PTCT-01intercontinental Multidisciplinary Data Collection and Treatment Optimization Study for Patients With Choroid Plexus Tumors

Bahar, Michal; Dhir, Aditi; Kordes, Uwe; Wolff, Johannes

doi:10.1093/neuonc/nov229.01

Cited by 3 publications

(4 citation statements)

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“…The subsequent CPT-SIOP-2009 trial ventured to randomize patients between 4 chemotherapy arms with alternating cycles of CarbEV/CycEV as the backbone but was closed prematurely due to insufficient accrual. 30 Owing to the still less than satisfactory outcomes, the use of myeloablative chemotherapy and autologous stem cell rescue as consolidation was explored in the Head Start series. 9 However, the added intensity did not appear to enhance patient survival and the 5-year EFS was reported as 38%.…”

Section: Discussionmentioning

confidence: 99%

Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial

Liu

Orr

et al. 2020

Neuro-Oncology Advances

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Background Choroid plexus carcinoma (CPC) is a rare and aggressive tumor of infancy without a clear treatment strategy. This study describes the outcomes of children with CPC treated on the multi-institutional phase 2 SJYC07 trial and reports on the significance of clinical and molecular characteristics. Methods Eligible children <3 years-old with CPC were postoperatively stratified to intermediate-risk (IR) stratum if disease was localized or high-risk (HR) stratum, if metastatic. All received high-dose methotrexate–containing induction chemotherapy. IR-stratum patients received focal irradiation as consolidation whereas HR-stratum patients received additional chemotherapy. Consolidation was followed by oral antiangiogenic maintenance regimen. Survival rates and potential prognostic factors were analyzed. Results Thirteen patients (median age: 1.41 years, range: 0.21–2.93) were enrolled; 5 IR, 8 HR. Gross-total resection or near-total resection was achieved in ten patients and subtotal resection in 3. Seven patients had TP53-mutant tumors, including 4 who were germline carriers. Five patients experienced progression and died of disease; 8 (including 5 HR) are alive without progression. The 5-year progression-free survival (PFS) and overall survival rates were 61.5 ± 13.5% and 68.4 ± 13.1%. Patients with TP53-wild-type tumors had a 5-year PFS of 100% as compared to 28.6 ± 17.1% for TP53-mutant tumors (P = .012). Extent of resection, metastatic status, and use of radiation therapy were not significantly associated with survival. Conclusions Non-myeloablative high-dose methotrexate–containing therapy with maximal surgical resection resulted in long-term PFS in more than half of patients with CPC. TP53-mutational status was the only significant prognostic variable and should form the basis of risk-stratification in future trials.

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Section: Discussionmentioning

confidence: 99%

Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial

Liu

Orr

et al. 2020

Neuro-Oncology Advances

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“…Двухлетние ОВ и БСВ для 12 пациентов с ХК, получавших альтернирующие циклы CarbEV/CycEV в рукаве А при медиане наблюдения 1,7 года, составили 78% и 35% соответственно, что оказалось ниже, чем у больных, получавших неальтернирующие циклы CarbEV (от 6 циклов): 76% и 63% соответственно. Основываясь на этих данных, авторы не рекомендуют применение альтернирующих циклов CarbEV/CycEV для лечения детей с ХК [21].…”

Section: рисунокunclassified

Choroid plexus carcinoma in children

Valiakhmetova

Papusha

Sanakoeva

et al. 2021

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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Choroid plexus carcinomas (CPCs) are rare pediatric tumors with a generally poor prognosis. Currently there is no definite optimal treatment strategy for this neoplasm. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. This study included patients with a verified diagnosis of CPC at the age 0 to 18 years in the period from 01.01.2009 to 31.12.2019. A total of 32 patients were registered. The median age was 2.65 years; 93.5% of CPCs were localized in the lateral ventricles. Initial metastases were found in 21.8% of cases; 5-year event-free survival (EFS) in children with metastases was lower than in those who did not have metastases 29 ± 17% and 49 ± 12%. In our cohort, gross total resection (GTR) was performed in 65.6% of patients. The five-year EFS in patients with complete CPC removal was higher than in patients who underwent subtotal and partial resection (63 ± 13%, 12 ± 11%, and 0%, respectively). In addition, overall survival (OS) was slightly higher in those who underwent GTR compared with subtotal and partial resection (74 ± 12%, 67 ± 16%, and 0%, respectively). Of the 32 children with CPCs, 15 children received programm chemotherapy, 17 non-programm chemotherapy, 5-year EFS in patients who received programm and non-programm chemotherapy was 79 ± 11% and 0%, respectively (p = 0.0006), 5-year OS in patients who received programm and non-programm chemotherapy was 93 ± 7% and 36 ± 14% (p = 0.0054).

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“…P. Pencalet et al отметили, что 9 детей в их серии нуждались в постановке шунтов через несколько месяцев после удаления опухоли [24]. C. Bettegowda et al отметили разрешение гидроцефалии после резекции опухоли у большинства пациентов (> 78 %); однако 9 больным потребовалось послеопе-Российский журнал ДЕТСКОЙ ГЕМАТОЛОГИИ и ОНКОЛОГИИ R u s s i a n J o u r n a l o f P e d i a t r i c H e m a t o l o g y а n d O n c o l o g y 56…”

Section: хирургическое удаление опухолиunclassified

“…Основываясь на этих данных, авторы не рекомендуют использование альтернирующих циклов карбоплатин/ циклофосфамид вместе с этопозидом и винкристином для лечения детей с ХК. Однако следует отметить, что в данном исследовании не учитывалась информация о наличии синдрома Ли-Фраумени [56].…”

Section: лекарственная терапия цитостатическая химиотерапияunclassified

Choroid plexus carcinoma: review of literature

Valiakhmetova

Papusha

Yasko

et al. 2020

Ross. ž. det. gematol. onkol.

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Choroid plexus carcinoma (CPC) is a rare malignant tumor arising from the epithelium of the choroid plexus of the brain. More than 80 % of CPCs occur in children. Mutations in the TP53 gene is played the main role in the pathogenesis of these tumors. Choroid plexus carcinomas in 40 % of cases are associated with Li–Fraumeni syndrome. Survival rates in patients with CPC and Li–Fraumeni syndrome are extremely low. The standards of the therapy for patients with CPC are not defined. The extent of surgical resection and treatment modality correlate with prognosis. The role of adjuvant therapy in CPC remains unclear: doses and volumes of radiation therapy (RT), combinations of chemotherapeutic drugs, timing, and a combination of RT and chemotherapy (CT) have not been identified. Also, there is neither a standard CT regimen nor a prospective international study assessing the efficacy and toxicity of various combinations of cytostatics in patients with CPC. The article presents an overview of the existing molecular genetic changes, existing methods for the diagnosis and treatment of choroid plexus carcinoma.

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PTCT-01intercontinental Multidisciplinary Data Collection and Treatment Optimization Study for Patients With Choroid Plexus Tumors

Cited by 3 publications

References 0 publications

Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial

Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial

Choroid plexus carcinoma in children

Choroid plexus carcinoma: review of literature

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