Treatment Options for Stages I and II
Hepatoblastoma of pure fetal histology: For tumors of pure fetal histology, complete surgical resection followed by watchful waiting or single-agent doxorubicin.In the Children's Oncology Group (COG) study COG-P9645, stage I pure fetal histology hepatoblastomas with two or fewer mitoses per 10 high power fields were not treated with chemotherapy. Completely excised tumor of purely fetal and favorable histology may be carefully followed without...
Revised text to state that the RB1 germline mutation may have been inherited from an affected progenitor or may have occurred in utero at the time of conception, in patients with sporadic disease. Also revised text to state that the penetrance of the mutation is probably dependent on concurrent genetic modifiers such as MDM2 and MDM4 (cited de Oliveira et al. as reference 5).
Added text about how early-in-life screening by fundus exams under general anesthesia at regular intervals can improve prognosis in terms of globe sparing in children with positive family histories of retinoblastoma (cited Rothschild et al. as reference 10).
The Subsequent neoplasms subsection was renamed from Second malignant neoplasms.
Revised text to state that more recent studies analyzing cohorts of patients treated with more advanced radiation planning and delivery technology have reported the incidence to be about 9.4% in nonirradiated patients and about 30.4% in irradiated patients.
Revised text to state that extraocular retinoblastoma may be confined to the tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system, bone marrow, or lymph nodes (metastatic retinoblastoma).
Intraocular Retinoblastoma Treatment
This section was extensively revised.
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