Clinical significance of cerebral microbleeds in patients with germinoma who underwent long-term follow-up.

PURPOSE: This study identified the factors affecting cerebral microbleed (CMBs) development. Moreover, their effects on intelligence and memory and association with stroke in patients with germinoma who had long-term follow-up were evaluated. METHODS: This study included 64 patients with germinoma who were histologically and clinically diagnosed with and treated for germinoma. These patients were evaluated cross-sectionally, with a focus on CMBs on susceptibility-weighted magnetic resonance imaging (SWI), brain atrophy assessed through volumetric analysis, and intelligence and memory. RESULTS: The follow-up period was from 32 to 412 (median: 175.5) months. In total, 43 (67%) patients had 509 CMBs and 21 did not have CMBs. Moderate correlations were observed between the number of CMBs and time from initial treatments and recurrence was found to be a risk factor for CMB development. Increased temporal CMBs had a marginal effect on the processing speed and visual memory, whereas brain atrophy had a statistically significant effect on verbal, visual, and general memory and a marginal effect on processing speed. Before SWI acquisition and during the follow-up periods, eight strokes occurred in four patients. All of these patients had ≥ 15 CMBs on SWI before stroke onset. Meanwhile, 33 patients with < 14 CMBs or 21 patients without CMBs did not experience stroke. CONCLUSION: Patients with a longer time from treatment initiation had a higher number of CMBs, and recurrence was a significant risk factor for CMB development. Furthermore, brain atrophy had a stronger effect on memory than CMBs. Increased CMBs predict the stroke onset.

Diffuse peritoneal dissemination of intracranial pure germinoma via ventriculoperitoneal shunt.

Germinomas frequently cause hydrocephalus, and ventriculoperitoneal shunts (VPS) have been commonly used for their management. Although VPS can potentially serve as a route for peritoneal dissemination of germinomas, the abdominal imaging characteristics of this rare yet important complication remain unknown. In this article, we report the computed tomography imaging findings of diffuse peritoneal dissemination of intracranial germinoma.

Intracranial germinoma in the lateral ventricle with polydipsia and polyuria: a case report and literature review.

Central nervous system germ cell tumors (CNSGCTs) are rare neoplasms which usually develop in the midline structures. They are occasionally involved in off-midline structures of the brain. Here, we report an extremely rare case of an intracranial germinoma in the lateral ventricle. The patient was a 10-year-old boy with a 1-year history of polydipsia and polyuria. Brain magnetic resonance imaging (MRI) showed a relatively homogeneously enhancing lesion in the lateral ventricle, and the posterior pituitary gland was not hyperintense on T1-weighted imaging. Subependymoma was suspected, and tumor removal operation was performed; however, because the intraoperative pathological investigation revealed germinoma, we could only perform partial removal of the tumor. Postoperative histology also confirmed germinoma. Then, the patient received chemotherapy, followed by radiation therapy. MRI showed no recurrence for 6 years after treatment. Intracranial germinoma in the lateral ventricle is extremely rare. The diagnosis is occasionally challenging, especially when the tumors are located in atypical locations. This paper presents a literature review of previously described CNSGCTs of the lateral ventricle to improve awareness of CNSGCTs in atypical locations. We also consider the relationship between imaging findings and clinical manifestations.

Pediatric pineal region masses: a single-center experience over 25 years.

PURPOSE: Pineal region tumors (PRT) represent less than 1% of brain neoplasms. The rare and heterogeneous nature of these tumors is reflected in the variety of treatment modalities employed. METHODS: A single-center retrospective review of all pediatric patients with pineal region tumors between November 1996 and June 2021 was performed. Fifty-six cases of pineal tumors were reviewed for age and symptoms upon presentation, diagnostic methods, imaging characteristics, histological classification, treatment modalities, recurrence, and mortality rates. RESULTS: The average age at diagnosis was 11.3 years. The majority of patients were male (82.1%) and Caucasian (73.2%). The most common presenting symptoms were headache (n = 38, 67.9%) and visual problems (n = 34, 60.7%). Hydrocephalus was present in 49 patients (87.5%). Germinoma (n = 20, 35.7%) and non-germinomatous germ cell tumor (NGGCT) (n = 17, 30.4%) were the most common tumors. Chemotherapy was employed for 54 patients (96.4%), radiation for 49 (87.5%), and surgical resection for 14 (25.0%). The average duration of treatment was 5.9 months. Progression-free survival was 74.4% at 5 years and 72.0% at 10 years. Overall survival was 85.7% at 5 years and 77.1% at 10 years. CONCLUSION: Treatment of pineal region tumors must be targeted to each patient based on presentation, subtype, presence of hydrocephalus, and extent of disease. Upfront surgical resection is usually not indicated. As advances in oncological care proceed, treatment modalities may continue to improve in efficacy.

Diencephalic syndrome in a female child due to intracranial germinoma: a case report.

INTRODUCTION: Diencephalic syndrome (DS) is a rare syndrome with failure to thrive (FTT) as the primary manifestation, which is often associated with astrocytoma or glioma and rarely caused by germinoma. To our knowledge, there are no reports of female patients presenting with DS secondary to germinoma. CASE REPORT: we report a case (an 11-year-old girl) of diencephalic syndrome presenting with FTT. She was diagnosed with severe malnutrition in the local hospital two years before admission and still did not show normal development after long-term nutritional support. Finally, after ruling out increased metabolism, inadequate caloric intake, and nutrient absorption, intracranial MRI showed a space-occupying lesion in the suprasellar cisterna-hypothalamus area. After excluding other causes of FTT, a biopsy was performed for pathological examination and demonstrated a germinoma. An excellent therapeutic effect was achieved during the three-month follow-up after radiotherapy. CONCLUSION: This case reminds us that intracranial tumors should be considered an indispensable etiology for patients with suspicious FTT, and early diagnosis and intervention may achieve a good prognosis.

Diagnostic radiation-induced regression of a metastatic primary intracranial germinoma: a case report.

Pineal region germinomas are sensitive to radiotherapy. Standard neurosurgical management involves obtaining a tissue biopsy and to relieve the often accompanying obstructive hydrocephalus. We present a patient with a suspected hyper-radiosensitive metastatic primary intracranial germinoma where computed tomography scanning resulted in tumor regression before radiotherapy could be administered.

Unusual presentation of epilepsy of germinoma located in the frontal-lobe: case report and literature review.

Germinoma is rare in peripheral lobar locations in the brain, with only 10 cases of primary frontal lobe germinoma having been reported in the previous literature. Epilepsy is a rare manifestation of germinomas. We describe an unusual case of a primary frontal germinoma in a 21-year-old man who presented with epilepsy. A presumptive diagnosis of abscess or cystic glioma was made, and then, we performed microsurgery under magnetic resonance imaging (MRI) neuronavigation guidance. Postoperative histopathologic examination identified the tumour as a rare germinoma. Subsequently, adjuvant radiotherapy and chemotherapy programmes were adopted in the present case, and there were no recurrence and postoperative seizure symptoms observed in the follow-up 6 months after operation.

A GIANT GERMINOMA MIMICS HIGH-GRADE GLIOMA: A RARE FORM OF THALAMIC REGION TUMOR.

A fourteen-year-old boy initially presented with weakness in the right extremity, worsening in the last three months with stiffness and convulsions in his right extremity. Magnetic resonance imaging of the brain revealed an intra-axial tumor measuring 8.3x7.3x6.8 cm, leading to obstructive hydrocephalus. The patient's condition suddenly worsened with decreased consciousness, and then emergency surgery was performed for tumor resection and external ventricular drainage before switching to a ventriculoperitoneal shunt on the fifth day after surgery. Histopathological examination revealed a germinoma, which is rare in the thalamic region. The patient responded well to radiation therapy after surgery. Ectopic GCT may be difficult to differentiate on radiological examination alone. The current case was initially diagnosed as a high-grade glioma based on radiological findings. A definite diagnosis can be made only after a histopathological examination, which requires a tissue sample. Therefore, many tumors are surgically excised for biopsy purposes A good preoperative examination is very important to determine the approach to patient management. Furthermore, radiotherapy is mandatory for germinoma because of its radiosensitivity.

The clinical and imaging features of infratentorial germinomas compared with supratentorial ectopic germinomas.

BACKGROUND: Intracranial germinoma is very sensitive to chemoradiotherapy, while the risk of infratentorial operation is high. The accurate diagnosis can provide the more reasonable treatment, avoiding the unnecessary therapeutic risks. PURPOSE: To evaluate the clinical and imaging features of infratentorial germinomas. MATERIAL AND METHODS: The clinical and imaging data of 13 infratentorial germinomas were collected and compared with 17 supratentorial germinomas in the same period. The clinical and imaging findings were retrospectively analyzed. RESULTS: Infratentorial germinomas were more common in female patients than supratentorial ones (53.85% vs. 11.76%, P = 0.020). The mean age of the infratentorial group (23.0 ± 10.2 years) was significantly older than that of supratentorial group (12.4 ± 3.3 years, P = 0.003). Most infratentorial germinomas (12/13, 92.31%) underwent surgical resection, while stereotactic biopsy was more common in the supratentorial group (11/17, 64.71%, P = 0.002). Infratentorial germinomas were significantly smaller than supratentorial ones (25.85 ± 8.13 mm vs. 37.18 ± 18.11 mm, P = 0.031). Cystic lesions were more common in supratentorial germinomas (12/17, 70.59%), while most infratentorial germinomas were solid lesions (10/13, 76.92%, P = 0.025). On post-contrast T1-weighted imaging, obvious enhancement was more common in infratentorial germinomas than in supratentorial ones (100% vs. 64.71%, P = 0.024). CONCLUSION: In addition to the common findings with supratentorial germinomas, infratentorial lesions have some specific clinical and imaging features.

[Lesion of optic nerves and chiasm following bifocal germinoma recurrence]. / Porazhenie zritel'nykh nervov i khiazmy kak proyavlenie retsidiva bifokal'noi germinomy.

The authors report a patient with recurrent bifocal germinoma of the optical nerves and chiasm after previous combined treatment. The tumor resulted progressive visual acuity loss despite subsequent therapy (glucocorticoid therapy, chemo- and radiotherapy). Differential diagnosis between tumor progression and consequences of radiotherapy was complicated by MRI negative pattern. Subsequent development of the process, signs of anterior visual pathway damage and tumor spread throughout the ventricular system and subarachnoid spaces according to neuroimaging data indicated recurrence of disease.

Intracranial Pure Germinoma With Optic Nerve Infiltration.

A 19-year-old man presented with a 3-year history of episodic headaches, right hemiparesis, and progressive vision loss in both eyes. Initially, extensive laboratory testing was unrevealing. MRI later demonstrated progressive enlargement and enhancement of the left optic nerve poorly correlated with the timing of his clinical manifestations. There was no clinical or radiological response to treatment with corticosteroids, mycophenolate mofetil, or rituximab administered empirically for possible inflammatory processes. Later in the disease course, he developed diabetes insipidus (DI), worsening vision to light perception bilaterally, severe cognitive decline, and spastic quadriparesis. Cerebrospinal fluid (CSF) beta human chorionic gonadotropin (ß-hCG) was elevated. Eventually, a left optic nerve biopsy was performed, which was consistent with an intracranial pure germinoma with infiltration of the optic nerve and disseminated leptomeningeal disease. Although rare, intracranial germ cell tumors can primarily involve the anterior visual pathways and should be considered in the setting of DI and elevated CSF ß-hCG.

A Rare Case of Intra-Fourth-Ventricular Germinoma, Derived from the Medulla Oblongata.

INTRODUCTION: Extragonadal germinomas rarely emerge from the brain stem; however, proper diagnosis and treatment can result in favorable prognosis. Unfortunately, the preoperative diagnosis of medulla oblongata germinoma is difficult due to insufficient clinical signs and symptoms that are specific to this diagnosis. Case Representation: We present a 12-year-old male patient with an intra-fourth-ventricular germinoma, derived from the medulla oblongata, with no abnormalities in the supratentorial region. The germinoma was initially assessed by advance MRI sequences, including diffusion-weighted imaging, T1 perfusion, and spectroscopy. CONCLUSION: In summary, although existing imaging technologies cannot completely distinguish germinomas from other primary brain neoplasms in the fourth ventricle, in patients aged between 12 and 40 years, a small mass on the dorsal side of medulla oblongata that emerges into the fourth ventricle and is characterized by homogeneous contrast enhancement, the absence of calcification and hemorrhage, and the lack of hydrocephalus should be considered for a potential medulla oblongata germinoma diagnosis.

Primary cavernous sinus germinoma with atypical extension pattern: a case report and review of the literature.

Primary intracranial germinoma is a rare central nervous system tumor that usually arises in the pineal and the supra-sellar region. Here, we report a rare case of primary intracavernous sinus germinoma with an atypical extension pattern, with a comparison to germinomas originating from the cavernous sinus as described in the existing literature. A 12-year-old boy was admitted to our hospital with the chief complaint of the left-side ptosis and double vision. Magnetic resonance imaging showed homogenous enhanced mass lesion in the pineal region together with mass lesions in the lateral ventricle, left cavernous sinus, and temporal lobe, extending into the left masticator space. The enhanced mass in the intracavernous sinus originated from the cavernous sinus. Endoscopic third ventriculostomy and tumor biopsy was done. Pathological diagnosis was pure germinoma. After six courses of chemotherapy followed by radiation therapy, all the lesions decreased in size significantly. Only faint enhancement around the masticator space remained. We report a rare case of a germinoma that developed mainly in the cavernous sinus with additional tumor masses in the pineal region, ventricles, and temporal lobe. Although the lesions shrank significantly on the post-chemoradiation imaging, a long follow-up is necessary not only to check for symptoms, but also monitor imaging findings for possible serial changes in the residual region of the masticator space.

Imaging Features Predictive of Recurrence in Pediatric Intracranial Germ-Cell Tumors.

BACKGROUND/AIMS: Intracranial germ-cell tumors (GCTs) are a heterogeneous group of tumors that vary in their response to treatment. Standard treatment consists of chemotherapy and radiation therapy, with the consideration of second-look surgery in resistant disease. The present study aims to inform therapy by characterizing features on pretreatment imaging associated with recurrence. METHODS: Children with intracranial GCTs treated at a single institution between January 2000 and October 2016 were retrospectively reviewed under an Institutional Review Board-approved protocol. Imaging variables identified on pretreatment imaging were calcifications, cysts, heterogeneity of enhancement, blood products, hydrocephalus, gradient echo susceptibility, restricted diffusion, invasiveness, and extent of edema. Tumor recurrence was used as the primary outcome variable. RESULTS AND CONCLUSION: Fifty-two patients (39 males, mean age at diagnosis: 13 ± 5 years, 34 germinoma, 18 nongerminomatous GCT [NGGCT]) were reviewed. Thirty-three percent of the patients reviewed had recurrence (7 germinoma, 11 NGGCT). Recurrence was associated with invasiveness as seen on preoperative imaging (p = 0.0385) and cystic tumor (p = 0.048).

T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study.

PURPOSE: The role of T2*-based MR imaging in intracranial germ cell tumors (GCTs) has not been fully elucidated. The aim of this study was to evaluate the susceptibility-weighted imaging (SWI) or T2* gradient echo (GRE) features of germinomas and non-germinomatous germ cell tumors (NGGCTs) in midline and off-midline locations. METHODS: We retrospectively evaluated all consecutive pediatric patients referred to our institution between 2005 and 2016, for newly diagnosed, treatment-naïve intracranial GCT, who underwent MRI, including T2*-based MR imaging (T2* GRE sequences or SWI). Standard pre- and post-contrast T1- and T2-weighted imaging characteristics along with T2*-based MR imaging features of all lesions were evaluated. Diagnosis was performed in accordance with the SIOP CNS GCT protocol criteria. RESULTS: Twenty-four subjects met the inclusion criteria (17 males and 7 females). There were 17 patients with germinomas, including 5 basal ganglia primaries, and 7 patients with secreting NGGCT. All off-midline germinomas presented with SWI or GRE hypointensity; among midline GCT, all NGGCTs showed SWI or GRE hypointensity whereas all but one pure germinoma were isointense or hyperintense to normal parenchyma. A significant difference emerged on T2*-based MR imaging among midline germinomas, NGGCTs, and off-midline germinomas (p < 0.001). CONCLUSION: Assessment of the SWI or GRE characteristics of intracranial GCT may potentially assist in differentiating pure germinomas from NGGCT and in the characterization of basal ganglia involvement. T2*-based MR imaging is recommended in case of suspected intracranial GCT.

Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism.

A young woman presented with primary amenorrhoea, progressive haemiparesis, visual disturbance, dementia and focal motor seizures. Investigations showed hypopituitarism, unilateral cerebral atrophy and inflamed cerebrospinal fluid. A trans-sphenoidal biopsy gave a unifying diagnosis of a pituitary germinoma.

Visual complaints in intracranial germinomas.

OBJECTIVE: Patients with brain tumors often report having visual complaints. This may be due to increased intracranial pressure, compression/invasion of the optic pathway or diplopia. We assessed the incidence and the etiology of visual symptoms in patients with intracranial germinoma tumors (ICGTs). METHODS AND MATERIALS: We performed a blinded retrospective review of the clinical charts and the initial magnetic resonance imaging (MRI) of 28 patients with ICGT. Thirteen tumors were pineal, five suprasellar, seven bifocal, and further three involved either the optic nerve, the corpus callosum, or the brainstem. RESULTS: Twelve patients reported visual disturbances, seven of whom mainly experienced a decrease in vision. Two of those were initially managed as "retrobulbar neuritis" when endocrinologic symptoms prompted assessment by MRI. Involvement of the optic pathway was underestimated, and both relapsed. Field deficits were definitive sequelae, whereas visual acuity was sometimes regressive in the absence of optic atrophy. CONCLUSIONS: Compression or invasion of the optic pathway by germinomas is not a rare occurrence, and this possibility should not be overlooked when thickening or contrast enhancement is detected. Radiotherapy fields should be extended accordingly.

Efficacy of Ifosfamide-Cisplatin-Etoposide (ICE) Chemotherapy for a CNS Germinoma in a Child With Down Syndrome.

Intracranial germ cell tumor is sometimes associated with Down syndrome; however, no optimal treatment has been developed due to the high risk of recurrence and treatment-related mortality. Here, we report on a patient with an intracranial germinoma in the bilateral basal ganglia. The patient received 3 courses of ifosfamide-cisplatin-etoposide in combination with whole-brain irradiation (24 Gy), with no serious complications. The patient is alive and disease free 16 months after the initial diagnosis. This regimen is a feasible treatment for intracranial germ cell tumor associated with Down syndrome, although careful attention must be paid to the increased risk for severe infection.

Mixed pineal mature teratoma and germinoma in two brothers of the fraternal triplets.

INTRODUCTION: Intracranial teratomas are rare germ cell neoplasms that contain tissues derived from all three germ cell layers and most commonly occurring during childhood. This is the first report of pineal region mixed mature teratoma and germinoma in two fraternal brothers of fraternal triplets. CASE PRESENTATION: We report the case of a mixed mature teratoma and germinoma of the pineal region in two brothers of fraternal triplets. Older brother was initially diagnosed at the age of 11 years with the pure teratoma of the pineal region but the review of the pathology 3 years after initial surgery revealed the mixed mature teratoma with 5% germinomatous component. The younger brother was diagnosed at the age of 13 years with the mixed mature teratoma with 10% germinomatous component tumor of the pineal region. Younger brother has been treated with adjuvant chemo-radiotherapy and older brother was treated without adjuvant therapy. Both brothers had no recurrence. CONCLUSION: Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor.