Successful treatment of primary intracranial sarcoma with the ICE chemotherapy regimen and focal radiation in children

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Primary CNS sarcomas are very rare pediatric tumors with no defined standard of care.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>This study was a retrospective review of children diagnosed with a primary CNS sarcoma and treated at 2 Canadian tertiary care centers between 1995 and 2012. This report focuses on patients with cerebral hemispheric tumor location due to their specific clinical presentation.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Fourteen patients with nonmetastatic primary CNS sarcoma were identified; in 9 patients, tumors were located in the cerebral hemisphere and 7 of these patients presented with intratumoral hemorrhage. One infant who died of progressive disease postoperatively before receiving any adjuvant therapy was not included in this study. The final cohort therefore included 8 patients (4 males). Median patient age at diagnosis was 11.8 years (range 5.8–17 years). All tumors were located in the right hemisphere. Duration of symptoms prior to diagnosis was very short with a median of 2 days (range 3–7 days), except for 1 patient. Three (37.5%) patients had an underlying diagnosis of neurofibromatosis Type 1 (NF1). Gross-total resection was achieved in 5 patients. The dose of focal radiation therapy (RT) ranged between 54 Gy and 60 Gy. Concomitant etoposide was administered during RT. ICE (ifosfamide, carboplatin, etoposide) chemotherapy was administered prior to and after RT for a total of 6–8 cycles. Seven of the 8 patients were alive at a median time of 4.9 years (range 1.9–17.9 years) after treatment.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>In this retrospective series, patients with primary CNS sarcomas located in the cerebral hemisphere most commonly presented with symptomatic acute intratumoral hemorrhage. Patients with NF1 were overrepresented. The combination of adjuvant ICE chemotherapy and focal RT provided encouraging outcomes.</jats:p></jats:sec>

Primary central nervous system sarcoma with DICER1 mutation—treatment results of a novel molecular entity in pediatric Peruvian patients

“…This strategy was associated with the best PFS and OS also in our series. The use of postoperative chemotherapy is also supported by the German series of Benesch et al 4 Moreover, response of residual lesions after surgery and the observation of extra‐CNS relapses support the use of chemotherapy 4,6 …”

“…Previously, varying treatment strategies were used in patients with CNS sarcoma (Supporting Table 3). [2][3][4][5][6][23][24][25] Most promising data derive from a Canadian series of 14 patients treated with surgery, ICE chemotherapy, and local radiotherapy. 6 This strategy was associated with the best PFS and OS also in our series.…”

“…[2][3][4][5][6][23][24][25] Most promising data derive from a Canadian series of 14 patients treated with surgery, ICE chemotherapy, and local radiotherapy. 6 This strategy was associated with the best PFS and OS also in our series. The use of postoperative chemotherapy is also supported by the German series of Benesch et al 4 Moreover, response of residual lesions after surgery and the observation of extra-CNS relapses support the use of chemotherapy.…”

“…Several chemotherapy regimens were applied, including vincristine, cyclophosphamide, and actinomycin (VAC); vincristine, cyclophosphamide, and doxorubicin; ifosfamide/doxorubicin; or ICE. Since 2018, all patients received a regimen adapted to the suggestion by Lafay‐Cousine et al 6 We usually administered 2 cycles of ICE chemotherapy (ifosfamide, 1800 mg/m 2 days 1‐5; carboplatin, 560 mg/m 2 day 1; and etoposide, 100 mg/m 2 days 1‐5) followed by focal radiotherapy without concomitant etoposide and a further 4 or 6 cycles of ICE chemotherapy, depending on toxicity and tolerability.…”

A case of primary CNS embryonal rhabdomyosarcoma with PAX3-NCOA2 fusion and systematic meta-review

“…In the WHO 2016 classi cation of CNS tumors, primary CNS rhabdomyosarcoma is characterized by histological features of undifferentiated small cells and positive immunostaining for desmin and myogenin [5]. Generally, the prognosis of primary CNS rhabdomyosarcoma is poor, although successfully treated cases have been reported [6,7].…”