LINC-14. TREATMENT OF PEDIATRIC CNS TUMORS IN ARMENIA. 10 YEARS OF EXPERIENCE IN A 29 YEARS OLD RESOURCE-LIMITED SETTING

Abstract BACKGROUND Pediatric CNS tumors are the most common solid childhood malignancies with many challenges facing optimal outcome due to multimodality complex therapies, abandonment, and long-term morbidity. In our three-decades young, country the field of neuro-oncology is in its infancy. MATERIALS: The aim of our study is to assess incidence, epidemiology and treatment outcomes of children diagnosed and treated with CNS tumors within the last 10 years (2009–2019) in the Chemotherapy Clinic of “Muratsan” Hospital Complex of Yerevan State Medical University. RESULTS During these periods 20 patients with CNS tumors were treated in our clinic. 13 patients (65%) were diagnosed with medulloblastoma (2 patients were infants), two patients (10%) with optic pathway glioma, and 5 patients each with pilocytic astrocytoma, ATRT, ETANTR, DIPG, and glioblastoma. Five patients (3 patients with medulloblastoma, 1 patient with pilocytic astrocytoma, 1 patient with ATRT) had metastatic disease at the time of diagnosis. Seventeen patients (80%) had undergone surgery, 8 patients with medulloblastoma received chemo-RT with vincristine. Median follow up time was 15.5 months (range 5–94). Twelve patients (60%) are alive without evidence of disease. 5 patients had disease progression and three patients relapsed. From them, 3 patients died. Long-term survivors are mainly standard risk medulloblastoma patients. All medulloblastoma patients were treated according to HIT-MED guidelines. CONCLUSION Here we report about the pediatric brain tumors of one of the main pediatric oncology units in Armenia for a period of 10 years. The numbers are quite small for firm conclusions, but it shows the emerging need for further research.

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Survival and Late Mortality in Long-Term Survivors of Pediatric CNS Tumors

Journal of Clinical Oncology ◽

10.1200/jco.2006.09.8194 ◽

2007 ◽

Vol 25 (12) ◽

pp. 1532-1538 ◽

Cited By ~ 34

Author(s):

E. Brannon Morris ◽

Amar Gajjar ◽

James O. Okuma ◽

Yutaka Yasui ◽

Dana Wallace ◽

...

Keyword(s):

Cause Of Death ◽

Progressive Disease ◽

Cns Tumors ◽

Tumor Type ◽

Cns Tumor ◽

Late Mortality ◽

Significant Difference ◽

Long Term Survivors ◽

Pediatric Cns Tumors

Purpose To describe the pattern of survival and late mortality among contemporary long-term survivors of pediatric CNS tumor. Patients and Methods The study population comprised 643 pediatric patients with primary CNS tumor treated at St Jude Children's Research Hospital (Memphis, TN) from 1985 to 2000 who survived ≥ 5 years from diagnosis. Patients were classified according to primary tumor type, location of tumor, and survival. Cause of death was obtained from the medical record and categorized as progression, malignant transformation, second malignancy, medical complication, or external cause. Results Overall survival estimates for patients who survived at least 5 years postdiagnosis was 91.3% ± 2% and 86% ± 3% at 10 and 15 years postdiagnosis, respectively. A significant difference in the survival rates according to original tumor type (P = .001) was seen. Sixty-six (10%) of 643 patients experienced late mortality: 38 patients (58%) died of progressive disease while 14 patients (21%) died of second malignant tumor. Twelve patients (18%), predominantly with diencephalic tumor location, died of a specific medical cause: cardiovascular disease (n = 2), cerebrovascular accident (n = 1), metabolic collapse and/or sepsis (n = 7), respiratory failure (n = 1), or shunt malfunction (n = 1). Conclusion Late mortality occurs in a substantial number of long-term survivors of pediatric CNS tumors and is most influenced by the initial tumor histopathology. Progressive disease remains the most common cause of death within the first decade of diagnosis. Teenage patients requiring treatment for panhypopituitarism may be especially vulnerable and deserve significant medical surveillance.

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EXTH-16. TREATMENT OF THREE DIFFERENT BRAF-V600E POSITIVE BRAIN TUMORS WITH VEMURAFENIB AND DABRAFENIB/TRAMETINIB: A CASE SERIES

Neuro-Oncology ◽

10.1093/neuonc/noaa215.370 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii90-ii90

Author(s):

Nikita Dhir ◽

Sheila Chandrahas ◽

Chibuzo O’Suoji ◽

Mohamad Al-Rahawan

Keyword(s):

Targeted Therapy ◽

Brain Tumors ◽

Tumor Regression ◽

Case Series ◽

Pediatric Brain Tumors ◽

Braf V600e ◽

Cns Tumors ◽

Braf Inhibitors ◽

Pediatric Brain ◽

Pediatric Cns Tumors

Abstract BACKGROUND The BRAF-V600E gene is a protein kinase involved in regulation of the mitogen activated protein kinase pathway (MAPK/MEK) and downstream extracellular receptor kinase (ERK). The BRAF-V600E mutation has a significant role in the progression of pediatric brain tumors. 85% of pediatric CNS tumors express the BRAF mutation. Thus, BRAF targeted therapy in pediatric CNS malignancies has potential to become the standard of care for tumors expressing this mutation. OBJECTIVE Current pediatric CNS brain tumor treatment focuses on chemotherapy and radiation, causing significant toxic side effects for patients. The significance of this case series lies in relaying our experience using targeted therapy in BRAF-V600E positive CNS pediatric brain tumors. METHODS We followed the disease course, progression, and treatment of three pediatric patients with three different CNS tumors. Each of these individuals was treated with surgical resection, chemotherapy, and/or radiation as per standard protocol. When that modality failed to reduce tumor progression, we found that each of their different tumors was BRAF-V600E positive and they were all started on targeted therapy. DISCUSSION Vemurafenib, Dabrafenib, and Trametinib are BRAF-V600E/MEK inhibitors that were initially used to treat melanomas. However, more research has shown that various pediatric CNS tumors are BRAF-V600 positive. Therapy with these BRAF inhibitors has been shown to slow tumor progression, but toxicity can be severe. This case series shows one patient with successful tumor regression, one patient with prolonged disease stabilization, and one patient with initial response but subsequent progression and ultimate death. It has been shown that using BRAF inhibitors in lower grade CNS tumors are more effective than higher grade CNS tumors. CONCLUSION The success of Vemurafenib and Dabrafenib/Trametinib in causing pediatric CNS tumor regression is promising, but further studies are needed to solidify their role in pediatric CNS cancers.

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Quality of life in long-term survivors of CNS tumors of childhood and adolescence.

Journal of Clinical Oncology ◽

10.1200/jco.1991.9.4.592 ◽

1991 ◽

Vol 9 (4) ◽

pp. 592-599 ◽

Cited By ~ 137

Author(s):

E N Mostow ◽

J Byrne ◽

R R Connelly ◽

J J Mulvihill

Keyword(s):

Quality Of Life ◽

Late Effects ◽

Pediatric Brain Tumor ◽

Adverse Outcomes ◽

Cns Tumors ◽

Childhood And Adolescence ◽

Long Term Survivors

Clinical reports of small numbers of pediatric brain tumor patients observed for brief periods suggest that long-term survivors continue to have major handicaps into adulthood. To quantify these late effects we interviewed 342 adults (or their proxies) who had CNS tumors diagnosed before the age of 20 between 1945 and 1974, survived at least 5 years, and reached 21 years of age. Survivors were 32 years old on average at follow-up. When compared with 479 matched siblings as controls. CNS tumor survivors were more likely to have died or to have become mentally incompetent sometime during the follow-up period. They were more likely to be at risk for such adverse outcomes as unemployment (odds ratio [OR], 10.8; 95% confidence interval [CI], 4.6 to 25.7], to have a health condition that affected their ability to work (OR, 5.9; CI, 3.7 to 9.4), to be unable to drive (OR, 28.8; CI, 6.9 to 119.9), or to describe their current health as poor (OR, 7.8; CI, 1.7 to 35.7). Unfavorable outcomes were more frequent in male survivors than in females, in those with supratentorial tumors compared with infratentorial ones, and in those who received radiation therapy. As clinicians consider improving therapies, they should anticipate late effects, such as those we observed, and attempt to target subgroups for interventions that may improve subsequent quality of life.

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Incidence, risk factors, and longitudinal outcome of seizures in long-term survivors of pediatric brain tumors

Epilepsia ◽

10.1111/epi.13112 ◽

2015 ◽

Vol 56 (10) ◽

pp. 1599-1604 ◽

Cited By ~ 27

Author(s):

Nicole J. Ullrich ◽

Scott L. Pomeroy ◽

Kush Kapur ◽

Peter E. Manley ◽

Liliana C. Goumnerova ◽

...

Keyword(s):

Risk Factors ◽

Brain Tumors ◽

Pediatric Brain Tumors ◽

Longitudinal Outcome ◽

Incidence Risk ◽

Long Term Survivors ◽

Pediatric Brain

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BT-4 The treatment history of long-term survivors of Optic Pathway Glioma in Kobe Children’s Hospital

Neuro-Oncology Advances ◽

10.1093/noajnl/vdab159.104 ◽

2021 ◽

Vol 3 (Supplement_6) ◽

pp. vi27-vi27

Author(s):

Atsufumi Kawamura ◽

Junji Koyama ◽

Nobuyuki Akutsu ◽

Masashi Higashino ◽

Kenji Fujita

Keyword(s):

Treatment Plan ◽

Continuous Treatment ◽

Optic Pathway Glioma ◽

Optic Pathway ◽

Children's Hospital ◽

Fibrillary Astrocytoma ◽

Children’S Hospital ◽

History Of ◽

Long Term Survivors

Abstract Optic pathway glioma(OPG) is almost recognized in childhood and about 0.01–0.02% of whole brain tumor in Japan. Because of the rare tumor, there are few reports about results of its treatment. In 2021, Guideline of Optic pathway/hypothalamic glioma is indicated from The japan Society for Neuro-Oncology. We retrospectively study 9 cases history of OPG who have treated for more than 5 years from January 2005 to March 2021 in Kobe Children’s Hospital. Cases are 4 boys and 5 girls. Average age at diagnosis is 3.8 years old and average follow up term is 11 years 10 months. They are 4 Pilocytic astrocytoma, 3 Pilomyxoid astrocytoma, and 1 Fibrillary astrocytoma. Al l cases have survived. There are only 2 cases who could be controlled with single series of surgical treatment and chemotherapy. Most cases need several times of resection and chemotherapy and uncontrollable 5 cases required radiological treatment. 2 cases are still under treatment for over 10 years. For OPG, partial resection to control hydrocephalus is recommended and several trial of chemotherapy must be carried out. There exist a few cases who need continuous treatment for long term. The other side, a few cases are uncontrollable those need radiotherapy to manage tumor volume. Because the history of OPG would be long term, we should adjust the treatment plan to environment of patients.

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