Intracranial germ cells tumour: a single institution experience in Argentina

Baroni, Lorena; Oller, Agustina; Freytes, Candela; Sampor, Claudia; Pinto, Natalia; Fernandez, Nicolas Ponce; Rugilo, Carlos; Lubieniecki, Fabiana; Zubizarreta, Pedro; Alderete, Daniel

doi:10.1007/s11060-021-03709-7

Cited by 6 publications

(3 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Sixteen patients (37.2%) from other regions of Brazil came as newly diagnosed patients referenced by the unified health system (SUS). Other retrospectives experiences in MIC also shows the importance of cooperative practices in other MIC 34 , 35 and barriers to be addressed such as late diagnosis, poor adherence to treatment, and treatment-related complications. 36 , 37 …”

Section: Discussionmentioning

confidence: 96%

Outcome of Children and Adolescents With Primary Intracranial Germinoma Treated With Chemotherapy and Reduced Dose-Field Irradiation: A Prospective Brazilian Experience

Cappellano

Dassi

Mançano

et al. 2023

JCO Global Oncology

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 96%

Outcome of Children and Adolescents With Primary Intracranial Germinoma Treated With Chemotherapy and Reduced Dose-Field Irradiation: A Prospective Brazilian Experience

Cappellano

Dassi

Mançano

et al. 2023

JCO Global Oncology

View full text Add to dashboard Cite

show abstract

“…Platinum‐based chemotherapy is quite effective. Chemotherapy alone is associated with a high risk of relapse [ 10 ].…”

Section: Methodsmentioning

confidence: 99%

Clinical characteristics of malignant germ cell tumors in adolescents: A multicenter 10‐year retrospective study in Beijing

Zhao,

Li,

Sun

et al. 2023

Cancer Innovation

View full text Add to dashboard Cite

BackgroundThe aim of this study was to review clinical features of adolescent malignant germ cell tumors (MGCTs) in Beijing and analyze the peculiar characteristics of this age group.MethodsClinical characteristics, pathological presentations, and survival outcomes of 34 patients were analyzed retrospectively.ResultsOf 34 patients, 12 girls and 22 boys, 18 (52.9%) had an extra‐cranial tumor, including one testicular tumor, five ovarian tumors, one sacrococcygeal tumor, and 11 mediastinal tumors. Histologically, we found immature teratomas (n = 6), yolk sac tumors (n = 5), mixed malignant tumors (n = 5), an embryonic carcinoma (n = 1), and seminoma (n = 1). Three‐year event‐free survival (EFS) and overall survival (OS) were 48.8% and 62.9%, respectively. Another 16 (47.1%) patients had an intracranial tumor, including nine in the pineal region, five in the suprasellar region, one in basal ganglia, and one in cerebellopontine. All patients had localized disease and an excellent outcome with 3‐year EFS and OS of 93.7% and 100%, respectively.ConclusionsAdolescent MGCTs are rare with a strong dependence on gender, and the mediastina and pineal region are the most common tumor locations. The prognosis is promising compared with that of other adolescent tumors and MGCTs in other age groups. MGCTs in mediastina have a tendency to companion with other hematological malignancies, and the prognosis is extremely poor in these patients.

show abstract

“…Currently, there is no consensus regarding the superior sensitivity between serum and CSF markers. Initial evaluation shall be carried out 1–2 months after treatment completion and every 4–6 months thereafter, for the first 2–5 years, followed by annual assessments [ 51 , 76 , 148 , 149 ].…”

Section: Current Managementmentioning

confidence: 99%

Molecular Pathology and Targeted Therapies for Personalized Management of Central Nervous System Germinoma

Ilcus

Silaghi

Georgescu

et al. 2021

JPM

View full text Add to dashboard Cite

Intracranial germinomas are rare tumours, usually affecting male paediatric patients. They frequently develop in the pineal and suprasellar regions, causing endocrinological disturbances, visual deficits, and increased intracranial pressure. The diagnosis is established on magnetic resonance imaging (MRI), serum and cerebrospinal fluid (CSF) markers, and tumour stereotactic biopsy. Imaging techniques, such as susceptibility-weighted imaging (SWI), T2* (T2-star) gradient echo (GRE) or arterial spin labelling based perfusion-weighted MRI (ASL-PWI) facilitate the diagnosis. Germinomas are highly radiosensitive tumours, with survival rates >90% in the context of chemoradiotherapy. However, patients with resistant disease have limited therapeutic options and poor survival. The aim of this review is to highlight the genetic, epigenetic, and immunologic features, which could provide the basis for targeted therapy. Intracranial germinomas present genetic and epigenetic alterations (chromosomal aberrations, KIT, MAPK and PI3K pathways mutations, DNA hypomethylation, miRNA dysregulation) that may represent targets for therapy. Tyrosine kinase and mTOR inhibitors warrant further investigation in these cases. Immune markers, PD-1 (programmed cell death protein 1) and PD-L1 (programmed death-ligand 1), are expressed in germinomas, representing potential targets for immune checkpoint inhibitors. Resistant cases should benefit from a personalized management: genetic and immunological testing and enrolment in trials evaluating targeted therapies in intracranial germinomas.

show abstract

Intracranial germ cells tumour: a single institution experience in Argentina

Cited by 6 publications

References 29 publications

Outcome of Children and Adolescents With Primary Intracranial Germinoma Treated With Chemotherapy and Reduced Dose-Field Irradiation: A Prospective Brazilian Experience

Outcome of Children and Adolescents With Primary Intracranial Germinoma Treated With Chemotherapy and Reduced Dose-Field Irradiation: A Prospective Brazilian Experience

Clinical characteristics of malignant germ cell tumors in adolescents: A multicenter 10‐year retrospective study in Beijing

Molecular Pathology and Targeted Therapies for Personalized Management of Central Nervous System Germinoma

Contact Info

Product

Resources

About