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Cancer Control : Journal of the Moffitt... 2022The correlations of epidemiological characteristics and clinical outcomes with different tumor sites in patients with intracranial typical site germinomas (ICTSGs) have...
Epidemiology, Management, and Long-Term Survival Outcomes of Intracranial Typical Site Germinomas: An Analysis of the Surveillance, Epidemiology, and End-Results (SEER) Database.
BACKGROUND
The 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.
METHODS
Patients 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.
RESULTS
Among 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%; < .001). Smaller tumors (<24 mm) were more common in the suprasellar region (37.74 vs 18.87%; < .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.
CONCLUSIONS
Despite significant differences in epidemiology and management, pineal and suprasellar germinomas had a similar long-term clinical outcome.
Topics: Brain Neoplasms; Germinoma; Humans; Male; Pineal Gland; Skull Base Neoplasms
PubMed: 35446714
DOI: 10.1177/10732748221095944 -
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 -
Neuro-oncology Jun 2022
Topics: Brain Neoplasms; Child; Germinoma; Humans; Pineal Gland
PubMed: 35171291
DOI: 10.1093/neuonc/noac047 -
Frontiers in Endocrinology 2021Intracranial germ cell tumors (GCTs) are relatively rare, which account for 0.5% of all primary intracranial neoplasms. Intracranial germinomas most commonly occur in... (Review)
Review
Intracranial germ cell tumors (GCTs) are relatively rare, which account for 0.5% of all primary intracranial neoplasms. Intracranial germinomas most commonly occur in the pineal and suprasellar region, making up the majority of all intracranial GCTs. For its diversified clinical manifestations, the diagnosis is easily confused with other diseases. Here, we present a case of a 19-year-old boy with intracranial germinoma who was preliminarily misdiagnosed as hyperthyroidism for the symptoms of weight loss and thyroid dysfunction.
Topics: Adrenal Insufficiency; Brain Neoplasms; Diagnostic Errors; Germinoma; Humans; Hyperprolactinemia; Hyperthyroidism; Hypogonadism; Magnetic Resonance Imaging; Male; Thyrotropin; Thyroxine; Triiodothyronine; Young Adult
PubMed: 35140682
DOI: 10.3389/fendo.2021.789109 -
Surgical Neurology International 2022Pineal tumors are uncommon tumors that affect <1% of adults, with 50% of them being germinomas. A combination of endoscopic third ventriculostomy (ETV) and tumor biopsy...
BACKGROUND
Pineal tumors are uncommon tumors that affect <1% of adults, with 50% of them being germinomas. A combination of endoscopic third ventriculostomy (ETV) and tumor biopsy is usually used. Cerebral vasospasm in association with aneurysmal subarachnoid hemorrhage (aSAH) has been extensively studied. However, at least according to the research, this is not the case in intraventricular hemorrhage (IVH) cases. We present a case with two distinct findings: (1) an unexpected large IVH following the removal of an external ventricular drain (EVD) in a patient who had undergone ETV and tumor biopsy, resulting in severe clinical vasospasm and (2) incidental pineal region germinoma regression on follow-up magnetic resonance imaging (MRI) without any prior adjuvant chemoradiation to explain such regression.
CASE DESCRIPTION
The authors describe an 18-year-old male patient who had a routine, uneventful combined ETV and tumor biopsy, as well as the placement of an EVD. Histopathological examination revealed germinoma. His postoperative course was complicated by IVH after EVD removal, which resulted in clinical vasospasm. Without any prior adjuvant chemoradiation, follow-up MRI of the b rain revealed a significant reduction in the size of the germinoma as well as reconstitution of the patency of the previously obstructed aqueduct of Sylvius.
CONCLUSION
The take-home message from this case is that in the case of postoperative clinical deterioration in a patient with concurrent IVH and ETV, a high index of suspicion for vasospasm is required, as this may allow a significant amount of blood to pass down to the basal cisterns. Early detection and management of clinical vasospasm are critical for a better neurological outcome. Furthermore, unexpected tumor size changes can occur due to a variety of factors, so recent preoperative MRI of the brain should be obtained in the lead-up to surgery, and postoperative computed tomography should be used sparingly to avoid radiation-related tumor changes.
PubMed: 35127229
DOI: 10.25259/SNI_944_2021 -
NMC Case Report Journal 2021Although the synchronous occurrence of testicular seminoma and systemic sarcoidosis has been reported, that of intracranial germinoma and systemic sarcoidosis is...
Although the synchronous occurrence of testicular seminoma and systemic sarcoidosis has been reported, that of intracranial germinoma and systemic sarcoidosis is unknown. A 26-year-old man presented with symptoms of panhypopituitarism and consciousness disturbance. Imaging demonstrated a large nodule in the upper right lung field and swelling of multiple bilateral pulmonary and mediastinal lymph nodes in addition to the bifocal pineal and suprasellar tumors with obstructive hydrocephalus. The pathological diagnosis of the intracranial bifocal tumors was pure germinoma, whereas that of the mediastinal lymph nodes was epithelioid granuloma. Three courses of chemotherapy using carboplatin and etoposide were administered, followed by whole ventricle irradiation. The intracranial tumors completely disappeared, but the lung nodule and mediastinal lymph nodes progressed. Whole-body fluorine-18-fluorodeoxyglucose positron emission tomography demonstrated accumulation in the mediastinal lymphadenopathy, lung masses, and multiple lymph nodes of the whole body. Transbronchial lung biopsy revealed epithelioid granuloma with multinucleated giant cells. In conjunction with the high blood concentration of angiotensin-converting enzyme and soluble interleukin-2 receptor, these findings established a diagnosis of sarcoidosis. This is the first report of synchronous occurrence of intracranial germinoma and sarcoidosis. Such coexistence is extremely rare, but we should mind that sarcoidosis can occur with intracranial germinoma.
PubMed: 35079529
DOI: 10.2176/nmccrj.cr.2021-0078 -
Surgical Neurology International 2021Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have...
BACKGROUND
Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have been reported, but the clinical course was poor; there was no case of survival for more than 2 years.
CASE DESCRIPTION
We report the case of a 12-year-old boy with DMG in the pineal region who is living a normal daily life for more than 6 years following multimodal treatment. He complained of a headache accompanied by vomiting that had gradually worsened 1 month previously, and initial magnetic resonance imaging revealed a pineal tumor. Germinoma was initially suspected; however, a combination of chemotherapy using carboplatin and etoposide was ineffective. The first surgery was performed through the left occipital transtentorial approach (OTA); the diagnosis was DMG. After 60 Gy radiotherapy concomitant with temozolomide (TMZ), the tumor enlarged. Second surgery was performed through bilateral OTAs, and 90% of the tumor was removed. In addition, stereotactic radiotherapy (30 Gy, six fractions) was administered, and the local equivalent dose in 2 Gy/fraction reached 97.5 Gy. Maintenance chemotherapy using TMZ and bevacizumab was continued for 2 years. After finishing chemotherapy, the enhancing lesion enlarged again, and bevacizumab monotherapy was effective. Now, at 6 years after diagnosis, the patient leads an ordinary life as a student.
CONCLUSION
Maximum resection and high-dose radiotherapy followed by bevacizumab may have been effective in the present case.
PubMed: 34992928
DOI: 10.25259/SNI_1141_2021 -
Neuro-oncology Aug 2022This multinational study was conducted to report clinical presentations and treatment strategies in patients with intracranial germinomas across selected Asian centers,...
BACKGROUND
This multinational study was conducted to report clinical presentations and treatment strategies in patients with intracranial germinomas across selected Asian centers, including failure patterns, risk factors, and outcomes.
METHODS
A retrospective data collection and analysis of these patients, treated between 1995 and 2015 from eight healthcare institutions across four countries was undertaken.
RESULTS
From the results, 418 patients were analyzed, with a median follow-up of 8.9 years; 79.9% of the patients were M0, and 87.6% had β-human chorionic gonadotropin values <50 mIU/mL. The 5/10-year overall survival (OS) and recurrence-free survival (RFS) rates were 97.2%/96.2% and 89.9%/86.9%, respectively. RFS was predicted by the radiotherapy (RT) field, with focal RT having the worst outcome, whereas chemotherapy usage had no impact on survival. Among patients who received chemotherapy, response to chemotherapy did not predict survival outcomes. In M0 patients, primary basal ganglia tumors predicted a worse RFS. In patients with bifocal tumors, an extended field RT was associated with better outcomes. In multivariable analysis, only RT fields were associated with RFS. In relapsed patients, salvage rates were high at 85.7%. Additionally, patients who received salvage RT had a better outcome (91.6% vs. 66.7%).
CONCLUSIONS
Survival outcomes of patients with germinoma were excellent. Thus, the focus of treatment for intracranial germinoma should be on survivorship. Further studies are warranted to find the optimal intensity and volume of radiation, including the role of chemotherapy in the survival of patients with intracranial germinomas, considering age, primary tumor location, and extent of disease.
Topics: Brain Neoplasms; Germinoma; Humans; Pineal Gland; Retrospective Studies; Salvage Therapy
PubMed: 34935949
DOI: 10.1093/neuonc/noab295 -
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 -
Cancer Reports (Hoboken, N.J.) Aug 2022Intracranial germ cell tumors (GCTs) comprise 3%-5% of pediatric primary central nervous system (CNS) tumors in Western countries. Though they are related in embryonic...
BACKGROUND
Intracranial germ cell tumors (GCTs) comprise 3%-5% of pediatric primary central nervous system (CNS) tumors in Western countries. Though they are related in embryonic origin to gonadal GCTs, which are considered highly treatable with cisplatin-based chemotherapy regimens, intracranial GCTs vary in malignant potential and sensitivity to radiation and chemotherapy, generally carrying a worse prognosis. Metastases of intracranial GCTs outside of the CNS are rare, indicate a poor prognosis, and their salvage treatment is not well established.
CASE
A 15-year-old boy presented with bifocal (suprasellar and pineal) intracranial nongerminomatous germ cell tumors of mixed origin. The tumors were treated to full response with a multimodal approach of neoadjuvant chemotherapy, surgical resection, and adjuvant craniospinal proton radiation. Nine months following treatment completion, the patient presented with an enlarged cervical lymph node determined on excisional biopsy to be a recurrence of pure germinoma from the primary tumors. Salvage treatment involved high-dose chemotherapy and autologous stem cell transplantation; however, the patient denied further treatment prior to planned focal radiotherapy. Thirty months post-treatment, the patient is well with no evidence of recurrence.
CONCLUSION
This case demonstrated the successful salvage treatment of an extraneural recurrence of an intracranial GCT using surgical resection and a high-dose chemotherapy and autologous stem-cell transplantation regimen, highlighting the unique factors which led to the selection of this regimen.
Topics: Adolescent; Brain Neoplasms; Child; Germinoma; Hematopoietic Stem Cell Transplantation; Humans; Lymph Nodes; Male; Neoplasms, Germ Cell and Embryonal; Transplantation, Autologous
PubMed: 34796700
DOI: 10.1002/cnr2.1586