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  • Neuro-Ophthalmology/Orbit

    This study examines the clinical course of atypical pituitary adenoma with orbital invasion, a rare and aggressive condition that is often refractory to treatment.

    Study design

    The authors present a single case of pituitary adenoma invasion into the orbit, and review the disease course and treatment of this case combined with the 22 other cases reported to date.

    Outcomes

    Researchers describe the clinical features of a 31-year-old man who presented with biopsy-proven atypical pituitary adenoma invading the right orbit after a prior resection. The patient was treated with radiation, somatuline depot and temozolomide, but continued to experience recurrent tumor growth in the orbital cavity. Immunohistochemical staining of a biopsy specimen revealed high immunoreactivity. The patient experienced further vision loss, proptosis, ophthalmoplegia and cranial nerve palsies, consistent with an aggressive, refractory intracranial neoplasm.

    Among the 22 additional cases of pituitary adenomas with orbital involvement reported thus far in the literature, half have been female and patient age has ranged from 8 to 85 years, with an average age of 33 years. The most common presenting symptom was headache, whereas progressive vision loss, symptomatic proptosis, diplopia, facial swelling, weight loss and vomiting were also reported. Presenting signs have included third nerve palsy, ptosis, proptosis, afferent pupillary defect, optic disk pallor, anisocoria, optic disk edema, bitemporal hemianopia and sixth nerve palsy. Before presenting with orbital invasion, 4 cases reported previous surgical interventions that included resection of pituitary adenoma. However, many other case reports did not indicate whether the patient underwent prior surgery. With respect to adenoma subtype, 6 of the 22 cases were prolactin secreting, 4 were ACTH secreting, 2 were GH secreting, 1 was TSH secreting, 4 were nonfunctional and 5 were unspecified.

    Limitations

    This is a single case report with a review of the literature, which limits the conclusions that can be drawn.

    Clinical significance

    Atypical or aggressive pituitary adenomas with orbital invasion are uncommon but may result in serious ophthalmic deficits and system complications. Early diagnosis and management are key to reducing morbidity. This diagnosis should be considered in a patient with previous pituitary pathology, who presents with acute or progressive ocular dysfunction. Treatment and management depends on tumor subtype and histopathology indicative of aggressive behavior and increased risk of recurrence. Optimal management is not clearly defined, but surgical and medical intervention with or without adjuvant radiotherapy is the current practice.