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Child's Nervous System : ChNS :... Sep 2023This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution...
PURPOSE
This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period.
MATERIAL AND METHODS
Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types.
RESULTS
Germinoma was found in 33.1%, with an overall 60-month survival rate of 88%; the female sex was the only factor of a worse prognosis. Non-germinomatous germ cell tumors were found in 27.1%, with an overall 60-month survival rate of 67.2%; metastasis upon diagnosis, residual tumor, and the absence of radiotherapy were associated with a worse diagnosis. Pineoblastoma was found in 22.5%, with an overall 60-month survival rate of 40.7%; the male sex was the only factor of a worse prognosis; a tendency toward a worse outcome was found in patients < 3 years of age and those with metastasis upon diagnosis. Glioma was identified in 12.5%, with an overall 60-month survival rate of 72.6%; high-grade gliomas were associated with a worse prognosis. Atypical teratoid rhabdoid tumors was found in 3.3%, and all patients died within a 19-month period.
CONCLUSION
Pineal region tumors are characterized by the heterogeneity of histological types, which exert an influence on the outcome. Knowledge of the prognostic factors for each histological types is of extreme importance to the determination of guided multidisciplinary treatment.
Topics: Child; Humans; Male; Female; Pinealoma; Prognosis; Pineal Gland; Brain Neoplasms; Glioma
PubMed: 37202535
DOI: 10.1007/s00381-023-05952-x -
JMA Journal Apr 2023Intracranial germ cell tumors are uncommon brain tumors; germinoma is the most common tumor in children and young adults, and the most common regions affected are pineal...
Intracranial germ cell tumors are uncommon brain tumors; germinoma is the most common tumor in children and young adults, and the most common regions affected are pineal gland and suprasellar region. Germinomas of the suprasellar region are accompanied by endocrine alterations, with adipsia being a rare presentation. Here, we present the case of a patient with an extensive intracranial germinoma whose initial presentation was adipsia, without any other endocrinological alteration, with development of severe hypernatremia and unusual manifestations derived from it, such as deep vein thrombosis, myopathy with rhabdomyolysis, and neurological axonal damage.
PubMed: 37179712
DOI: 10.31662/jmaj.2022-0194 -
Journal of Neurosurgery. Pediatrics Aug 2023Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a...
OBJECTIVE
Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort.
METHODS
A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy.
RESULTS
The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas.
CONCLUSIONS
The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.
Topics: Male; Child; Humans; Pinealoma; Pineal Gland; Glioma; Germinoma; Brain Neoplasms
PubMed: 37148224
DOI: 10.3171/2023.3.PEDS22468 -
Eye and Brain 2023Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner,... (Review)
Review
Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner, addressing the anatomic relationships that provide the basis for the uniqueness of pineal germinoma. Ocular findings and signs and symptoms of elevated intracranial pressure are the keys to suspecting the diagnosis and obtaining the necessary imaging and cerebrospinal fluid studies. Other symptoms can suggest spread beyond the pineal region. Surgery may only be needed to obtain tissue for a definitive diagnosis, as germinoma is highly responsive to chemotherapy and focused radiation therapy. Hydrocephalus, usually related to tumor obstruction of the cerebral aqueduct, may also need to be addressed. Outcome for pineal germinoma is usually excellent, but relapse can occur and may require additional intervention. These issues are detailed in this review.
PubMed: 37077304
DOI: 10.2147/EB.S389631 -
JCO Global Oncology Apr 2023This prospective Brazilian single-arm trial was conducted to determine response to chemotherapy and survival after response-based radiotherapy in children with...
PURPOSE
This prospective Brazilian single-arm trial was conducted to determine response to chemotherapy and survival after response-based radiotherapy in children with intracranial germinomas, in the setting of a multi-institutional study in a middle-income country (MIC) with significant disparity of subspecialty care.
PATIENTS AND METHODS
Since 2013, 58 patients with histologic and/or serum and CSF tumor marker evaluations of primary intracranial germ cell tumors were diagnosed; 43 were germinoma with HCGβ levels ≤200 mIU/mL and five between 100 and 200 mIU/mL. The treatment plan consisted of four cycles of carboplatin and etoposide followed by 18 Gy whole-ventricular field irradiation (WVFI) and primary site(s) boost up to 30 Gy; 24 Gy craniospinal was prescribed for disseminated disease.
RESULTS
Mean age 13.2 years (range, 4.7-25.5 years); 29 were males. Diagnosis was made by tumor markers (n = 6), surgery (n = 25), or both (n = 10). Two bifocal cases with negative tumor markers were treated as germinoma. Primary tumor location was pineal (n = 18), suprasellar (n = 14), bifocal (n = 10), and basal ganglia/thalamus (n = 1). Fourteen had ventricular/spinal spread documented by imaging studies. Second-look surgery occurred in three patients after chemotherapy. Thirty-five patients achieved complete responses after chemotherapy, and eight showed residual teratoma/scar. Toxicity was mostly grade 3/4 neutropenia/thrombocytopenia during chemotherapy. At a median follow-up of 44.5 months, overall and event-free survivals were 100%.
CONCLUSION
The treatment is tolerable, and WVFI dose reduction to 18 Gy preserves efficacy; we have demonstrated the feasibility of successfully conducting a prospective multicenter trial in a large MIC despite resource disparity.
Topics: Male; Humans; Child; Adolescent; Female; Prospective Studies; Brazil; Retrospective Studies; Brain Neoplasms; Germinoma; Biomarkers, Tumor
PubMed: 37075267
DOI: 10.1200/GO.22.00257 -
Journal of Neuro-oncology Apr 2023To determine long-term outcomes of a cohort of children with germinoma treated with chemotherapy and radiation therapy without primary tumor boost even in the absence of...
PURPOSE
To determine long-term outcomes of a cohort of children with germinoma treated with chemotherapy and radiation therapy without primary tumor boost even in the absence of complete response to chemotherapy METHODS: This retrospective study analyzed the outcome of patients with germinoma consecutively diagnosed and treated at a tertiary care center from January 2000 to December 2021. MRIs were reviewed by two radiologists, blinded to patient data. Tumor location at diagnosis, tumor response to chemotherapy and at completion of radiation therapy and site of relapse were assessed. Tumor response was assessed radiologically by determining the tumor size and response on diffusion-weighted imaging, in addition to biochemical, cytological parameters and neurological status.
RESULTS
Of 46 pediatric germinoma patients, 29 children (14 male; median age 12.8 years) received no primary tumor boost. Median follow-up was 63 months (range 9-187 months). Twenty-five children had localized disease and tumor location was suprasellar (n = 11), pineal (n = 10), bifocal (n = 3) and basal ganglia (n = 1) while 4 children had metastatic disease at presentation. All patients completed multi-agent chemotherapy followed by either ventricular irradiation (VI) (23.4 Gy) (n = 23), whole brain (WBI) (23.4 Gy) (n = 5) or craniospinal radiation (CSI) (23.4 Gy) (n = 1). Two children, who had localized disease at presentation and received VI after chemotherapy, relapsed 9 months and 32 months after completion of treatment respectively. No patient had a local relapse. Location of relapse was distant, outside (n = 1) and out- and inside (n = 1) the irradiation field. Five-year progression free survival (PFS) was 91% and overall survival (OS) was 100%.
CONCLUSIONS
In this case series, excellent 5-year PFS and OS rates were achieved with chemotherapy followed by radiation therapy of 23.4 Gy delivered without primary tumor boost. No local relapse was observed despite omitting primary tumor boost in patients with localized and metastatic germinoma.
Topics: Child; Humans; Male; Retrospective Studies; Brain Neoplasms; Neoplasm Recurrence, Local; Germinoma; Brain; Radiotherapy Dosage; Follow-Up Studies
PubMed: 37039951
DOI: 10.1007/s11060-023-04307-5 -
Journal of Clinical Neuroscience :... May 2023Primary suprasellar germinoma (PSG) is a rare malignant tumor of the central nervous system. This study aimed to explore the clinical characteristics, treatment...
Primary suprasellar germinoma (PSG) is a rare malignant tumor of the central nervous system. This study aimed to explore the clinical characteristics, treatment protocol, and prognosis of patients with PSG. This case series retrospectively analyzed the clinical data of patients with PSG in Tianjin Huanhu Hospital diagnosed between January 2016 and December 2021. Fifteen patients with an average age of 19.6 years were included, in which nine of them were males. The mean duration between initial symptoms and admission was 17.0 months. The mean follow-up was 40.8 months. Ten patients had polydipsia and polyuria, visual impairments were observed in 8 patients, and 2 cases (13.3%) had symptoms both from suprasellar and pineal regions. All 15 cases were histopathologically confirmed as germinoma through craniotomy or biopsy. Most patients (80%) underwent radiotherapy combined with chemotherapy. During follow-up, all the patients showed a reduction in tumor size, especially in the bifocal cases. Symptoms of polydipsia, polyuria, and visual impairment were markedly relieved to different degrees. All patients had recovered well at discharge. Patients with polydipsia and polyuria took desmopressin daily. A histological confirmation by open biopsy through craniotomy or endoscopic biopsy might be recommended for PSG to start the appropriate treatments. Patients with PSG will usually have a good prognosis, but attention should be paid to the treatment of endocrine deficiencies.
Topics: Male; Humans; Young Adult; Adult; Female; Brain Neoplasms; Retrospective Studies; Polyuria; Germinoma; Central Nervous System Neoplasms; Head and Neck Neoplasms
PubMed: 36963125
DOI: 10.1016/j.jocn.2023.03.007 -
NMC Case Report Journal 2023Primary germ cell tumors of the central nervous system (CNS) typically occur in the neurohypophysis, hypothalamus, or pineal gland and rarely in the spinal cord. We...
Primary germ cell tumors of the central nervous system (CNS) typically occur in the neurohypophysis, hypothalamus, or pineal gland and rarely in the spinal cord. We report a case of a spinal intramedullary tumor, which was first detected on magnetic resonance imaging (MRI) 41 months after the initial symptoms, with a verified pathological diagnosis of germinoma. The initial symptom was an abnormal sensation in the left plantar region that gradually worsened, resulting in severe sensory disturbance, difficulty in standing, and even bladder rectal disturbance. Repeated MRI after the onset failed to provide an imaging diagnosis. The MRI was performed 41 months after the onset and revealed a previously undiagnosed, contrast-enhancing spinal intramedullary neoplastic lesion at the Th11-12 level. Gross total resection of the tumor was successfully performed, and the pathology confirmed the diagnosis of pure germinoma. Postoperative chemotherapy, followed by local radiation, was successfully administered. Among primary germinomas of the CNS, occult germinoma that lacks imaging findings suggestive of tumors in the early stages of onset and becomes apparent over time is often reported as a primary neurohypophyseal germinoma, particularly in adolescents presenting with diabetes insipidus. In the present case, the lesion appeared to correspond to a primary occult germinoma of the intramedullary spinal cord.
PubMed: 36937498
DOI: 10.2176/jns-nmc.2022-0346 -
Clinical Neurology and Neurosurgery Apr 2023Intracranial germinomas are most commonly extra-axial germ cell tumors that are predominantly found in the pineal and suprasellar regions. Primary intra-axial midbrain... (Review)
Review
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.
Topics: Male; Humans; Adult; Germinoma; Brain Neoplasms; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Glioma; Mesencephalon
PubMed: 36863221
DOI: 10.1016/j.clineuro.2023.107643 -
Diagnostic Cytopathology Jun 2023Germ cell tumors are the most common malignancies in males between 20 and 40 years. However, primary extragonadal germ cell tumors are rare and account for 2%-5% of... (Review)
Review
Upper gastrointestinal bleed as the initial presentation of seminoma involving the duodenum in an elderly patient with 9 year follow up: A case report and literature review.
Germ cell tumors are the most common malignancies in males between 20 and 40 years. However, primary extragonadal germ cell tumors are rare and account for 2%-5% of all germ cell neoplasms in adults. Extragonadal germ cell tumors are characterized by their midline locations including pineal and suprasellar regions, mediastinum, retroperitoneum and sacrococcyx. These tumors have also been reported in rare locations such as prostate, bladder, vagina, liver and scalp. Extragonadal germ cell tumors can be primary but may also represent metastases from primary gonadal germ cell tumors. In this report we describe a case of a duodenal seminoma in a 66-year-old male with no history of testicular tumors, who presented with upper gastrointestinal bleed as the initial manifestation. He was effectively treated with chemotherapy and continues to progress well clinically, with no episodes of recurrence.
Topics: Male; Adult; Female; Humans; Aged; Seminoma; Testicular Neoplasms; Neoplasms, Germ Cell and Embryonal; Neoplasms, Second Primary; Duodenum
PubMed: 36861299
DOI: 10.1002/dc.25119