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Clinical Challenge
June 2016

A Rapidly Enlarging Forehead Mass in a Young Retinoblastoma Survivor

Author Affiliations
  • 1Section of Otolaryngology, Department of Surgery, The University of Chicago Medicine and Biological Sciences, Chicago, Illinois
  • 2Department of Pathology, The University of Chicago Medicine and Biological Sciences, Chicago, Illinois
JAMA Otolaryngol Head Neck Surg. 2016;142(6):597-598. doi:10.1001/jamaoto.2015.3704

A man in his 20s had a lump on the right side of his forehead. His medical history was notable for bilateral retinoblastoma when he was an infant, which was treated with right-eye enucleation and left-eye irradiation. As a teenager, he developed an advanced malignant small blue cell tumor, which was interpreted as an olfactory neuroblastoma and treated with radiation and chemotherapy. On examination, the patient had a hard, fixed, rounded 2-cm lesion in the right supraorbital region. Computed tomography (CT) showed an irregular mass in the right frontal, maxillary, and ethmoid sinuses with surrounding sclerotic and erosive bony changes. A fine-needle aspirate (FNA) revealed atypical, mitotically active cells. Bone shavings obtained operatively were interpreted as chronic sinusitis with sclerotic bone. The surgical specimen showed multinucleated giant cells and bizarre stromal cells suggestive of treatment effect (Figure, A) and normal-appearing bone. A second opinion was reported as chronic sinusitis and atypical stromal cells. When the patient returned 2 months later with increasing pain, endoscopy revealed a polypoid lesion occupying the biopsied site. On repeated operative biopsy, histologic examination showed similar giant cells and atypical stromal cells (Figure, B). However, other areas demonstrated osteoid formation with fine calcifications and severely atypical spindle cells with mitotic figures (Figure, C), as well as osteoid-entrapped, malignant-appearing cells (Figure, D).

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