Neoadjuvant/adjuvant treatment of high-risk retinoblastoma: a report from the German Retinoblastoma Referral Centre

Background Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high‐risk patients. Methods Retrospective mono‐institutional clinical, radiological, and histological review of patients with uni‐ or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed. Results Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11‐96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients—by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high‐dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5‐19.4]). Conclusion Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre‐operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Management of advanced uni‐ or bilateral retinoblastoma with macroscopic optic nerve invasion

Choucair

Brisse

Fréneaux

et al. 2019

“…Chemotherapy was defined as adjuvant chemotherapy, chemoreduction, thermochemotherapy, or any combination of these. The standard chemotherapy regimen in Germany for chemoreduction and neoadjuvant chemotherapy consisted of six cycles of chemotherapy resulting in cumulative doses of 1.2 g/m 2 carboplatin, 4.8 g/m 2 cyclophosphamide, 9 mg/m 2 vincristine, and 1.8 g/m 2 etoposide . Prior to the introduction of this chemotherapy regimen, different chemotherapy protocols were used.…”

Section: Methodsmentioning

confidence: 99%

“…The standard chemotherapy regimen in Germany for chemoreduction and neoadjuvant chemotherapy consisted of six cycles of chemotherapy resulting in cumulative doses of 1.2 g/m 2 carboplatin, 4.8 g/m 2 cyclophosphamide, 9 mg/m 2 vincristine, and 1.8 g/m 2 etoposide. 1,23 Prior to the introduction of this chemotherapy regimen, different chemotherapy protocols were used. Nearly all protocols contained alkylating agents ( Supplementary Tables S4 and S5).…”

Section: Treatment For Retinoblastomamentioning

confidence: 99%

“…Retinoblastoma is the most common eye cancer in childhood. Survival rates exceed 95% in developed countries, but late effects are a major concern for survivors. Half of retinoblastoma cases are heritable, with the child harboring an oncogenic variant of the RB1 tumor suppressor gene in the germline and developing multiple retinoblastoma foci within the first 5 years of life in most cases .…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Incidence of second cancers after radiotherapy and systemic chemotherapy in heritable retinoblastoma survivors: A report from the German reference center

Temming

Arendt

Viehmann

et al. 2016

Self Cite

111

Second cancers remain a major concern in heritable retinoblastoma survivors. Consistent with previous reports, radiotherapy increased second cancer incidence and influenced type and localization. However, chemotherapy was the strongest risk factor for second malignancies outside the periorbital region. Our results provide screening priorities during life-long oncological follow-up based on the curative therapy the patient has received and emphasize the need for less-detrimental therapies for children with heritable retinoblastoma.

Clinical features and imaging manifestations of retinoblastoma with hepatic metastasis

Wang

Huang

et al. 2021

Clinical data of five patients with hepatic metastases of retinoblastoma were analyzed retrospectively (two had bilateral tumors three had unilateral intraocular tumors). On computed tomography, multiple and single low‐density foci were observed. Four patients had tumor remission, and one showed no response after chemotherapy. Three patients who underwent enucleation were at high risk for extensive choroidal invasion. Central nervous system and bone metastases occurred in all five patients. Neuron‐specific enolase and lactate dehydrogenase levels were significantly elevated in all patients. Two patients died (not from hepatic metastasis). Three patients (one with tumor progression and two with shorter courses) are continuing treatment.