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Concomitant Intraocular Retinoblastoma and Choroidal Hemangioma in a 1-Year-Old Boy

Yacoub A Yousef1, Maysa Al-Hussaini, Fareed H Barakat

  • 1We report a case of concomitant choroidal hemangioma in an eye that harbored intraocular retinoblastoma (RB) in a 1-year-old child. A 12-month-old boy presented with right white pupil (Leukocoria). The initial clinical diagnosis was unilateral intraocular RB Group C. The eye was treated initially by systemic chemotherapy combined with focal consolidation therapy followed by I-125 plaque radiotherapy. Massive sub-retinal recurrence was seen 4 months later, and Magnetic Resonance Imaging (MRI) showed extra-scleral invasion of the tumor, and therefore enucleation was performed. Microscopic examination revealed a differentiated retinoblastoma associated at the site of the base of the tumor with choroidal hemangioma with trans-scleral invasion. Orbit MRI was repeated 3 months after the surgery, and there was no orbital tumor recurrence. We are reporting a rare case of pathologically concomitant choroidal hemangioma with trans-scleral invasion in an eye that harbored active recurrent intraocular RB. This trans-scleral extension of hemangioma was misinterpreted by MRI as extraocular retinoblastoma and resulted in enucleation.

Turk Patoloji Dergisi
|October 7, 2017
PubMed

Insights

A rare case of retinoblastoma (RB) in a child also had a choroidal hemangioma. Misinterpretation of imaging led to enucleation due to suspected extra-scleral invasion by the hemangioma.

Area of Science:

  • Ophthalmology
  • Pediatric Oncology
  • Pathology

Background:

  • Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
  • Leukocoria is a common presenting sign of intraocular retinoblastoma.
  • Accurate staging and diagnosis are crucial for appropriate treatment and prognosis.

Observation:

  • A 1-year-old boy presented with leukocoria, initially diagnosed as unilateral intraocular retinoblastoma (Group C).
  • Treatment included chemotherapy, focal consolidation, and plaque radiotherapy, followed by recurrence and suspected extra-scleral invasion on MRI.
  • Enucleation was performed due to suspected tumor invasion.

Findings:

  • Microscopic examination revealed a differentiated retinoblastoma with an associated choroidal hemangioma at the tumor base.
  • The choroidal hemangioma exhibited trans-scleral invasion, mimicking extraocular retinoblastoma on MRI.
  • Post-enucleation MRI confirmed no orbital recurrence.

Implications:

  • This case highlights the importance of histopathological correlation when imaging findings are equivocal.
  • Choroidal hemangioma with trans-scleral extension can be misdiagnosed as extraocular retinoblastoma, potentially leading to unnecessary aggressive treatment.
  • Accurate differentiation between retinoblastoma and concomitant choroidal hemangioma is critical for optimal management and surgical planning in pediatric eye tumors.

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