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Background <p>Pediatric orbital masses are uncommon and most reports originate from ophthalmology services, where benign lesions predominate; oncology-referral populations may differ and remain underreported.</p> Methods <p>We reviewed children evaluated for proptosis and orbital mass at a tertiary pediatric oncology unit (2000–2020). Demographic features, diagnostic spectrum, tumor origin, age distribution, diagnostic approach, and survival outcomes were analyzed. Overall survival (OS) was estimated by Kaplan–Meier analysis and compared by log-rank testing. Prespecified exploratory comparisons included age (&lt; 5 vs. ≥ 5&#xa0;years), diagnostic approach, and metastatic neuroblastoma versus other malignant orbital tumors. A univariable Cox model explored the association between metastatic neuroblastoma and OS.</p> Results <p>Sixty patients were included (65% male), with median age of 4.74&#xa0;years (IQR, 2.16–8.84&#xa0;years). Malignant tumors comprised 88% (<i>n</i> = 53); benign lesions were 12% (<i>n</i> = 7) and mainly vascular. The most frequent diagnoses were metastatic neuroblastoma and optic pathway glioma (each 21.7%), followed by non-Hodgkin lymphoma and rhabdomyosarcoma (each 13.3%). Histopathology confirmed 85% (<i>n</i> = 51), whereas 15% (<i>n</i> = 9) were diagnosed radiologically, predominantly optic pathway gliomas. Diagnosis differed by age (&lt; 5 vs. ≥ 5&#xa0;years; <i>p</i> = 0.009), with metastatic neuroblastoma concentrated in children younger than 5&#xa0;years (92.3%). Nine deaths occurred, all in malignant tumors. Metastatic neuroblastoma was associated with inferior overall survival and higher mortality risk than other orbital tumors.</p> Conclusions <p>Pediatric orbital masses referred to oncology centers represent a high-risk population with a high malignancy rate. Rapidly progressive proptosis, particularly in children &lt; 5&#xa0;years, should prompt urgent evaluation for metastatic neuroblastoma.</p>

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Pediatric orbital masses from a tertiary pediatric oncology referral center: clinical characteristics and survival outcomes from a 20-year single-center experience

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月28日

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  • Mehtap Ertekin,
  • Aytul Temuroglu,
  • Gokalp Rustem Aksoy

摘要

Background

Pediatric orbital masses are uncommon and most reports originate from ophthalmology services, where benign lesions predominate; oncology-referral populations may differ and remain underreported.

Methods

We reviewed children evaluated for proptosis and orbital mass at a tertiary pediatric oncology unit (2000–2020). Demographic features, diagnostic spectrum, tumor origin, age distribution, diagnostic approach, and survival outcomes were analyzed. Overall survival (OS) was estimated by Kaplan–Meier analysis and compared by log-rank testing. Prespecified exploratory comparisons included age (< 5 vs. ≥ 5 years), diagnostic approach, and metastatic neuroblastoma versus other malignant orbital tumors. A univariable Cox model explored the association between metastatic neuroblastoma and OS.

Results

Sixty patients were included (65% male), with median age of 4.74 years (IQR, 2.16–8.84 years). Malignant tumors comprised 88% (n = 53); benign lesions were 12% (n = 7) and mainly vascular. The most frequent diagnoses were metastatic neuroblastoma and optic pathway glioma (each 21.7%), followed by non-Hodgkin lymphoma and rhabdomyosarcoma (each 13.3%). Histopathology confirmed 85% (n = 51), whereas 15% (n = 9) were diagnosed radiologically, predominantly optic pathway gliomas. Diagnosis differed by age (< 5 vs. ≥ 5 years; p = 0.009), with metastatic neuroblastoma concentrated in children younger than 5 years (92.3%). Nine deaths occurred, all in malignant tumors. Metastatic neuroblastoma was associated with inferior overall survival and higher mortality risk than other orbital tumors.

Conclusions

Pediatric orbital masses referred to oncology centers represent a high-risk population with a high malignancy rate. Rapidly progressive proptosis, particularly in children < 5 years, should prompt urgent evaluation for metastatic neuroblastoma.