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Southern Medical Journal Oct 2010Germinomas are gonadal neoplasms that rarely occur extragonadally in the midline structures of the human body. Newly diagnosed adult cases of pineal gland germinomas are... (Review)
Review
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.
Topics: Adult; Combined Modality Therapy; Germinoma; Humans; Magnetic Resonance Imaging; Male; Pinealoma; Pituitary Hormones; Prognosis; Tomography, X-Ray Computed
PubMed: 20818311
DOI: 10.1097/SMJ.0b013e3181ebeeff -
Journal of Cancer Research and... Jan 2023Germ cell tumor of the central nervous system (CNS) is an infrequent entity consisting of only 0.2%-1.7% of all primary CNS tumors. The pineal gland is the commonest... (Review)
Review
Germ cell tumor of the central nervous system (CNS) is an infrequent entity consisting of only 0.2%-1.7% of all primary CNS tumors. The pineal gland is the commonest location of CNS germinoma. Traditionally, radiotherapy alone has been used to treat localized pineal germinoma, which has delivered a very high cure rate. Spinal drop metastases from pineal germinoma can develop after a long time from diagnosis and primary treatment. Currently, craniospinal irradiation is the standard of care in metastatic pineal germinoma with spinal drop metastases along with systemic chemotherapy. Very few cases of pineal germinoma with spinal drop metastases have been published in the literature. We report a pineal gland germinoma case with spinal drop metastases in an 18-year-old boy and reviewed the published literature.
Topics: Male; Humans; Adolescent; Pineal Gland; Germinoma; Brain Neoplasms; Neoplasms, Germ Cell and Embryonal
PubMed: 38384076
DOI: 10.4103/jcrt.jcrt_657_22 -
Radiographics : a Review Publication of... Nov 2010
Topics: Germinoma; Humans; Magnetic Resonance Imaging; Pineal Gland; Pinealoma
PubMed: 21105213
DOI: 10.1148/rg.307105923 -
European Journal of Radiology Feb 2023To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region...
PURPOSE
To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region tumors.
METHOD
This retrospective study consisted of 72 patients with pathologically proven pineal region tumors between January 2010 and August 2020. Tumors were classified as germinomas (40), non-germinomatous germ cell tumors (11) (NGGCT), pineal parenchymal tumors (10) (PPT), and other types of tumors (11). Visual scale score, ADC values and SWI intratumoral susceptibility signal (ITSS) score were analyzed and compared to histopathology data.
RESULTS
The mean apparent diffusion coefficient (ADCmean) and minimum apparent diffusion coefficient (ADCmin) ratio of germinoma were significantly lower than NGGCT. ADCmean or ADCmin cut-off ratio of ≤ 1.48 or ≤ 1.32 allowed for discrimination between germinoma and NGGCT with sensitivity and specificity of 100 % and 63.6 %. An ADCmin cut-off ratio of ≥ 0.93 allowed for discrimination between germinoma and PPT with sensitivity and specificity of 60 % and 80.0 %. ADCmin cut-off ratio of ≤ 1.15 allowed for discrimination of germinoma from other types of tumors with sensitivity and specificity of 87.5 % and 54.5 %.
CONCLUSIONS
ADC ratio can differentiate germinoma from other types of pineal region tumors. Our initial results suggest that ITSS score was not significantly correlated with specific histology subtype.
Topics: Humans; Pinealoma; Retrospective Studies; Magnetic Resonance Imaging; Diffusion Magnetic Resonance Imaging; Germinoma; Neoplasms, Germ Cell and Embryonal; Cell Differentiation; Brain Neoplasms; Pineal Gland
PubMed: 36584565
DOI: 10.1016/j.ejrad.2022.110663 -
Advances in Experimental Medicine and... 2020A wide and heterogeneous variety of tumors develop from the pineal gland. Pineal parenchymal tumors, germ cell tumors, and glial tumors represent most of them. The... (Review)
Review
A wide and heterogeneous variety of tumors develop from the pineal gland. Pineal parenchymal tumors, germ cell tumors, and glial tumors represent most of them. The molecular profiles and tumor microenvironment play a key role in the development and progression of pineal gland tumors. Consequently, they represent important factors that may determine the efficacy of the different treatment modalities and the clinical outcome. Current literature is scarce regarding the microenvironment research of pineal gland tumors. Here, we review the cellular and molecular profile of the pineal gland tumor microenvironment.
Topics: Brain Neoplasms; Glioma; Humans; Pineal Gland; Pinealoma; Tumor Microenvironment
PubMed: 34185290
DOI: 10.1007/978-3-030-59038-3_8 -
Cancer Reports (Hoboken, N.J.) Sep 2022Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes...
BACKGROUND
Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes neoadjuvant chemotherapy (NC) followed by radiotherapy (RT) or RT at a higher dose and larger field. These recommendations are based on studies focused mostly on children; it is currently unclear whether this treatment is applicable to adults.
CASE
We present a case of a 23-year-old adult male with no underlying pathologies, drug allergies, or family history of cancer, who presented for medical evaluation with blurred vision, diplopia, forgetfulness, and weight loss starting 3-4 months before the evaluation. Clinical examination indicated Parinaud's Syndrome. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a pineal tumor with ependymal dissemination in both lateral ventricles, which was causing obstructive hydrocephalus. The patient had surgery consisting of ventriculostomy, Holter shunt insertion, cisternal ventricular intubation, and cisterna magna anastomosis to improve ventricular drainage. Pathology confirmed pineal germinoma. Cerebrospinal fluid cytology and MRI of the axis were negative. Four cycles of NC were given to the patient (carboplatin, etoposide, and ifosfamide), with reduced dosage. Once a partial volumetric response was confirmed, whole-ventricular radiotherapy (WVR) was initiated with a total tumor bed dose of 45 Gy over 25 sessions in 5 weeks. Optimum clinical results were observed, and no short-term (<90 day) radiation toxicity was observed. The patient was able to resume his normal activities soon after treatment. Follow-ups over 2 years post-surgery indicated continued control of the lesion and absence of symptoms except for mild diplopia.
CONCLUSION
Although this is a case report, these data suggest that a reduced NC course and WVR may effectively treat adult GN. This protocol likely decreases the risk of undesirable NC and RT secondary effects, while providing excellent local control; however, using a narrower RT field is not recommended.
Topics: Adult; Brain Neoplasms; Child; Diplopia; Germinoma; Humans; Male; Pineal Gland; Pinealoma; Young Adult
PubMed: 35347869
DOI: 10.1002/cnr2.1611 -
British Journal of Neurosurgery Apr 2022Intracranial germinomas are uncommon and constitute less than 1% of all intracranial tumors. They usually arise in the midline of the brain, most commonly in the pineal... (Review)
Review
BACKGROUND
Intracranial germinomas are uncommon and constitute less than 1% of all intracranial tumors. They usually arise in the midline of the brain, most commonly in the pineal region. Pineal germinomas tend to spread through the cerebrospinal fluid (CSF). However, pineal germinomas with fast-developing diffuse subarachnoid/leptomeningeal dissemination are extremely rare, especially in young children.
METHODS
The case of a 4-year-old boy with a pineal germinoma who died of diffuse subarachnoid/leptomeningeal dissemination 1 month after radiotherapy is reported. A PubMed search with specific key terms was used to review cases of pineal germinomas with metastasis.
RESULTS
The patient presented with a two-week history of worsening headache, visual disturbances and nonprojectile vomiting. Parinaud's sign was positive on physical examination. Head computed tomography (CT)/magnetic resonance imaging (MRI) revealed a lesion in the pineal region with eccentric calcification and obvious supratentorial hydrocephalus. Pineal germinoma was suspected. A ventriculoperitoneal (VP) shunt followed by focal radiotherapy ameliorated the headaches and visual disturbances. The patient was discharged home without further treatment due to financial difficulties. One month after discharge, he was readmitted due to worsening headache, vomiting and lethargy. MRI showed a decrease in the size of the pineal lesion but revealed a diffuse leptomeningeal enhancement including the sulcus, basal cistern, prepontine cistern, and supravermian cistern. The patient's condition deteriorated rapidly, and he died 26 hours after readmission. The characteristics of pineal germinomas with metastasis are reported based on a review of the literature.
CONCLUSIONS
Metastases in pineal germinomas predominately occur in adolescents or young adults, most commonly as spinal "drop metastases." Dissemination usually develops several years after the initial tumor diagnosis and has a relatively good clinical prognosis. However, fast widespread subarachnoid/leptomeningeal dissemination and sudden death may occur in a young child before salvage treatment, as in the presented case.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Germinoma; Headache; Humans; Magnetic Resonance Imaging; Male; Pineal Gland; Vomiting; Young Adult
PubMed: 30451003
DOI: 10.1080/02688697.2018.1520804 -
Journal of Clinical Immunology Nov 2014Interferon-γ receptor 1 (IFN-γR1) deficiency is one of the primary immunodeficiencies conferring Mendelian Susceptibility to Mycobacterial Disease (MSMD). Some cases... (Review)
Review
Interferon-γ receptor 1 (IFN-γR1) deficiency is one of the primary immunodeficiencies conferring Mendelian Susceptibility to Mycobacterial Disease (MSMD). Some cases of neoplasms have been recently reported in patients with MSMD, underlying the already known link between immunodeficiency and carcinogenesis. We report the first case of intracranial tumour, i.e. pineal germinoma, in a 11-year-old patient with complete IFN-γR1 deficiency. The first clinical presentation of the genetic immunodeficiency dates back to when the child was aged 2 y and 10 mo, when he presented a multi-focal osteomyelitis caused by Mycobacterium scrofulaceum. The diagnosis of IFN-γR1 deficiency (523delT/523delT in IFNGR1 gene) was subsequently made. The child responded to antibiotic therapy and remained in stable clinical condition until the age of 11 years, when he started complaining of frontal, chronic headache. MRI revealed a solid pineal region mass lesion measuring 20 × 29 × 36 mm. Histological findings revealed a diagnosis of pineal germinoma. The patient received chemotherapy followed by local whole ventricular irradiation with boost on pineal site, experiencing complete remission, and to date he is tumor-free at four years follow-up. Four other cases of tumors have been reported in patients affected by MSMD in our knowledge: a case of Kaposi sarcoma, a case of B-cell lymphoma, a case of cutaneous squamous cell carcinoma and a case of oesophageal squamous cell carcinoma. In conclusion, in patients with MSMD, not only the surveillance of infectious diseases, but also that of tumors is important.
Topics: Age of Onset; Antineoplastic Agents; Child; Germinoma; Humans; Immunologic Deficiency Syndromes; Male; Pineal Gland; Radiotherapy; Receptors, Interferon; Treatment Outcome; Interferon gamma Receptor
PubMed: 25216720
DOI: 10.1007/s10875-014-0098-0 -
Journal of Clinical Neuroscience :... Aug 2021Pineal germinoma is rare with high cure rates following craniospinal radiotherapy. Efforts to reduce the radiotherapy dose and field via combination with chemotherapy...
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.
Topics: Adolescent; Adult; Australia; Brain Neoplasms; Chemoradiotherapy; Child; Cohort Studies; Disease-Free Survival; Female; Germinoma; Humans; Male; Middle Aged; Pineal Gland; Queensland; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 34275531
DOI: 10.1016/j.jocn.2021.05.006 -
Neuro-oncology Jun 2022The study aimed to evaluate whether simplified chemotherapy followed by dose-reduced irradiation was effective for treating patients (ages 3-21 years) with localized...
BACKGROUND
The study aimed to evaluate whether simplified chemotherapy followed by dose-reduced irradiation was effective for treating patients (ages 3-21 years) with localized germinoma. The primary endpoint was 3-year progression-free survival (PFS) rate.
METHODS
Patients with a complete response to chemotherapy with carboplatin and etoposide received 18 Gy WVI + 12 Gy boost to the tumor bed. Patients with partial response proceeded to 24 Gy WVI + 12 Gy. Longitudinal cognitive functioning was evaluated prospectively on ALTE07C1 and was a primary study aim.
RESULTS
One hundred and fifty-one patients were enrolled; 137 were eligible. Among 90 evaluable patients, 74 were treated with 18 Gy and 16 with 24 Gy WVI. The study failed to demonstrate noninferiority of the 18 Gy WVI regimen compared to the design threshold of 95% 3-year PFS rate, where, per design, patients who could not be assessed for progression at 3 years were counted as failures. The Kaplan-Meier (KM)-based 3-year PFS estimates were 94.5 ± 2.7% and 93.75 ± 6.1% for the 18 Gy and 24 Gy WVI cohorts, respectively. Collectively, estimated mean IQ and attention/concentration were within normal range. A lower mean attention score was observed at 9 months for patients treated with 24 Gy. Acute effects in processing speed were observed in the 18 Gy cohort at 9 months which improved at 30-month assessment.
CONCLUSIONS
While a failure according to the prospective statistical noninferiority design, this study demonstrated high rates of chemotherapy responses, favorable KM-based PFS and OS estimates in the context of reduced irradiation doses and holds promise for lower long-term morbidities for patients with germinoma.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Carboplatin; Child; Child, Preschool; Etoposide; Germinoma; Humans; Pineal Gland; Prospective Studies; Young Adult
PubMed: 34850169
DOI: 10.1093/neuonc/noab270