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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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Stereotactic biopsies are carried out to obtain tumor tissue of unknown entity from cerebral lesions. Thus, tumor tissue can be examined, resulting in correct diagnosis and treatment. These procedures go in hand with high accuracy, high diagnostic yield, and low complication rates., Objectives: The aim of this study is to evaluate the results and complications of stereotactic biopsies carried out for lesions of the pineal region., Methods: We performed a retrospective analysis of our prospective database and included 14 patients who underwent stereotactic biopsy of a pineal lesion between 2006 and February 2016. The Leksell stereotactic frame was used in all patients, and entry and target points were calculated using the BrainLab stereotactic system. We evaluated histopathologic results and postoperative complications such as hydrocephalus and hemorrhage., Results: Histopathologic diagnosis was established in all patients. On postoperative computed tomography, 7 patients (50%) showed a circumscribed blood collection at the site of biopsy. Six patients (42.8%) required a cerebrospinal fluid drain preoperatively. Two patients needed external ventricular drain postoperatively (14.2%). One of these patients showed a small hemorrhage area that caused the hydrocephalus. The other patient showed only postoperative swelling. Preoperatively, 2 patients (14.2%) already had a ventriculoperitoneal (VP) shunt, whereas postoperatively, another 4 patients (28.5%) needed a VP shunt. Thus, of the 14 patients, 6 (42.8%) required a VP shunt., Conclusions: Because of their localization, pineal lesions may result in emergence of a hydrocephalus. Stereotactic biopsies in this area may increase this effect and, thus, even VP shunts are necessary in some patients., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Primary intracranial germ cell tumors (IGCTs) represent an uncommon category of neoplasms, and familial occurrence is rare. We present the first report of parent-child patients with pathologically confirmed pure germinomas., Case Report: A 36-year-old Japanese man presented with diabetes insipidus and hypopituitarism. Magnetic resonance imaging (MRI) revealed a mass lesion in the pituitary stalk, which was diagnosed as a pure germinoma by open craniotomy tumor biopsy. Seven years later, his 13-year-old son also presented with diabetes insipidus. MRI revealed mass lesions in the pituitary stalk and the pineal region. He underwent endoscopic tumor biopsy for the pineal lesion, which was diagnosed as a pure germinoma. Both the father and his son were treated with combined radiochemotherapeutic regimens and achieved complete remission after one to two cycles of chemotherapy., Conclusion: Although there have been three previous case reports of familial germinoma, all of these involved sibling pairs. The present report represents the first parent-child cases. This type of familial occurrence suggests the possibility that germline mutations may also be involved in the development of IGCTs.

Recent advances in diagnostic imaging and experience with germinomas may allow for the differentiation of central nervous system germinomas from other tumors based on clinical information, without histological verification. We retrospectively analyzed clinically diagnosed germinoma-like tumors of the pineal and/or suprasellar regions. This was done to evaluate the efficacy of our strategy of defining germinoma-compatible tumors based on good responses to initial chemotherapy. The responses to chemotherapy and survival of 34 consecutive patients with germinoma-like tumors who underwent initial treatment from July 2001 to October 2010 were analyzed. The minimum apparent diffusion coefficient (minADC) value and proton magnetic resonance spectroscopy (MRS) were evaluated in recent patients. Twelve patients with histologically verified germinomas and 18 with germinoma-compatible tumors showed early logarithmic decreases in tumor volume in response to initial chemotherapy, typical low minADC values and typical MRS characteristics, including increased choline/creatine ratios, decreased N-acetylasparate/creatine ratios, and large lipid peaks. These patients had good progression-free survival. The other four patients, with histologically verified non-germinomas, showed no response to chemotherapy, and one patient with a pineoblastoma showed a similar minADC value and MRS characteristics to those of patients with germinomas. The response to initial chemotherapy can be used to distinguish germinoma-compatible tumors from non-germinoma in patients with germinoma-like tumors of the pineal and/or suprasellar regions. The evaluation of minADC and proton MRS are useful for distinguishing germinomas from other tumors. However, a subset of non-germinomas may show similar characteristics to germinomas. The benefit of bypassing unnecessary surgical intervention can be achieved, at least in Asian populations with a high incidence of germinomas., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

The authors report a rare case involving the occurrence of metachronous pure germinomas long after treatment of a mixed germ cell tumor (GCT) categorized as having a poor prognosis. A neurohypophysial germinoma occurred 4 years and 6 months after the initial treatment of a mixed pineal GCT containing a yolk sac tumor and a germinoma. Furthermore, intramedullary germinomas occurred 21 years after the initial treatment of the mixed GCT and 15 years after the second treatment of the neurohypophysial germinoma. The neurohypophysial germinoma was not confirmed histopathologically, but the intramedullary germinoma was histopathologically diagnosed as a pure germinoma. Serum α-fetoprotein levels at the second neurohypophysial and third intramedullary occurrences of the germinomas were less than 10 ng/ml. Therefore, no yolk sac components seemed to be contained in the tumors. The second neurohypophysial and third intramedullary germinomas might be recurrences of the germinoma component of the pineal mixed GCT, which consisted of a yolk sac tumor and a germinoma. However, it seems very unlikely that only the germinoma, categorized in the good prognosis group, would be the only one to recur. Hence, it seems plausible that both the second and the third occurrences of pure germinoma were de novo metachronous GCTs arising after the pineal mixed GCT was cured. The authors' case indicates the possibility of multicentric GCTs in the CNS.

Background: Intracranial germinomas (IG) are rare and highly curable tumors. The incidence and optimal treatment of recurrences are not well defined., Case Report: A 34-year-old male was diagnosed with a late recurrence of an IG 14 years after the initial diagnosis and treatment. The diagnosis was complicated by the absence of tumor markers and delayed histological sampling of the lesion. Upon histological confirmation, the patient received 2 cycles of conventional chemotherapy, followed by 2 cycles of highdose chemotherapy and peripheral blood stem cell transplantation. The patient achieved a complete remission on magnetic resonance imaging scan. Consolidating radiation of the involved field was performed after termination of the chemotherapy., Conclusion: Limited information on the optimal management of late relapses of IG call for individualized therapeutic approaches. Platinum-based chemotherapy, followed by high-dose chemotherapy and consolidative radiation, appears to be feasible and effective in this situation., (© 2013 S. Karger GmbH, Freiburg.)

External beam radiotherapy has proven effective in managing intracranial germinoma. However, concerns regarding long-term neurocognitive and endocrine sequelae led to the addition of chemotherapy, to reduce radiation target volumes. There is a paucity of data on patterns of failure in patients treated with differing radiation field sizes. We review our experience at a tertiary children's hospital treating children with intracranial germinoma, using induction chemotherapy followed by radiation therapy to various treatment volumes (craniospinal irradiation, whole ventricular irradiation, whole brain radiation therapy, and focal radiotherapy). Ten patients with primary intracranial germinoma, treated from November 1995-March 2011, were included. The primary treatment involved platinum-based chemotherapy, followed by definitive radiotherapy. The median follow-up period was 4.3 years (range, 0.75-13.25 years). The 5-year overall survival for the entire group was estimated at 85.7%, and the 5-year disease-free survival was estimated at 75.0%. Two treatment failures occurred at 5 and 28 months, both in patients with single lesions in the pineal region treated with focal radiotherapy only. Based on the patterns of failure, our outcomes support the continued use of the whole ventricular field vs a focal field, even in patients with limited disease who demonstrate a complete response to neoadjuvant chemotherapy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Pineal tumors are relatively rare central nervous system lesions with a predilection for the pediatric population. For the vast majority of these lesions, surgical resection is a critical step in effective treatment. This article discusses current strategies for preoperative evaluation, operative management, and postoperative care of the pediatric patient with a newly diagnosed pineal region tumor., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Germinomas are gonadal neoplasms that rarely occur extragonadally in the midline structures of the human body. Newly diagnosed adult cases of pineal gland germinomas are very rare since most of the cases are diagnosed in the mid teens. The estimated incidence of this tumor in western countries is between 0.4-3.4%. Typically, this tumor is diagnosed by its characteristic radiological appearance alone, supported by tumor marker(s) or by stereotactic biopsy of the tumor. We are reporting a very unique case of pineal germinoma diagnosed in an adult at our institute by cerebrospinal fluid cytology with literature review. We analyzed case reports, literature reviews, and therapy and diagnostic articles about pineal germinoma in the English literature from 1983 to 2009 through the national library of medicine, Pubmed, and OVID search engines. We used key words "pineal germinoma," "pineal gland tumor," "CNS germinoma," and "extragonodal germinomas" to search for our articles.

Here we report a case in which an intracranial germinoma displayed spontaneous regression. An 11-year-old boy presented with polyuria and headache. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed tumors in the suprasellar and pineal regions, and obstructive hydrocephalus. As repeat MRI demonstrated shrinkage of these tumors, resection was deferred. The patient was discharged and followed up with serial MRI. The tumor continued to regress for three weeks; however, the patient was readmitted due to tumor regrowth. We performed endoscopic biopsy, and histopathologic diagnosis was germinoma. The patient underwent three courses of combined chemotherapy and radiotherapy, and complete response was achieved. Although the precise cause of the transient regression is unknown, racranial germinoma may occasionally unde rgo spontane ous regression.

A 20-year-old man presented with a rare case of germinoma with a large component of epithelioid cell granuloma manifesting as oscillopsia. Magnetic resonance imaging demonstrated a mass in the pineal region with homogeneous enhancement with gadolinium. Craniotomy was performed, ending in biopsy. The initial histological diagnosis was epithelioid cell granuloma, but systemic investigation detected no evidence of granulomatous disorder. A revised diagnosis of germinoma was based on positive immunohistochemical staining for placental alkaline phosphatase (PLAP) and c-kit. Histological diagnosis is sometimes incorrect if granulomatous reaction is dominant. Immunohistochemical staining for PLAP and c-kit should be performed if germinoma is clinically suspected.

Objective and Importance: We report the extremely rare occurrence of a second germ-cell tumor at a different site and with different histological types long after total resolution of a pineal germinoma., Clinical Presentation: A 21-year-old man who presented with headache and diplopia was admitted to our hospital. Neuroradiological studies revealed a tumor in the pineal region. The tumor was biopsied with endoscope, and third ventriculostomy was performed. Histologically, the tumor proved to be a germinoma. The patient received 3 cycles of combination chemotherapy consisting of carboplatin and etoposide with radiotherapy. The tumor was totally resolute. Twelve months later, he was readmitted with headache and diplopia. Neuroradiological studies showed a tumor in the right temporal lobe., Intervention: The second tumor was totally removed. Histologically, the tumor proved to be a mixed germ-cell tumor, which consisted a yolk-sac tumor and a germinoma. After the second course of chemotherapy, magnetic resonance image studies revealed no evidence of the tumor., Conclusion: The second tumor was considered to be a metachronous neoplasm rather than a recurrence of the original mixed germ-cell tumor, which consisted a yolk-sac tumor and a germinoma.

A 20-year-old man presented with diplopia. Neurological examination revealed mild skew deviation and upbeat nystagmus. Computed tomography showed a clover-shaped isodense mass in the pineal region with homogeneous enhancement. The lesion was isointense on both T(1)- and T(2)-weighted magnetic resonance (MR) imaging with homogeneous enhancement by gadolinium-diethylenetriaminepenta-acetic acid. Cerebral angiography showed no tumor staining. Serum and cerebrospinal fluid were negative for beta-human chorionic gonadotropin, alpha-fetoprotein, and placental alkaline phosphatase. Open biopsy was performed using a right occipital transtentorial approach. Histological examination revealed a tumor consisting of clusters of germinoma cells, but with prominent infiltration of lymphocytes, plasma cells, and macrophages, and proliferation of small vessels. The histological diagnosis was germinoma with granulomatous reaction. MR imaging showed complete disappearance of the tumor after chemoradiotherapy. Neurosurgeons should be aware of this rare tumor to avoid misdiagnosis as granulomatous inflammation.

A 21-year-old man with a 5-month history of diplopia caused by isolated vertical ocular misalignment had normal laboratory studies, including brain magnetic resonance imaging (MRI). Eight months after the onset of diplopia, he reported dry mouth, polydipsia, polyuria, and absent sweating. Examination now disclosed light-near dissociation of the pupillary responses, convergence-retraction nystagmus, and upgaze palsy. MRI revealed enhancing suprasellar and pretectal masses presumed to be germinomas. Two years after brain irradiation and systemic chemotherapy, no lesions are apparent on MRI and hypothalamic dysfunction has partially resolved. In a young patient with isolated vertical diplopia and normal brain imaging, one should consider an early pretectal syndrome and inquire after manifestations of hypothalamic dysfunction.

A 13-year-old male was diagnosed as having pineal germinoma in July 1998. Since then, he had been treated with tumor excision, radiation and chemotherapy. In June 2002, he was diagnosed as having the chronic phase of chronic myelogenous leukemia (CML), and following treatment with interferon-alpha, he achieved hematological complete remission. Although CML is rare in secondary leukemia, the present case seemed therapy-related CML because of its clinical course, as the CML occurred after the period with radiation and chemotherapy. This is the first case of secondary CML following therapy for intracranial tumors.

In patients with pineal tumors, clinical symptoms are due to direct compression of adjacent structures. The most common signs include increased intracranial pressure (80%) caused by obstruction of the sylvian aqueduct, and Parinaud syndrome (50%) caused by direct compression of the superior colliculi. Hearing loss is rare in patients with tumors in this location. The authors report on the case of a 12-year-old boy in whom a malignant pineal germ cell tumor was found together with the unusual occurrence of severe hearing loss due to direct bilateral compression of the inferior colliculi. This condition resolved completely after tumor regression.

Pineal region lesions consist of a wide variety of rare tumor types, including deep midline cysts, intrinsic pineal tumors, germ cell tumors and vascular lesions. Advances in microsurgical, endoscopic and stereotactic techniques have helped to lower morbidity and mortality in the care of patients harboring these lesions. Surgery can be the definitive treatment in cysts and benign lesions. This report summarizes the retrospective experience of the authors with 64 pineal region and associated lesions encountered in multiple institutions over the last 20 years. Histology was obtained in 53 out of 64 radiographically apparent lesions. Direct surgical biopsy of solid and vascular tumors in the pineal region enables precise histological assessment of mixed tumors. By avoiding sampling error, precise treatment can be planned. This series, along with previously published data, shows a much higher incidence of intrinsic pineal tumors, glial tumors and nongerminomatous germ cell tumors in series from North America and Europe than in those from Japan and Korea, where germinoma is much more common. We experienced an incidence of 20.4% germinoma out of 49 solid and vascular pineal tumors, while other authors have described incidences of 51.2 and 53.5%, respectively. The fact that histology is more diverse in Western populations leads to a need to have more representative sampling. Early surgical resection combined with diversion of cerebrospinal fluid is effective in the treatment of pineal lesions and seems to be superior to the alternative of treatment based on the diagnostic response to radiation and/or on tumor markers alone., (Copyright 2003 S. Karger AG, Basel)

Recurrence along an endoscope tract has been described after endoscopic biopsy and resection of malignant tumors arising in multiple organ systems. We describe a case of tract recurrence following the ventriculoscopic biopsy of a central nervous system tumor. A retrospective review of the patient's clinical course, radiology and pathology was undertaken to formulate a case report. A 14-year-old boy was treated with endoscopic third ventriculostomy and endoscopic biopsy of a pineal germinoma associated with hydrocephalus. Persistent hydrocephalus required repeat third ventriculostomy. The patient subsequently received focal external beam radiotherapy. Seventeen months after the first third ventriculostomy, a routine MRI revealed a 2-cm enhancing mass in the ventriculoscope tract. This was removed via a right frontal craniotomy. Again the pathology was germinoma. Serum and CSF markers were negative at initial presentation and at the time of recurrence. The potential for tract recurrence and CSF dissemination should be considered following the endoscopic biopsy of pineal germ cell tumors as this could affect the extent of radiotherapy and/or chemotherapy., (Copyright 2001 S. Karger AG, Basel)

We analyzed clinically 30 germinomas (22 pure germinomas and 8 germinomas with syncytiotrophoblastic giant cell (STGC) and also investigated the proliferative potential of 18 germinomas immunohistochemically using MIB-1 monoclonal antibody in formalin-fixed paraffin-embedded sections. The majority of patients responded favorably and completely to the treatment. Seven patients (four had germinomas with STGC and the other three had pure germinomas) suffered recurrence after a complete response to radiotherapy and/or chemotherapy. The patients with germinomas with STGC experienced recurrence significantly more often than those with pure germinomas (P < 0.01). Three of the patients died. The MIB-1 indexes of the germinomas ranged from 18% to 80%. The average MIB-1 index of pure germinomas is 58.5 +/- 17.3%, that of germinomas with STGC is 45.3 +/- 17.1%. There was no significant difference between the two groups. The MIB-1 indexes of 3 recurrent cases were 64.3%, 51.9%, and 20.8%, respectively. There were no significant differences in the MIB-1 indexes between nonrecurrent cases and recurrent ones.