Primary midbrain germinomas: Report of a rare case with an updated review of the literature.

Intracranial germinomas are most commonly extra-axial germ cell tumors that are predominantly found in the pineal and suprasellar regions. Primary intra-axial midbrain germinomas are extremely rare, with only eight reported cases. Here we present a 30-year-old man who presented with severe neurological deficits, with an MRI that showed a heterogeneously enhancing mass with ill-defined margins in the midbrain, and with surrounding vasogenic edema extending to the thalamus. The presumptive preoperative differential diagnosis included glial tumors and lymphoma. The patient underwent a right paramedian suboccipital craniotomy and biopsy obtained through the supracerebellar infratentorial transcollicular approach. The histopathological diagnosis was reported as pure germinoma. After patient discharge, he received chemotherapy with carboplatin and etoposide, followed by radiotherapy. Follow-up MRI at up to 26 months showed no contrast-enhancing lesions but a mild T2 FLAIR hyperintensity adjacent to the resection cavity. Differential diagnosis of midbrain lesions can be challenging and should include glial tumors, primary central nervous system lymphoma, germ cell tumors, and metastasis. Accurate diagnosis requires adequate tissue sampling. In this report, we present a very rare case of a primary intra-axial germinoma of the midbrain which is biopsied via a transcollicular approach. This report is also unique as it provides the first surgical video of an open biopsy and the microscopic appearance of an intra-axial primary midbrain germinoma via a transcollicular approach.

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.

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.

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.

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.

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.

Endoscopic Ipsilateral Interhemispheric Approach for Resection of Selected Deep Medial Brain Tumors.

BACKGROUND: The interhemispheric fissure provides a natural surgical corridor to access tumors of the deep medial surface of the brain. Conventional microscopic approaches to these tumors are limited by the narrow width of the interhemispheric fissure and need for retraction of brain tissue or traversing overlying cortex. Over the last decade, the endoscope has been used to improve visualization of the operative field in neurosurgery, with benefits in terms of surgical ergonomics and extent of tumor resections. In the context of the interhemispheric fissure, an endoscopic approach may improve visualization of some tumors by providing a brighter, more divergent light source at depth and by enabling the operator to inspect around curved structures (e.g., corpus callosum). CASE DESCRIPTION: In this report, we present a series of 5 cases with tumors at various locations along the anteroposterior extent of the interhemispheric fissure that were resected using an endoscopic ipsilateral interhemispheric approach. CONCLUSIONS: The endoscopic ipsilateral interhemispheric approach is an effective and versatile approach to resection of selected deep medial brain tumors extending anteriorly from the genu of the corpus callosum to the splenium. It has notable advantages over the microscope and can be considered a useful adjunct in the surgeon's armamentarium.

Placental alkaline phosphatase in cerebrospinal fluid as a biomarker for optimizing surgical treatment strategies for pineal region germ cell tumors.

Pineal region germ cell tumors are a heterogenous group of tumors; of these, pure germinoma shows high sensitivity to adjuvant therapy, and the timing and sequence of surgical intervention and adjuvant/neoadjuvant therapy are important for devising a treatment strategy for intracranial germ cell tumors (IGCT). Biopsy is diagnostically useful, but is often insufficient because only a limited amount of specimen can be obtained. In the present study, we aimed to determine the value of cerebrospinal fluid placental alkaline phosphatase (PLAP) levels, reflecting the presence of germinoma, as a reliable indicator to determine treatment strategies for pineal germ cell tumors. To assess the relationship between elevated PLAP levels and the presence of germinoma, we retrospectively reviewed histopathological findings of 25 surgical cases of IGCT in the pineal region. The PLAP value reflects the existence of a germinoma component within a total tumor volume; consequently, tumor volume could be reduced in cases with elevated PLAP, while tumors negative for PLAP did not decrease in size. Therefore, PLAP levels may help neurosurgeons optimize surgical intervention timing for teratomas in the pineal region.

A Rare Case of a Pediatric Medullary Intracranial Germinoma.

BACKGROUND: Germinomas are rare intracranial lesions, with medulla germinomas being reported in only approximately 20 cases to date. These tumors are extremely sensitive to radiotherapy, and knowledge of them can allow for accurate diagnosis as well as significantly impact treatment and prognosis. CASE DESCRIPTION: We present a case of medullary germinoma and review the cases previously reported in the literature. CONCLUSIONS: A few imaging characteristics are noted in multiple cases, such as cystic components, enhancement, and absence of hydrocephalus. These features can provide clues to diagnosis of this rare entity.

The Purely Endoscopic Supracerebellar Infratentorial Approach for Resecting Pineal Region Tumors with Preservation of Cerebellomesencephalic Vein: Technical Note and Preliminary Clinical Outcomes.

BACKGROUND: The cerebellomesencephalic vein (CMV) was frequently sacrificed in surgery approached via the supracerebellar infratentorial (SCIT) route for resecting pineal region tumors, which resulted in potential risk of neurologic deficit. Preserving the CMV in the SCIT approach could enhance the safety and effectiveness of this natural corridor surgery. The aim of this article was to identify the probability and safety of preserving the CMV through the application of neuroendoscopy in the SCIT approach. METHODS: Clinical data of patients who underwent pineal region tumor resection through a purely endoscopic SCIT approach were retrospectively analyzed, focusing on surgical techniques and clinical outcomes. RESULTS: The study included 8 patients with pineal region tumors. The CMV was preserved intact in all patients. Total tumor removal was achieved in 7 of 8 patients. In 1 patient with 2 tumors in the pineal region and roof of the third ventricle, the tumor in the pineal region was resected completely, followed by subsequent chemotherapy combined with radiotherapy, after which the other tumor disappeared totally. All patients recovered normally with uneventful postoperative outcomes. CONCLUSIONS: The advantage of close observation and panoramic view provided by neuroendoscopy combined with meticulous manipulation improved the ability to preserve the CMV in resecting pineal region tumors via the SCIT approach. The neuroendoscopic technique enhances the safety and efficacy of the SCIT approach.

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.

Clinical case seminar: Familial intracranial germinoma.

BACKGROUND: Intracranial germinomas (ICG) are uncommon brain neoplasms with extremely rare familial occurance. Since ICG invades hypothalamus and/or pituitary, the endocrine dysfunction is one of the common determinants of these tumors. We presented two brothers with the history of ICG. Patient 1 is a 25-year-old male who had been suffering from the weakness of the right half of his body at the age of 18. Cranial MRI revealed mass lesion in the left thalamus. He underwent neurosurgery, tumor was removed completely. Histopathological (HP) and immunohistochemical analyses verified the diagnosis of pure germinoma. He experienced complete remission of the tumor after a radiation therapy. At the age of 22 the diagnosis of isolated growth hormone deficiency (IGHD) was established and GH replacement was initiated. Patient 2 is a 20-year old boy who was presented with diabetes insipidus at the age of 12. MRI detected tumor in the third ventricle and pineal region. After the endoscopic tumor biopsy the HP diagnosis was pure germinoma. He received chemotherapy followed by radiotherapy, and treated with GH during childhood. At the age of 18 GH replacement was reintroduced. A six month follow-up during the next two years in both brothers demonstrated the IGF1 normalization with no MRI signs of tumor recurrence. CONCLUSION: To the best of our knowledge so far, only six reports have been published related to familial ICG. The presented two brothers are the first report of familial ICG case outside of Japan. They are treated successfully with GH therapy in adult period. < /p > < p >.

Identification of characteristic features of pineal germinoma that enhance accuracy of preoperative differentiation in pineal region tumors: its significance on optimum surgical treatment.

The aim of the study is to identify characteristic features of pineal germinoma that enhance preoperative accuracy in differentiating germinoma from other pineal region tumors. Twenty-one consecutive patients with pineal region tumors were enrolled. In all patients, tumor resection was performed to verify the histology. Clinical records including upward gaze palsy of Parinaud's syndrome and neuroimaging were analyzed. In addition, we evaluated the relationship between magnetic resonance imaging (MRI) findings and tumor progression patterns in pineal germinoma. Among 21 patients, 15 patients were diagnosed with germ cell tumor, 4 with pineal parenchymal cell tumor, and 2 with meningioma. Upward gaze palsy was seen in 11 patients; nine had pure germinomas and two had mixed germ cell tumors. These tumors occupied the pineal region with extension to the area of the mesodiencephalic junction (MDJ) and the bi-epithalamic area between the bilateral pulvinar and the third ventricle. Tumor involvement of the former area could cause upward gaze palsy by insulting the rostral interstitial nucleus of the medial longitudinal fasciculus located in the MDJ area. Tumor invasion into the latter area is commonly seen as a cardioid-shaped tumor as the tumor image on the axial MRI view. Upward gaze palsy and a cardioid-shaped tumor image on the axial MRI views were demonstrated to be specific features of pineal pure germinoma. It is suggested that combination of both features may become useful tools to preoperatively differentiate germinoma from other pineal tumors, resulting in achievement of the optimum treatment of pineal region tumors.

Primary Intracranial Germinomas: Retrospective Analysis of Five Cases.

AIM: Primary intracranial germinomas (PIGs) are rare malignant brain tumors that represent approximately 0.2% to 1.7% of all primary intracranial tumors. PIGs have infrequent, but there is a possibility of spinal cord metastases. In this study, clinical outcomes of five consecutive PIGs have been presented. MATERIAL AND METHODS: Medical records were retrospectively reviewed in 1,849 cases of intracranial tumors who underwent surgery between the years 2005 and 2015 and cases confirmed as germinoma histopathologically were included in this study. RESULTS: Five cases of PIGs were detected in two female (40%) and 3 male (60%) patients. The mean age was 15.2±5.6 (8-23) years. The mean follow-up period was 52.3±56.4 (9-135) months. The most common complaint was headache (60%), followed by nausea±vomiting (40%). Four cases (80%) affected the suprasellar region while the fifth patient"s tumor was localized in the pineal region. The duration between the initial symptom and time of surgical intervention ranged between 15 days and 2 months. Twelve months after the first operation, one patient presented with drop seeding metastasis. Four-year survival (with exception of the case that died as a early surgical complication) was 100%. Gross total resection (GTR) was achieved in one patient. Surgical mortality rate was 20%. PIGs" morbidity rate was 60%. CONCLUSION: PIG is a mostly malignant tumor that generally affects the pediatric age group. They are radiosensitive tumors. Subtotal or near-total resection using stereotactic guide or direct surgery to confirm the histopathological diagnosis followed by chemotherapy and whole brain or in some cases craniospinal radiotherapy rather than GTR is therefore the treatment of choice.

Unusual presentation of plasmablastic lymphoma involving ovarian mature cystic teratoma: a case report.

BACKGROUND: Plasmablastic lymphoma (PBL) is relatively new clinical entity described as a distinct subtype of diffuse large B-cell lymphoma (DLBCL). It is characterized by its aggressive nature and proliferation of large neoplastic cells resembling immunoblasts including cells with more obvious plasmacytic differentiation. In this case report, we describe an unexpected finding of PBL associated with a mature cystic teratoma of the ovary in a young immune competent woman. CASE PRESENTATION: A 19-year old woman was admitted to the hospital with generalized lymphadenopathy, a pelvic tumor mass measuring 35 × 30 cm and a 4 cm lump in her right breast. She underwent a right salpingo-oophorectomy, lymphadenectomy, splenectomy, omentectomy, and a right breast lumpectomy. On macroscopic examination the right ovary was replaced by a thick-walled multilocular cystic tumor. Upon incision, the cysts were filled with thick, greasy sebaceous material and hair and there were several solid nodules within the cyst walls. Histological examination revealed a mature cystic teratoma and malignant non-Hodgkin lymphoma (NHL) within the solid nodules. Tumor tissue from the right breast, spleen and lymph nodes, all had the same histological, NHL morphology. After extensive immunostaining, a diagnosis of PBL was made. Following surgery, the patient was treated with different chemotherapy regimens, without any significant regression of the disease, and died of multiple organ failure. CONCLUSIONS: Primary NHL of the ovary is relatively rare occurrence while secondary involvement by lymphoma is much more common. PBL is a rare lymphoma, primarily reported in the jaw and oral mucosa, but also documented in extra-oral sites. To the best of our knowledge, this is the first case described in a mature ovarian cystic teratoma. Although the patient was HIV-negative and immune competent, she had progressive disease and died despite aggressive chemotherapy 11 months after the initial diagnosis.

Results of Combined Intraventricular Neuroendoscopic Procedures in 130 Cases with Special Focus on Fornix Contusions.

OBJECTIVE: Increasing experience with intraventricular neuroendoscopic procedures shows good results in the combination of endoscopic third ventriculostomy (ETV) and tumor biopsy. Other possible combinations are mainly presented in subgroups in the literature. Here, we present our experience with combined intraventricular procedures within 1 setting over the last 2 decades. METHODS: This study retrospectively analyzes data from neuroendoscopic intraventricular procedures between 1993 and 2015 in 3 different departments of neurosurgery. Inclusion criteria were a combination of at least 2 intraventricular endoscopic procedures (e.g. third ventriculostomy, cyst fenestration, tumor surgery or aqueductoplasty) within 1 setting. RESULTS: One-hundred and thirty cases with more than 300 procedures fulfilled the inclusion criteria. The most frequent combinations were ETV and tumor biopsy (n = 36), ETV and aqueductoplasty/stenting (n = 30), and ETV and cyst fenestration (n = 18). The complication rate was 16.9% with an overall morbidity of 1.6% and mortality of 0.8%. Fornix contusion was one of the most frequent intraoperative complications (16.4%). Shunt independency was achieved in 82.9% of cases with hydrocephalic symptoms. CONCLUSIONS: A combination of different intraventricular endoscopic procedures is safe and reliable, bearing similar risks of morbidities and mortality to single neuroendoscopic procedures. This study is one of the largest series in the literature and has similar low complication rates to others. Fornix contusion is the most frequent intraoperative complication in these patients. However, obvious clinical correlation is rare.