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Wiadomosci Lekarskie (Warsaw, Poland :... 2022A 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...
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.
Topics: Adolescent; Brain Neoplasms; Germinoma; Glioma; Humans; Magnetic Resonance Imaging; Male; Pineal Gland
PubMed: 36129092
DOI: 10.36740/WLek202208217 -
Child's Nervous System : ChNS :... Sep 2023The pineal region is a challenging area for neurosurgeons due to its innate anatomical features, such as its deep location, surrounding large draining veins, and... (Review)
Review
INTRODUCTION
The pineal region is a challenging area for neurosurgeons due to its innate anatomical features, such as its deep location, surrounding large draining veins, and adjacent critical neural structures.
DISCUSSION
There is a high proportion of malignant tumors in the pineal gland, especially in children, and they are frequently accompanied by obstructive hydrocephalus. These cases require that surgical procedures can make a pathological diagnosis to guide further treatment strategies and immediately resolve increased intracranial pressure. Simultaneous endoscopic third ventriculostomy and biopsy have been regarded as the first-line surgical intervention before establishing a definite treatment plan. However, it is not always successful because various factors affect the surgical procedures, such as the location and extent of the tumor, degree of ventriculomegaly, location and size of the massa intermedia, and size of the foramen of Monro.
CONCLUSION
Here, we briefly reviewed the points to be considered in endoscopic biopsy of pineal tumors and introduced an alternative surgical procedure, the endoscopic endonasal trans-tuber cinereum approach, to surmount the anatomical hurdles.
Topics: Child; Humans; Pinealoma; Tuber Cinereum; Pineal Gland; Biopsy; Hydrocephalus; Brain Neoplasms; Cerebral Ventricles
PubMed: 36112201
DOI: 10.1007/s00381-022-05654-w -
Journal of Neurosurgery. Pediatrics Nov 2022In patients with intracranial germ cell tumors, residual lesions are sometimes observed after completion of primary chemoradiotherapy. Although salvage resection of...
OBJECTIVE
In patients with intracranial germ cell tumors, residual lesions are sometimes observed after completion of primary chemoradiotherapy. Although salvage resection of these end-of-treatment residual lesions is recommended for patients with nongerminomatous germ cell tumors, the necessity of early salvage resection for those with germinoma is not clear. The aim of this study was to investigate the frequency of residual germinoma lesions after primary chemoradiotherapy, as well as their management, long-term consequences, and prognosis.
METHODS
The authors retrospectively reviewed patients who were primarily treated for germinoma between 2002 and 2021. Residual lesions were evaluated with MRI with and without contrast enhancement within 2 weeks after chemoradiotherapy. The decision to perform salvage resection of residual lesions was at the discretion of the treating physicians. The change in appearance of residual lesions was assessed with serial MRI. Overall survival (OS), progression-free survival (PFS), and recurrence pattern were also investigated.
RESULTS
Sixty-nine patients were treated with chemoradiotherapy for germinoma, with a mean follow-up period of 108 months. Residual lesions were radiologically observed in 30 patients (43.5%). Among these, 5 patients (3 with pineal lesions and 2 with basal ganglia lesions) underwent salvage resection. Pathological examination revealed teratomatous components in 3 patients, whereas no tumoral components were identified in 2 patients. One patient with a basal ganglia lesion showed worsening of hemiparesis postoperatively. The remaining 25 patients received watchful observation without surgical intervention. Chronological periodic radiological change in residual lesions was evaluated in 21 patients. One year after primary treatment, the size of the residual lesions was stable and had decreased in 10 and 11 patients, respectively. None of the lesions increased in size. The 10-year PFS and OS rates were 96.7% and 97.3% in patients without residual lesions (n = 39), and 87.1% and 100% in patients with residual lesions (n = 30), respectively. Presence of residual lesions had no significant effect on PFS or OS. All recurrences occurred at distant sites or via dissemination without progression of the primary tumor site, regardless of the presence of residual lesion.
CONCLUSIONS
End-of-treatment residual lesions are not rare in patients with germinoma, and these residual lesions seldom show progression. Because of the potential risk of surgical complications, the indication for early salvage surgery for residual lesions should be carefully determined. Watchful observation is recommended for the majority of these cases.
PubMed: 36087334
DOI: 10.3171/2022.8.PEDS22301 -
Cureus Aug 2022Tumors involving the pineal gland include germinomas, non-germinomatous, and parenchymal tumors. Sometimes these tumors can be differentiated into rhabdomyosarcoma,...
Tumors involving the pineal gland include germinomas, non-germinomatous, and parenchymal tumors. Sometimes these tumors can be differentiated into rhabdomyosarcoma, which is an aggressive and rapidly recurring sarcoma but is a rare event. We present the case of a 23-year-old male, with an eight-year-long history of a non-treated brain tumor compatible with a teratoma. Chemotherapy and radiotherapy were offered, and two years later, malignant transformation to astrocytoma, rhabdomyosarcoma, neural cell carcinoma, ganglioglioma, and low-grade chondrosarcoma was noted. Immunohistochemistry was valuable in differentiating these entities that confirmed the diagnosis. Malignant transformations may be secondary to the normal transformation of multipotent embryonic cells into more developed tissues after radiotherapy of teratoma and malignant ectomesenchymoma transformation.
PubMed: 36081970
DOI: 10.7759/cureus.27711 -
Cureus Jul 2022Germ cell tumors are rare tumors that most commonly occur in the pediatric population. Their usual location is in the pineal gland and above the suprasellar cistern....
Germ cell tumors are rare tumors that most commonly occur in the pediatric population. Their usual location is in the pineal gland and above the suprasellar cistern. Pure germinomas, the most common type of germ cell tumor, are exquisitely sensitive to radiation, and rarely recur after radiotherapy treatment. We present a case of a pure germinoma that (1) occurred in the cerebellar hemisphere, (2) in the fifth decade of life, and (3) after being adequately treated with radiotherapy.
PubMed: 36060381
DOI: 10.7759/cureus.27535 -
Modern Pathology : An Official Journal... Dec 2022Central nervous system (CNS) germ cell tumors (GCTs) represent 2-3% of all primary CNS tumors. The majority are germinomas, which are radiosensitive and have an...
Central nervous system (CNS) germ cell tumors (GCTs) represent 2-3% of all primary CNS tumors. The majority are germinomas, which are radiosensitive and have an excellent prognosis. Contrarily, CNS non-germinomatous GCTs (NGGCTs) have less favorable prognosis and require more aggressive treatment. The expression of checkpoint/immune markers in CNS GCTs, particularly NGGCTs, is unknown. We previously reported a case of a patient whose intracranial NGGCT (predominantly choriocarcinoma) responded to immune checkpoint inhibition therapy. This case led us to evaluate our archive of intracranial GCTs for expression of PD-L1 and PD-1. With IRB approval, we searched the pathology archives at our institution for CNS GCTs. Demographic, radiologic, clinical, and histologic information was extracted from the medical records. Immunohistochemistry for lymphocytic markers (CD4, CD8, CD20), PD-1, and PD-L1 was performed. PD-L1 was considered positive if greater than 1% of tumor cells were positive and PD-1 was reported as a percentage of positive inflammatory cells. Fifty cases were identified, including 28 germinomas (mean age at diagnosis: 15.5 years; 17 males, 11 females), and 22 NGGCTs (mean age at diagnosis: 12.0 years, 21 males, 1 female). Germinomas were mostly suprasellar (17/28) and NGGCTs were predominantly pineal (17/22). Twenty-two germinomas (79%) were positive for PD-L1 expression, and 13 NGGCTs (57%) were positive for PD-L1. Cases of choriocarcinoma showed the most diffuse PD-L1 expression. PD-1 expression was seen in lymphocytes among 27/28 of the germinomas and 20/23 of the NGGCTs (ranging from 1-40% of lymphocytes). As expected, larger quantities of inflammatory cells were present in cases of germinoma. We demonstrate immune activity in CNS GCTs, and our results suggest that immune checkpoint inhibitors may be efficacious in the treatment of intracranial GCTs. Among NGGCTs, cases of choriocarcinoma showed the highest expression of PD-L1 in tumor cells, suggesting that this subtype may have the greatest benefit from checkpoint blockade.
Topics: Child; Male; Humans; Female; Adolescent; Programmed Cell Death 1 Receptor; B7-H1 Antigen; Neoplasms, Germ Cell and Embryonal; Germinoma; Brain Neoplasms; Central Nervous System Neoplasms; Choriocarcinoma; Central Nervous System
PubMed: 36057740
DOI: 10.1038/s41379-022-01142-3 -
Cancers Jul 2022Pineal region tumors are rare intracranial tumors, accounting for less than 1% of all adult intracranial tumor lesions. These lesions represent a histologically... (Review)
Review
Pineal region tumors are rare intracranial tumors, accounting for less than 1% of all adult intracranial tumor lesions. These lesions represent a histologically heterogeneous group of tumors. Among these tumors, pineal parenchymal tumors and germ cell tumors (GCT) represent the most frequent types of lesions. According to the new WHO 2021 classification, pineal parenchymal tumors include five distinct histotypes: pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID), papillary tumor of the pineal region (PTPR), pinealoblastoma (PB), and desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant; GCTs include germinoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, mixed GCTs. Neuroradiological assessment has a pivotal role in the diagnostic work-up, surgical planning, and follow-up of patients with pineal masses. Surgery can represent the mainstay of treatment, ranging from biopsy to gross total resection, yet pineal region tumors associated with obstructive hydrocephalus may be surgically managed via ventricular internal shunt or endoscopic third ventriculostomy. Radiotherapy remains an essential component of the multidisciplinary treatment approach for most pineal region tumors; however, treatment volumes depend on the histological subtypes, grading, extent of disease, and the combination with chemotherapy. For localized germinoma, the current standard of care is chemotherapy followed by reduced-dose whole ventricular irradiation plus a boost to the primary tumor. For pinealoblastoma patients, postoperative radiation has been associated with higher overall survival. For the other pineal tumors, the role of radiotherapy remains poorly studied and it is usually reserved for aggressive (grade 3) or recurrent tumors. The use of systemic treatments mainly depends on histology and prognostic factors such as residual disease and metastases. For pinealoblastoma patients, chemotherapy protocols are based on various alkylating or platinum-based agents, vincristine, etoposide, cyclophosphamide and are used in association with radiotherapy. About GCTs, their chemosensitivity is well known and is based on cisplatin or carboplatin and may include etoposide, cyclophosphamide, or ifosfamide prior to irradiation. Similar regimens containing platinum derivatives are also used for non-germinomatous GCTs with very encouraging results. However, due to a greater understanding of the biology of the disease's various molecular subtypes, new agents based on targeted therapy are expected in the future. On behalf of the EURACAN domain 10 group, we reviewed the most important and recent developments in histopathological characteristics, neuro-radiological assessments, and treatments for pineal region tumors.
PubMed: 35954310
DOI: 10.3390/cancers14153646 -
Cancers Jul 2022Background: CNS germinoma, being marker-negative, are mainly diagnosed by histological examination. These tumors predominantly appear in the suprasellar and/or pineal...
Background: CNS germinoma, being marker-negative, are mainly diagnosed by histological examination. These tumors predominantly appear in the suprasellar and/or pineal region. In contrast to the suprasellar region, where biopsy is the standard procedure in case of a suspected germ-cell tumor to avoid mutilation to the endocrine structures, pineal tumors are more accessible to primary resection. We evaluated the perioperative course of patients with pineal germinoma who were diagnosed by primary biopsy or resection in the SIOP CNS GCT 96 trial. Methods: Overall, 235 patients had germinoma, with pineal localization in 113. The relationship between initial symptoms, tumor size, and postoperative complications was analyzed. Results: Of 111 evaluable patients, initial symptoms were headache (n = 98), hydrocephalus (n = 93), double vision (n = 62), Parinaud syndrome (n = 57), and papilledema (n = 44). There was no significant relationship between tumor size and primary symptoms. A total of 57 patients underwent primary resection and 54 underwent biopsy. Postoperative complications were reported in 43.2% of patients after resection and in 11.4% after biopsy (p < 0.008). Biopsy was significantly more commonly performed on larger tumors (p= 0.002). Conclusions: These results support the practice of biopsy over resection for histological confirmation of pineal germinoma.
PubMed: 35884617
DOI: 10.3390/cancers14143555 -
Neuro-ophthalmology (Aeolus Press) 2022Suprasellar germinomas can present with non-diagnostic, or even normal results on imaging. The spectrum of reported cases ranges from normal imaging, thickened pituitary...
Suprasellar germinomas can present with non-diagnostic, or even normal results on imaging. The spectrum of reported cases ranges from normal imaging, thickened pituitary stalks, to discrete tumour growths. This similar phenomenon is less commonly seen in the pineal region, or bifocal germinomas, and the literature is sparse with only a few case series or reports mentioning a similar presentation of signs and symptoms preceding radiological evidence of diagnosis. We report a case of pineal germinoma presenting with dorsal midbrain syndrome with no evidence of tumour growth on initial imaging despite symptoms. For patients presenting with this clinical radiological latent period, follow-up imaging is useful to identify interval development of germinomas. This applies to patients with dorsal midbrain syndrome, or even other unexplained ophthalmoplegia, as the initial sign of pineal region germinoma, despite normal imaging.
PubMed: 35859628
DOI: 10.1080/01658107.2022.2034890 -
Neurology Sep 2022
Topics: Atrophy; Brain Neoplasms; Corpus Callosum; Germinoma; Humans; Magnetic Resonance Imaging; Pineal Gland
PubMed: 35853744
DOI: 10.1212/WNL.0000000000201096