Pineal Germinoma Research Articles

A rare case of pineoblastoma – Case report

Pineoblastoma is a very rare and aggressive malignant tumor of pineal gland. We report a case of 12-year-old male patient presented to S.V.P. tertiary care hospital with complain of progressive loss of vision in both eyes with nausea and headache. On examination bilateral upward gaze palsy was present. The magnetic resonance imaging report revealed possibility of pineal Germinoma. The patient underwent ventriculoperitoneal shunt insertion surgery for obstructive hydrocephalus and suboccipital craniectomy for tumor resection. The Histopathological examination report stated the tumor as Pineoblastoma, Grade IV. Immunohistochemistry studies were positive for synaptophysin and Integrase interactor-1 (INI-1) markers with high ki-67 labelling index and negative for CD-117 and Glial fibrillary acid protein (GFAP) markers. Microscopic and Immuno histochemisty findings are important to differentiate pineoblastoma from other pineal region tumors.

Plasma miRNA expression profile in pediatric pineal pure germinomas.

Pure germinomas account for 40% of pineal tumors and are characterized by the lack of appreciable tumor markers, thus requiring a tumor biopsy for diagnosis. MicroRNAs (miRNA) have emerged as potential non-invasive biomarkers for germ cell tumors and may facilitate the non-invasive diagnosis of pure pineal germinomas. A retrospective chart review was performed on all patients treated at the Children's Cancer Hospital Egypt diagnosed with a pineal region tumor between June 2013 and March 2021 for whom a research blood sample was available. Plasma samples were profiled for miRNA expression, and DESeq2 was used to compare between pure germinoma and other tumor types. Differentially expressed miRNAs were identified. The area under the curve of the receive;r operating characteristic curve was constructed to evaluate diagnostic performance. Samples from 39 pediatric patients were available consisting of 12 pure germinomas and 27 pineal region tumors of other pathologies, including pineal origin tumors [n = 17; pineoblastoma (n = 13) and pineal parenchymal tumors of intermediate differentiation (n = 4)] and others [n = 10; low-grade glioma (n = 6) and atypical teratoid rhabdoid tumor (n = 4)]. Using an adjusted p-value <0.05, three miRNAs showed differential expression (miR-143-3p, miR-320c, miR-320d; adjusted p = 0.0058, p = 0.0478, and p = 0.0366, respectively) and good discriminatory power between the two groups (AUC 90.7%, p < 0.001) with a sensitivity of 25% and a specificity of 100%. Our results suggest that a three-plasma miRNA signature has the potential to non-invasively identify pineal body pure germinomas which may allow selected patients to avoid the potential surgical complications.

A Pineal Germinoma with Rapid Enlargement following Tumor Resection

The natural course of pineal germ cell tumors (GCTs), particularly their post-operative progression, is not well understood. We report a rare case of pineal region GCT showing rapid enlargement within 2 weeks following surgical resection. A young adult male presented with progressive headache and diplopia for several weeks. Although elevation of β-human chorionic gonadotropin (β-HCG) and α-fetoprotein (AFP) levels suggested that a large pineal mass lesion observed on magnetic resonance imaging (MRI) might be a β-HCG/AFP-producing tumor, whether the mass was truly a GCT remained unclear. We performed an endoscopy-assisted suboccipital infratentorial approach with removal of the tumor that was diagnosed as germinoma via histopathological investigation. During the week preceding chemotherapy, the patient’s consciousness rapidly worsened. MRI showed that the residual pineal germinoma had enlarged and even compressed the tectum and thalamus. Emergency chemotherapy and radiotherapy were prescribed, and the patient received invasive ventilation for respiratory failure. Unexpectedly, the patient recovered within a short period. Importantly, total regression of the pineal germinoma, accompanied by β-HCG and AFP levels returning to normal range, was observed 4 months after chemotherapy. These phenomena suggest that the rapid enlargement of the pineal germinoma, which might be induced by aggressive surgical cytoreduction, responds well to chemoradiotherapy.

Machine learning-based classification of pineal germinoma from magnetic resonance imaging

IntroductionSurgical approaches for tissue diagnosis of pineal tumors have been associated with morbidity and mortality. The classification of images by machine learning (ML) may assist physicians in determining the extent of resection and treatment plans for a specific patient. Therefore, the present study aimed to evaluate the diagnostic performances of the ML-based models for distinguishing between pure and non-germinoma of the pineal area. In addition, the secondary objective was to compare diagnostic performances among feature extraction methods. MethodsThis is a retrospective cohort study of patients diagnosed with pineal tumors. We used the RGB feature extraction, histogram of oriented gradients (HOG), and local binary pattern methods from magnetic resonance imaging (MRI) scans; therefore, we trained an ML model from various algorithms to classify pineal germinoma. Diagnostic performances were calculated from a test dataset with several diagnostic indices. ResultsMRI scans from 38 patients with pineal tumors were collected and extracted features. As a result, the k-nearest neighbors (KNN) algorithm with HOG had the highest sensitivity of 0.81 (95% CI 0.78–0.84), while the random forest (RF) algorithm with HOG had the highest sensitivity of 0.82 (95% CI 0.79–0.85). Moreover, the KNN model with HOG had the highest AUC, at 0.845. Additionally, the AUCs of the artificial neural network and RF algorithms with HOG were 0.770 and 0.713, respectively. ConclusionsThe classification of images using ML is a viable way for developing a diagnostic tool to differentiate between germinoma and non-germinoma that will aid neurosurgeons in treatment planning in the future.

Major improvement of diabetes insipidus 15 years after diagnosis of panhypopituitarism in a patient with pineal germinoma - a case report

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Diagnosis and Management of Pineal Germinoma: From Eye to Brain.

Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner, addressing the anatomic relationships that provide the basis for the uniqueness of pineal germinoma. Ocular findings and signs and symptoms of elevated intracranial pressure are the keys to suspecting the diagnosis and obtaining the necessary imaging and cerebrospinal fluid studies. Other symptoms can suggest spread beyond the pineal region. Surgery may only be needed to obtain tissue for a definitive diagnosis, as germinoma is highly responsive to chemotherapy and focused radiation therapy. Hydrocephalus, usually related to tumor obstruction of the cerebral aqueduct, may also need to be addressed. Outcome for pineal germinoma is usually excellent, but relapse can occur and may require additional intervention. These issues are detailed in this review.

Pineal gland germinoma and pituitary insufficiency presenting with weight loss: Rare Case

Pineal gland germinoma and pituitary insufficiency presenting with weight loss: Rare Case

PMON107 Pineal Germinoma with Pituitary Involvement: Long-Term Survival following Treatment with Whole Ventricular Radiation

Abstract Introduction Intracranial germinomas usually arise from midline structures such as the pineal gland or suprasellar region. Involvement of both structures occur in 6% of cases. We are reporting long-term follow up of a patient with pineal germinoma with pituitary involvement treated successfully with whole ventricular radiation. Clinical Case A 36-year-old man presented with polydipsia and polyuria 12 years ago. He also reported fatigue and cold intolerance. Vital signs: BP 130/75 mmHg, HR 69 bpm. Physical examination demonstrated moist mucous membranes and the rest of the examination was unremarkable. Labs: serum albumin 4.9 g/dL, calcium 9.8 mg/dL, glucose 91 mg/dL, potassium 4.2 mEq/L, sodium 145 mEq/L, and serum osmolality 294 mOsm/kg (reference 275-295). The patient also had multiple urinalyses showing a specific gravity of 1.005. Results of a water deprivation test: urine osmolality 114 mOsm/kg (50-1350) at 0805, 87 mOsm/kg at 0858, 73 mOsm/kg at 0955, 80 mOsm/kg at 1045, 88 mOsm/kg at 1150, and 102 mOsm/kg at 1253. His serum antidiuretic hormone during the water deprivation test was &amp;lt;0.8 pg/mL (ref 0.0-4.7). Since these labs suggested central diabetes insipidus, a pituitary MRI was performed that demonstrated two mass lesions in the region of the cephalad portion of the pituitary stalk, hypothalamus, and optic chiasm as well as in the pituitary gland region. Biopsy of the mass lesions was consistent with germinoma. MRI of the spine revealed no evidence of disseminated disease. Additional labs: TSH 1.88 mIU/L, free thyroxine 0.46 ng/dL, IGF-1 62 ng/mL (109-284), prolactin 25 ng/mL, FSH 1.9 mIU/mL, LH 2.5 mIU/mL, total testosterone 183 ng/dL, and cortisol 3.2 ug/dL. An ACTH stimulation test confirmed central adrenal insufficiency. The patient was treated with radiation therapy to the whole ventricles with for a total dose of 45 Gray per cycle. Follow-up MRI in our patient following treatment demonstrated no evidence of persistent or recurrent disease. However, he continues desmopressin for diabetes insipidus and levothyroxine 88 mcg daily for central hypothyroidism whereas the adrenal insufficiency has completely resolved. The patient has been without disease recurrence for the last 12 years and is doing well with a good quality of life. Radiation therapy alone has been highly effective in the management of intracranial germinomas with an overall survival of up to 85%. However, recent studies have shown that chemotherapy followed by lower dose of complete ventricular radiation is as effective as high dose ventricular radiation for local or bifocal germinomas with lower risk of long-term consequences. Conclusion This case illustrates that long-term survival can be achieved in patients with germinomas following appropriate treatments. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Evaluation of the Perioperative and Postoperative Course of Surgery for Pineal Germinoma in the SIOP CNS GCT 96 Trial.

Simple SummaryThere are many studies that discuss pineal tumors (not specifically germinoma). Some advocate for biopsy over resection due to the fact that germinoma is radio- and chemosensitive; others advocate for primary resection due to multiple differential diagnoses that require resection in the pineal region, such as pineocytoma, teratoma, meningioma, epidermoid, etc. However, with respect to solitary pineal germinoma, there is still no study that analyzes and compares both surgical options regarding peri- and postoperative courses and complications. We evaluated the perioperative course and postoperative complications of patients with isolated pineal germinoma who underwent a primary biopsy or resection, treated according to the European SIOP CNS GCT 96 trial. The key finding of our study was that postoperative complications, including hydrocephalus, Parinaud syndrome, double vision, etc., were significantly higher in patients who underwent primary resection (p < 0.008).Background: CNS germinoma, being marker-negative, are mainly diagnosed by histological examination. These tumors predominantly appear in the suprasellar and/or pineal region. In contrast to the suprasellar region, where biopsy is the standard procedure in case of a suspected germ-cell tumor to avoid mutilation to the endocrine structures, pineal tumors are more accessible to primary resection. We evaluated the perioperative course of patients with pineal germinoma who were diagnosed by primary biopsy or resection in the SIOP CNS GCT 96 trial. Methods: Overall, 235 patients had germinoma, with pineal localization in 113. The relationship between initial symptoms, tumor size, and postoperative complications was analyzed. Results: Of 111 evaluable patients, initial symptoms were headache (n = 98), hydrocephalus (n = 93), double vision (n = 62), Parinaud syndrome (n = 57), and papilledema (n = 44). There was no significant relationship between tumor size and primary symptoms. A total of 57 patients underwent primary resection and 54 underwent biopsy. Postoperative complications were reported in 43.2% of patients after resection and in 11.4% after biopsy (p < 0.008). Biopsy was significantly more commonly performed on larger tumors (p= 0.002). Conclusions: These results support the practice of biopsy over resection for histological confirmation of pineal germinoma.

Non-invasive preoperative imaging differential diagnosis of pineal region tumor: A novel developed and validated multiparametric MRI-based clinicoradiomic model

BackgroundPreoperative differential diagnosis of pineal region tumor can greatly assist clinical decision-making and avoid economic costs and complications caused by unnecessary radiotherapy or invasive procedures. The present study was performed to pre-operatively distinguish pineal region germinoma and pinealoblastoma using a clinicoradiomic model by incorporating radiomic and clinical features. Methods134 pineal region tumor patients (germinoma, 69; pinealoblastoma, 65) with complete clinic-radiological and histopathological data from Tiantan hospital were retrospectively reviewed and randomly assigned to training and validation sets. Radiomic features were extracted from MR images, then the elastic net and recursive feature elimination algorithms were applied to select radiomic features for constructing a fusion radiomic model. Subsequently, multivariable logistic regression analysis was used to select the clinical features, and a clinicoradiomic model incorporating the fusion radiomic model and selected clinical features was constructed for individual predictions. The calibration, discriminating capacity, and clinical usefulness were also evaluated. ResultsSeven significant radiomic features were selected to construct a fusion radiomic model that achieved an area under the curve (AUC) value of 0.920 and 0.880 in the training and validation sets, respectively. A clinicoradiomic model that incorporated the radiomic model and four selected clinical features was constructed and showed good discrimination and calibration, with an AUC of 0.950 in the training set and 0.940 in the validation set. The analysis of the decision curve showed that the radiomic model and clinicoradiomic model were clinically useful for patients with pineal region tumor. ConclusionsOur clinicoradiomic model showed great performance and high sensitivity in the differential diagnosis of germinoma and pinealoblastoma, and could contribute to non-invasive development of individualized diagnosis and treatment of patients with pineal region tumor.

Epidemiology, Management, and Long-Term Survival Outcomes of Intracranial Typical Site Germinomas: An Analysis of the Surveillance, Epidemiology, and End-Results (SEER) Database

BackgroundThe correlations of epidemiological characteristics and clinical outcomes with different tumor sites in patients with intracranial typical site germinomas (ICTSGs) have not yet been well established. We analyzed ICTSGs using a multicenter database, focusing on its demographic, management patterns, and long-term survival outcomes.MethodsPatients diagnosed with ICTSGs were selected from the Surveillance, Epidemiology, and End-Results (SEER) database. Demographic information and management patterns of ICTSGs were extracted for data analysis stratified by different tumor sites. Kaplan–Meier curves were used to evaluate the survival outcome stratified by treatment, tumor site and tumor size.ResultsAmong the 327 patients enrolled in the study, 16.21% had tumors located in the suprasellar region and 83.79% in the pineal region. The proportion of males was significantly higher among pineal germinomas (94.16 vs 66.04%; P < .001). Smaller tumors (<24 mm) were more common in the suprasellar region (37.74 vs 18.87%; P < .001). A higher percentage of patients with suprasellar germinomas underwent surgery. Radiotherapy (RT) and chemotherapy (CT) was, respectively, administered to 82.97 and 60.61% of patients during the treatment period, with no significant difference between suprasellar and pineal germinomas. CT plus RT was the most common treatment modality for both pituitary (30.19%) and pineal (33.94%) germinomas. Both RT and CT were associated with improved long-term survival. No survival difference was observed between suprasellar and pineal germinomas.ConclusionsDespite significant differences in epidemiology and management, pineal and suprasellar germinomas had a similar long-term clinical outcome.

Characteristics of growth disturbances in patients with intracranial germinomas of different origins.

To explore the characteristics of growth disturbance in patients with intracranial germinoma with different origins. Clinical data of 151 patients with single-origin germinomas were studied retrospectively. Z-score of height (ZSOH) at both diagnosis and the last follow-up was calculated using the WHO AnthroPlus software. Linear regression was used to analyse the correlation between the absolute change in ZSOH (|ZSOH last follow-up - ZSOH diagnosis|) and clinical factors. The mean ZSOH decreased significantly in every origin subgroup at the last follow-up. In patients with sellar germinoma (n = 62), the mean ZSOH values at both diagnosis and the last follow-up were significantly lower than those in patients with pineal (n = 30) (p < 0.001) or basal ganglia germinomas (n = 59) (p < 0.001), respectively. In patients with basal ganglia germinoma, the mean absolute change in ZSOH decreased significantly compared to that in the patients with sellar (p = 0.006) or pineal germinomas (p = 0.04). Linear analysis revealed that sex (male vs female; p = 0.003) and age at diagnosis (≤10 years vs >10 years; p = 0.026) had negative correlations, while radiation dose at the hypothalamic-pituitary axis (HPA) (≤40 Gy vs >40 Gy; p = 0.085) had a marginally positive correlation, with absolute change in ZSOH. Patients with germinoma experienced growth retardation after treatments. The growth disturbance was consistent and more severe in patients with germinoma of sellar origin, while the greatest aggravation was observed in patients with germinoma of basal ganglia origin. Decreasing radiation dose to the HPA may minimize the negative impact of radiotherapy on growth.

Management trends and outcomes of pineal germinoma in a multi-institutional Australian cohort

Pineal germinoma is rare with high cure rates following craniospinal radiotherapy. Efforts to reduce the radiotherapy dose and field via combination with chemotherapy suggest comparable disease control and reduced neurocognitive impairments, while the efficacy of immunotherapy in pineal germinoma remains undetermined. This report aimed to review clinical outcomes in patients treated for pineal germinoma in Queensland, Australia, and assess for Programmed Death-Ligand1 (PD-L1) expression. Patients who commenced radiation and/or chemotherapy for pineal germinoma from 2005 to 2017 were retrospectively identified using Queensland Oncology Online database. Demographic, diagnostic, treatment, and outcome data was obtained from electronic medical records. PD-L1 immuno-histochemistry was performed on available specimens. Eighteen patients with long-term follow-up data were identified. Median age at diagnosis was 16.8 years (range 9–46 years). Diagnosis was made histologically in fifteen patients, and radiologically in three. All patients underwent radiotherapy (median 36 Gy (range 21–54 Gy)) with lower median dose delivered with whole ventricle irradiation (12/18patients) than craniospinal irradiation (5/18patients). Sixteen patients received chemotherapy preceding radiotherapy. All patients are alive at median 7.25 years from primary treatment completion (range 2.03–13.1 years). Relapse occurred in three patients (16.67%) following treatment response, all of whom achieved remission following high-dose chemotherapy with stem-cell support and craniospinal radiotherapy. Post-treatment functional outcomes were similarly excellent. PD-L1 expression was low (1–49% cells) or negative in 87% of tumours tested but results were confounded by specimen quality and availability. Reduced-dose radiotherapy with chemotherapy does not compromise outcome and is standard of care at this institution. Immunotherapy is unlikely to become standard treatment in the near future.

Treatment outcomes in patients with central nervous system germinoma (neuro-ophthalmic aspects)

The analyze the neuro-ophthalmic outcomes in patients with pineal and suprasellar germinoma after complex treatment. There were 125 patients (88 males, 37 females) with primary CNS germinoma for the period 2008-2017. All patients were divided into 3 groups depending on the tumor localization: pineal area - 62 patients, suprasellar neoplasm - 38 patients, bifocal germinoma - 25 patients. Treatment was performed in accordance with the «Germinoma-2008» protocol. Tumors were histologically patients (partial - 32 cases, subtotal - 16 cases). Surgery was followed by postoperative chemotherapy and stereotactic radiotherapy. The results were evaluated by neuro-ophthalmological examination at all stages of complex treatment. Pineal germinoma resulted oculomotor and pupillary disorders (58 patients - 94%) and papilledema. The last one completely regressed under the treatment. Partial reduction of oculomotor and pupillary abnormalities occurred throughout chemotherapy (6 patients - 10%). More than half of patients (n=24, 63%) with suprasellar germinoma had visual impairment. Improvement of visual functions including severe visual disturbance was observed in 14 patients (58%). Predominantly, functional recovery occurred under chemotherapy (12 patients - 86%). Bifocal germinoma was followed by various combinations of oculomotor, pupillary and/or visual impairments. We observed regression of only mild visual disorders. Stereotactic radiotherapy did not ensure any changes in neuro-ophthalmic symptoms in all groups.

Нейроофтальмологическая симптоматика и ее динамика у больных после комплексного лечения первичной герминомы пинеальной области

Нейроофтальмологическая симптоматика и ее динамика у больных после комплексного лечения первичной герминомы пинеальной области

GCT-49. EVALUATION OF THE PERIOPERATIVE AND POSTOPERATIVE COURSE OF SURGERY OF PINEAL GERMINOMA ACCORDING TO THE SIOP CNS GCT 96 TRIAL

INTRODUCTIONCNS germinoma, being marker-negative, are diagnosed by surgical biopsy. Here we evaluate the perioperative status and postoperative complications of patients with pineal germinoma who underwent a primary biopsy or resection, treated according to SIOP CNS GCT 96.METHODS235 patients with histologically confirmed germinoma were registered, of which 113 were pineal: 55 were biopsied and 58 underwent primary resection. Initial symptoms, tumour size, complications and neurological status were assessed. 111 patients were evaluable.RESULTSPure germinoma was present in 101 patients; 10 had additional teratoma components. The main clinical symptoms at diagnosis were headache (n=98), hydrocephalus (n=93), double vision (n=62), Parinaud syndrome (n=57) and papilloedema (n=44). Tumour size was documented in 81 patients (<2cm, n=14; 2-3cm, n=35; ≥3cm, n=32). 17 patients underwent primary total resection, 14 subtotal resection >50%, 26 subtotal resection <50%, 39 stereotactic biopsy, 11 endoscopic biopsy, 2 open biopsy and 2 not documented. The postoperative neurological status after resection was improved in 23 patients, unchanged in 27, deteriorated in 6 and not documented in one. Clinical status after biopsy improved in 26 patients, was unchanged in 15, deteriorated in 2 and not documented in 11. Postoperatively, 16/57 patients after resection and 5/54 after biopsy developed complications (Parinaud syndrome, double vision and hydrocephalus).CONCLUSIONAlthough surgical techniques have improved within recent decades, these results support the practice of biopsy over resection for histological confirmation of germinoma arising at the pineal site. Supported in part by German Cancer Aid.

Could Aberrant Migration Explain Metachronous Germ Cell Tumors?

Background Extragonadal germ cell tumors (GCTs) are thought to arise as a result of local transformation of primordial gonadal cells (PGCs) that become misplaced during embryogenesis. With the exception of bilateral testis tumors, metachronous GCT (i.e., occurring at a site classically described for primary GCTs) are rare events. Patients and Methods The clinical, radiological, and molecular data (if available) of patients with metachronous GCT were analyzed. Results Three Caucasian males were identified: case 1 presented with a pineal germinoma 19 years after a mediastinal seminoma that had been treated with chemotherapy, case 2 presented with a pineal non-seminomatous GCT (NSGCT) that occurred three years after a mediastinal seminoma treated with chemotherapy, and case 3 presented with a mediastinal seminoma concomitant with a suprasellar germinoma that occurred two years after a stage I testicular NSGCT treated exclusively with surgery. None of these patients had a positive family history or disorder of sex development. Molecular data were available for cases 2 and 3. In case 2, a CHEK2 gene biallelic inactivation in the second tumor suggested chemoresistance to cisplatin. This was further confirmed by tumor progression during second-line treatment. In case 3, the molecular analysis revealed different profiles in the three tumors, thus suggesting distinct tumor cell origins. Conclusion These rare cases should alert clinicians of the possibility of multiple GCTs that should not be considered to be relapses. The underlying physiopathology is unknown, but multiple PGC mismigrations is a likely cause. Initial treatment with cisplatin may select chemo-resistant clones, thereby making the subsequent treatment more of a challenge.

A Rare Case of Primary CNS Germinoma in the Pineal Region with Long-standing Hearing Loss

Germinomas in the brain are rare neoplasms which make up less than 1% of all intracranial tumors. Their clinical presentation is usually related to the increased intracranial pressure and/or hydrocephalus. This report describes a case who presented to our hospital for headaches and visual changes, including Parinaud's syndrome, that had started recently. The patient also had been complaining of chronic bilateral hearing loss for several years which seemed not properly evaluated previously. MRI brain showed a markedly enhancing lesion in the pineal region. CSF and serum tumor markers including B-human chorionic gonadotropin (B-HCG) and alfa-fetoprotein (AFP) were normal. Lumbar puncture with cytology was negative for malignancy. Biopsy was taken from the mass and sent to the pathology lab for testing. Histopathological examination showed changes consistent with germinoma and the immunohistochemical staining including that for placental alkaline phosphatase (PLAP) confirmed the diagnosis. There was no evidence of metastasis and the patient was treated with radiation-alone therapy that resulted in marked regression of the mass and significant clinical improvement except for the issue of hearing loss which had persisted, as expected, given its long-standing existance. In addition to highlighting the diagnostic evaluation and treatment approach for such rare tumors, this case suggests considering pineal germinomas among the differential diagnosis when dealing with hearing loss which needs careful evaluation and should never be underestimated.

Neuro-ophthalmological symptoms in patients with pineal and suprasellar germinoma

To analyze neuro-ophthalmology symptoms in patients with germinomas located in pineal and suprasellar regions. The study analyzed 133 patients with germinoma who were receiving treatment in Burdenko Neuro-surgical Institute from 2008 to 2017. Assessment of results was performed by neuro-ophthalmology examination, a computerized oculograph was used for the objectification of the severity of ocular-motor and pupillary disorders. Among the patients of the study, 65 had pineal germinoma, 42 - suprasellar germinoma and 26 - bifocal germinoma. Among patients with pineal germinomas, males prevailed, while more females had suprasellar germinomas. No statistical difference was found between gender and bifocal germinoma. Pineal germinomas were manifested predominantly by the development of the dorsal midbrain syndrome in the form of oculomotor and pupillary dysfunction (75%) and the development of papilledema (53%). Germinomas of suprasellar localization are most often presented by asymmetric chiasmatic syndrome (24%). Dorsal midbrain syndrome in patients with bifocal germinomas was detected in 76% cases, combination of oculomotor and pupillary dysfunction with visual impairments was found in 38% cases. Neuro-ophthalmological symptoms of germinoma of these regions manifest in the early stages of the disease and require ophthalmological examination during all stages of diagnosis and treatment.

Management of hydrocephalus in a pediatric bifocal germinoma

Germinomas represent the most frequent tumour type in the pineal region. However, the synchronous involvement of the suprasellar region, frequently termed bifocal intracranial germinoma (BFG) is quite rare. A literature search identified a further of 48 pediatric cases with a median age of 12.9 years. BFG is known to be highly radiosensitive. We presented a clinical case of pediatric bifocal germinoma. A 14-year-old boy presented with clinical features of progressive headaches since three months associated with vomits. MR showed two distinct lesions in the pineal and suprasellar regions. Neuroaxis spine MR was normal. Tumor markers for germ cell tumors from blood and cerebrospinal fluid were within the normal range. An endoscopic procedure was performed to treat the hydrocephalus (endoscopic third ventriculostomy) and, in the same procedure the suprasellar lesion was biopsied and the histology revealed a germinoma. She received chemotherapy and a focal radiotherapy covering the sites of primary disease and the ventricular system. Three months after the completion of treatment, the patient achieved a complete clinical and radiological response, which was maintained during the 5-years follow-up period. The optimum management of primary intracranial pineal and suprasellar (bifocal) germinomas still remains controversial. The definition as either localized or disseminated disease has major implications on required treatment and its associated late morbidity. Prospective studies with large cooperative groups worldwide and consensus meetings may reduce uncertainties regarding available management options.