pineocytomas, for whom clinical and imaging follow-up of at least 6 months was available. RESULTS: In total, 38 patients underwent SRS as part of the management of a pineocytoma. The median age at SRS was 39 years (range 8-76). SRS was performed as primary approach in 68%, adjuvant after partial resection 19%, and at recurrence in 13% of patients. The median margin dose was 15 Gy (range 11-25 Gy). The median treatment volume was 3.35 cc (range 0.1-17.9 cc). Local tumor control was achieved in 92% of patients, with a mean actuarial progression-free survival of 21.6 years (median not reached). At last follow-up, 82% were still controlled, 8% had local recurrence, and 10% had cerebrospinal fluid dissemination. Tumor control was significantly better when SRS was used as primary care compared with the adjuvant or recurrent setting ( P = .016). Five patients (13%) died during follow-up, all from tumor progression. The actuarial mean survival duration was 24.3 years, with a 5-year survival rate of 91%, and an estimated rate of 76% at 29 years. Larger tumor volume at SRS was found to be correlated to increased risk of death ( P = .045). Transient symptomatic adverse radiation effects were observed in 4 patients (11%). CONCLUSION: SRS appears safe and effective for the management of pineocytomas. Long-term tumor control is achieved in most cases. SRS can be offered to selected patients as an alternative to surgical resection.

• MeSH
• Child MeSH
• Adult MeSH
• Pineal Gland pathology   radiation effects   MeSH
• Middle Aged MeSH
• Humans MeSH
• Neoplasm Recurrence, Local MeSH
• Adolescent MeSH
• Young Adult MeSH
• Brain Neoplasms * mortality   radiotherapy   MeSH
• Follow-Up Studies MeSH
• Pinealoma * mortality   radiotherapy   MeSH
• Radiosurgery * methods   MeSH
• Retrospective Studies MeSH
• Aged MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH

Představujeme kazuistické sdělení zkušeností s léčbou mladé 35leté pacientky s vzácným tumorem CNS - pineoblastomem. Po parciálním neurochirurgickém zákroku byla pacientka indikována multidisciplinárním týmem k protonové terapii tužkovým svazkem s profylaktickým ozářením kraniospinální osy dávkou 36 Gy (Gray equivalent), do celkové dávky na lůžko tumoru 59,4 Gy a konkomitantní aplikací chemoterapie temozolomidem. Po ukončení chemoradioterapie pacientka absolvovala 4 cykly vysokodávkované chemoterapie s podporou periferních kmenových buněk. 11 měsíců po ukončení protonové chemoradioterapie s dobrou tolerancí pacientka zůstává bez příznaků reziduálního onemocnění. Kazuistika poukazuje na příznivou krátkodobou léčebnou odpověď a příznivý neurologický výsledek terapie tohoto vzácného tumoru pineální krajiny. Jsou prodiskutovány různé literárně popisované léčebné strategie.

We present our experience with a treatment of a 35-year old adult female patient with a rare central nervous system tumor pineoblastoma. Postoperative patient was referred to adjuvant pencil beam scanning therapy (PBS) starting with 36 Gy dose of prophylactic craniospinal irradiation followed by a local 23.4 Gy boost to the tumor bed. A total dose of 59.4 Gy was given to the patient along with concurrent temozolomide. After well tolerated proton beam chemoradiotherapy the patient finished 4 cycles of high-dose chemotherapy with peripheral blood hematopoietic stem cell support. At 11 months after proton beam chemoradiotherapy the patient has remained free of recurrence. This case report shows favorable short-term response and neurological outcome in management of this rare pineal region tumor. The various treatment options reported in the literature are discussed.

• MeSH
• Radiation Dosage MeSH
• Adult MeSH
• Histological Techniques MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Neurosurgical Procedures MeSH
• Pinealoma * surgery   diagnostic imaging   drug therapy   radiotherapy   therapy   MeSH
• Proton Therapy * methods   MeSH
• Radiotherapy methods   MeSH
• Neoplasm, Residual MeSH
• Temozolomide administration & dosage   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

Pineální cysty jsou benigní afekce šišinky, jejichž prevalence v populaci dosahuje až 1 %. Pineální cysta bývá nejčastěji diagnostikována na magnetické rezonanci jako sférický cystický útvar dorzálně od třetí komory nad mezencefalem. Klinický přístup k pacientům s pineální cystou je velmi kontroverzní, zejména pokud přichází s nespecifickými potížemi. U všech pacientů považujeme za důležité ujištění o benigní povaze pineálních cyst a o příznivé prognóze. Léčebnou modalitou je na prvním místě observace, dále pak symptomatická terapie. U malé části nemocných lze zvažovat chirurgickou léčbu, ovšem indikační kritéria nejsou jasně definována.

Pineal cysts are benign lesions of the pineal gland whose prevalence in the population reaches as much as 1%. A pineal cyst is most commonly diagnosed on magnetic resonance imaging as a spherical cystic mass dorsally from the third ventricle above the midbrain. The clinical approach to patients with a pineal cyst is very controversial, particularly if they present with nonspecific complaints. In all patients, we consider it important to assure them of a benign nature of their pineal cysts and of a favourable prognosis. The primary therapeutic modality is observation, followed by symptomatic treatment. In a small proportion of patients, surgical treatment can be considered; however, the indication criteria have not been defined clearly.

• MeSH
• Cysts therapy   MeSH
• Diagnostic Imaging methods   MeSH
• Pineal Gland * diagnostic imaging   pathology   MeSH
• Humans MeSH
• Magnetic Resonance Imaging methods   MeSH
• Neurosurgical Procedures methods   MeSH
• Pinealoma diagnosis   epidemiology   therapy   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

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.

• MeSH
• Child MeSH
• Adult MeSH
• Pineal Gland pathology   surgery   MeSH
• Internationality * MeSH
• Cohort Studies MeSH
• Middle Aged MeSH
• Humans MeSH
• Neoplasm Recurrence, Local mortality   pathology   surgery   MeSH
• Survival Rate trends   MeSH
• Adolescent MeSH
• Young Adult MeSH
• Brain Neoplasms mortality   pathology   surgery   MeSH
• Follow-Up Studies MeSH
• Pinealoma mortality   pathology   surgery   MeSH
• Child, Preschool MeSH
• Radiosurgery mortality   trends   MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Research Report MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Child, Preschool MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study 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.

• MeSH
• Databases, Factual MeSH
• Child MeSH
• Adult MeSH
• Pineal Gland drug effects   pathology   radiation effects   MeSH
• Infant MeSH
• Combined Modality Therapy MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Brain Neoplasms drug therapy   mortality   radiotherapy   therapy   MeSH
• Pinealoma drug therapy   mortality   radiotherapy   therapy   MeSH
• Child, Preschool MeSH
• Disease-Free Survival MeSH
• Prospective Studies MeSH
• Antineoplastic Agents therapeutic use   MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Infant MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Geographicals
• Europe MeSH
• United States MeSH

Úvod: Pineální krajina je hluboká oblast mozku obklopená vysoce elokventními strukturami. Expanzivní léze, které nacházíme v této oblasti, jsou cysty glandula pinealis, nádory glandula pinealis (od pineocytomu po pinealoblastom) a dále metastázy, germinální nádory, meningeomy, gliomy, hemangioblastomy a neuroektodermální nádory. Léčebná strategie je závislá především na anatomickém uložení a histologickém typu nádoru. S výjimkou germinativních tumorů je metodou volby u všech nádorů chirurgická resekce. Metody: Mikrochirurgické přístupy: V současné době je nejrozšířenější mikrochirurgický supracerebelární-infratentoriální přístup. I přes jistá rizika umožňuje přehledný, kompletně extracerebrální přístup až do pineální krajiny s minimální retrakcí mozečku. Druhý základní přístup je mikrochirurgický okcipitální-transtentoriální přístup, který lze s výhodou použít, pokud nádor zasahuje výrazně supratentoriálně. Další možností je interhemisferický-transkalózní přístup či transkortikální-transventrikulární přístup. Endoskopické přístupy: Neuroendoskopie umožnuje minimálně invazivním přístupem provést biopsii nádoru a v jedné době léčit i případný hydrocefalus. Stereotaktická biopsie: Stereotaktická jehlová biopsie představuje alternativu k endoskopické biopsii u pacientů bez hydrocefalu nebo u dorzálně uložených lézí, které nejsou přístupné ze třetí komory. Závěr: Současná neurochirurgie disponuje širokou paletou operačních přístupů k pineální krajině. Komplexní problematika nádorů v této oblasti vyžaduje individualizovanou léčbu, pečlivé předoperační plánování a precizní chirurgickou techniku.

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.

• MeSH
• Diagnosis, Differential MeSH
• Pineal Gland anatomy & histology   physiology   pathology   MeSH
• Biopsy, Needle MeSH
• Craniotomy MeSH
• Humans MeSH
• Minimally Invasive Surgical Procedures MeSH
• Neurosurgical Procedures methods   MeSH
• Neuroendoscopy utilization   MeSH
• Pinealoma * diagnosis   etiology   surgery   MeSH
• Stereotaxic Techniques MeSH
• Check Tag
• Humans MeSH
• Publication type
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH

Oblast kvadrigeminální cisterny s přilehlými anatomickými strukturami, v jejímž centru se nachází glandula pinealis, je obecně označována jako pineální krajina. Seznam možných místních expanzí je vzhledem k významné cytologické heterogenitě této lokality poměrně široký. Začíná nezhoubnými pineálními cystami a končí vysoce zhoubnými nádory. Tumory, které je možné rozdělit zhruba do tří základních skupin, představují asi 1 % všech intrakraniálních nádorů. Nejpočetnější zastoupení mají germinální nádory. Pineální parenchymatózní nádory představují druhou skupinu a do třetí jsou řazeny ostatní nádory vyrůstající z tkání okolních anatomických struktur (gliomy, ependymomy, meningeomy, metastázy, dermoidní a epidermoidní cysty a další). Optimální léčba a prognóza může být stanovena pouze na základě jejich histologického vyšetření. Chirurgické řešení je vzhledem k centrální lokalizaci těchto lézí a jejich vztahu k životně důležitým nervovým a cévním strukturám velmi obtížné. Perioperační morbidita a mortalita byla v současné době významně redukována díky rozvoji mikrochirurgických technik. Také byla prodloužena doba přežití u maligních forem díky agresivní adjuvantní léčbě.

Quadrigeminal cistern and the surrounding anatomical structures with the pineal gland at their center are usually referred to as pineal region. Due to great histological diversity of this location, the list of possible local expansions is relatively extensive, ranging from benign pineal cysts to highly malignant tumors. Neoplasms of the pineal region represent about one percent of all intracranial tumors and can be divided into three main groups. Germ cell tumors form the largest group, followed by pineal parenchymal tumors. The third group is composed of different types of tumors growing from tissues of the surrounding anatomical structures (gliomas, ependymomas, meningiomas, metastases, dermoid and epidermoid cysts etc.). Optimal treatment and prognosis can only be determined on the basis of histological examination. Central location of the pineal region with relationship to vital neural and vascular structures makes surgery of these lesions very difficult. However, microsurgical techniques have dramatically reduced perioperative morbidity and mortality and aggressive adjuvant therapy of malignant forms have prolonged survival. Key words: pineal gland – pineal region tumors – germ cell tumors – pineal parenchymal tumors – surgical approaches – radiotherapy – chemotherapy The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manu­script met the ICMJE “uniform requirements” for biomedical papers.

• Keywords
• glandula pinealis, chemoterapie, chirurgické přístupy, pineální parenchymatózní nádory, germinální nádory, nádory pineální krajiny,
• MeSH
• Biopsy methods   MeSH
• Pineal Gland * anatomy & histology   physiopathology   pathology   MeSH
• Drug Therapy MeSH
• Neoplasms, Germ Cell and Embryonal classification   pathology   therapy   MeSH
• Hydrocephalus complications   MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Meningioma diagnosis   pathology   therapy   MeSH
• Biomarkers, Tumor blood   MeSH
• Neurosurgical Procedures * methods   MeSH
• Pinealoma * diagnosis   pathology   therapy   MeSH
• Tomography, X-Ray Computed MeSH
• Posture MeSH
• Radiotherapy MeSH
• Check Tag
• Humans MeSH

Úvod: Primárne intrakraniálne germinatívne tumory predstavujú zriedkavú skupinu nádorov, ktoré sa vyskytujú v detstve a u mladých dospelých. WHO klasifikácia delí intrakraniálne tumory na germinómy a negerminómy. Najčastejšia lokalita týchto nádorov je pineálna a supraselárna oblasť. Klinické príznaky a symptómy závisia od lokalizácie tumoru, najčastejšie sú to príznaky v dôsledku zvýšeného intrakraniálnho tlaku, Parinaudov syndróm, bitemporálna hemianopsia, príznaky z endokrinného deficitu. Zobrazovacím vyšetrením voľby pri diagnostike intrakraniálnych germinatívnych tumorov je MRI vyšetrenie mozgu s použitím kontrastnej látky gadolinium. Zobrazovacie vyšetrenia však neposkytnú dostatočnú informáciu o type nádoru, k tomu je potrebná bioptizácia. Výnimkou sú prípady s charakteristicky zvýšenými hladinami nádorových markerov (AFP a β-hCG) stanovenými v sére a cerebrospinálnom moku. Klinický prípad: U 26-ročného pacienta so zhoršeným zrakom s poruchou zaostriť a svetloplachosťou, výraznou únavou a ospalosťou, nevoľnosťou, občasným zvracaním, s intermitentnými bolesťami hlavy a klinicky prítomným Parinaudovým syndróm sa zistil germinatívny nádor pineálnej oblasti. Zrealizované MR vyšetrenie mozgu ukázalo tumoróznu expanziu v pineálnej oblasti a v pravej časti mezencefala. Bola vykonaná radikálna exstirpácia tumoru v pineálnej oblasti. Pri kontrolnom MR vyšetrení mozgu sa zistila recidíva ochorenia. Pacient absolvoval kraniospinálne ožiarenie s následnou pooperačnou chemoterapiou (schéma cisplatina a etoposid), celkovo tri cykly. V súčasnej dobe je pacient 30 mesiacov po ukončení úspešnej onkologickej liečby v klinickej remisii ochorenia. Záver: Liečba a prognóza tohto nádorového ochorenia je rozdielna v jednotlivých skupinách. Germinómy majú lepšie prežívanie než negerminómy. 5-ročné prežívanie germinómov po aplikácii samotnej rádioterapie bolo > 90 %. Pridanie chemoterapie viedlo k zníženiu dávky a zmenšeniu ožarovanej oblasti, s dosiahnutím menších nežiadúcich účinkov a bez zníženia kurability. Negerminómy sú menej rádiosenzitívne než germinómy, ale použitím adjuvantnej chemoterapie sa dosiahlo zlepšenie prežívania. Naďalej však menežment týchto nádorov ostáva kontroverzný.

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, Parinaud’s 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 cere­brospinal 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 Parinaud’s 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
• Chemotherapy, Adjuvant MeSH
• Radiotherapy, Adjuvant MeSH
• alpha-Fetoproteins secretion   MeSH
• Headache MeSH
• Adult MeSH
• Pineal Gland surgery   pathology   MeSH
• Gadolinium diagnostic use   MeSH
• Germinoma * diagnosis   surgery   pathology   radiotherapy   MeSH
• Combined Modality Therapy MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Biomarkers, Tumor MeSH
• Brain Neoplasms * diagnosis   pathology   therapy   MeSH
• Pinealoma diagnosis   pathology   MeSH
• Ocular Motility Disorders etiology   MeSH
• Antineoplastic Agents MeSH
• Vomiting MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH
• Review MeSH

Popisujeme případ pětileté dívky s rozsáhlým nádorem pineální oblasti, třetí i čtvrté mozkové komory, který se prezentoval příznaky syndromu intrakraniální hypertenze. Byla provedena endoskopická ventrikulostomie s biopsií tumoru, jenž byl odečten jako papilární nádor pineální oblasti. Po radikální resekci došlo s odstupem 14 měsíců k lokální recidivě, která byla ošetřena stereotaktickou zevní frakcionovanou radioterapií. Papilární nádor pineální oblasti je vzácný nádor s obtížnou diagnostikou, nejistou prognózou a vysokým rizikem lokální recidivy. Jediný známý prognostický faktor je radikalita resekce. Většina dospělých pacientů je léčena kombinací chirurgického výkonu a následné lokální radioterapie. Standardní léčba pro děti není jasně definována.

We describe a case of a five years old girl with a large tumor of the pineal region, the 3rd and 4th ventricles, presented with the increased intracranial pressure syndrome. Endoscopic third ventriculostomy with tumor biopsy was performed, and the tumor was classified as papillary tumor of the pineal region. Local relapse occurred fourteen months after complete resection and it was treated with stereotactic external fractionated radiotherapy. Papillary tumor of the pineal region is a rare tumor with a difficult diagnosis, uncertain prognosis, and a high risk of local recurrence. The only defined prognostic factor is radicality of the resection. Most adult patients are treated with a combination of surgery and adjuvant local radiotherapy. A standard of care for children has not been clearly defined yet.

• Keywords
• chemoterapie, pineální oblast,
• MeSH
• Biopsy MeSH
• Pineal Gland surgery   pathology   MeSH
• Dose Fractionation, Radiation MeSH
• Immunohistochemistry MeSH
• Remission Induction MeSH
• Intracranial Hypertension etiology   MeSH
• Humans MeSH
• Neoplasm Recurrence, Local diagnosis   radiotherapy   MeSH
• Magnetic Resonance Imaging MeSH
• Brain Neoplasms * diagnosis   surgery   pathology   MeSH
• Neurosurgical Procedures methods   MeSH
• Carcinoma, Papillary * diagnosis   surgery   pathology   MeSH
• Pinealoma diagnosis   surgery   pathology   MeSH
• Child, Preschool * MeSH
• Disease-Free Survival MeSH
• Prognosis MeSH
• Radiotherapy MeSH
• Stereotaxic Techniques MeSH
• Check Tag
• Humans MeSH
• Child, Preschool * MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

• MeSH
• Child MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Brain Neoplasms * diagnosis   etiology   therapy   MeSH
• Pinealoma diagnosis   therapy   MeSH
• Child, Preschool MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Case Reports MeSH