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A 39-year-old black man with sickle cell trait presented with a rapidly progressive, painful proptosis of the left eye. A computed tomographic scan was interpreted as revealing a cavernous hemangioma. Medial orbitotomy revealed a hard, gray mass supranasal to the optic nerve and invading the medial rectus muscle. Intraoperative frozen section specimens were read initially by the pathologist as metastatic adenocarcinoma. On gross examination, the conspicuous hemorrhage, necrosis, and bright yellow color characteristic of renal cell carcinoma were not present. Final pathologic analysis of the orbital lesion revealed metastatic renal medullary cell carcinoma. The primary lesion was located in the right kidney. Renal medullary carcinoma is a rare tumor, often affecting young individuals with sickle cell trait or disease. We report the first confirmed case of renal medullary carcinoma metastatic to the orbit with orbital symptoms preceding the diagnosis of the primary tumor.

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Summary: Pleomorphic adenoma of the lacrimal gland is a rare benign finding in adults, and extremely uncommon in children. These tumors often present with increased propotosis or hypoglobus, and they invariably require operative excision. While many of these lesions can be removed through a subcranial or transorbital exposure, larger and more posterior tumors occasionally necessitate an intracranial approach. This report describes a large lacrimal gland pleomorphic adenoma in a 16 year-old patient that required use of an expansive intracranial exposure to ensure complete excision including the capsule. We contrast this case and its management to other pediatric cases described in the literature.

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