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Bilateral reactive lymphoid hyperplasia of the orbit in a child with Russell-Silver syndrome
Yen N T Dang1, In C Shin, Robert A Gordon
1Department of Ophthalmology, Tulane University Health Science Center, New Orleans, LA 70112-2699, USA.
Insights
Russell-Silver syndrome (RSS) causes growth failure and body asymmetry. Patients with RSS have an increased risk of developing specific cancers, including Wilms tumor.
Area of Science:
- Pediatric Endocrinology
- Genetics
- Oncology
Background:
- Russell-Silver syndrome (RSS) is a rare genetic disorder characterized by intrauterine growth retardation, postnatal growth failure, and distinctive facial features.
- Key features include body asymmetry and craniofacial disproportion, impacting overall development.
Observation:
- A notable observation in RSS patients is an increased predisposition to certain malignancies.
- Previous reports have documented associations with specific tumor types.
Findings:
- Four types of malignancies have been specifically reported in individuals with Russell-Silver syndrome.
- These include craniopharyngioma, testicular seminoma, hepatocellular carcinoma, and Wilms tumor.
Implications:
- This highlights the importance of vigilant cancer surveillance in the management of Russell-Silver syndrome.
- Understanding these associations can inform personalized risk assessment and early detection strategies for affected individuals.
Abstract:
Russell-Silver syndrome (RSS) primarily includes intrauterine growth retardation, postnatal growth failure, body asymmetry, and craniofacial disproportion. Four types of malignancies have been reported in patients with RSS: craniopharyngioma, testicular seminoma, hepatocellular carcinoma, and Wilms tumor.
