Sarcomatous transformation of the orbit in a patient with Paget's disease.
OBJECTIVE
To report the clinical and pathologic features of sarcomatous transformation of the skull with involvement of the orbit, in Paget's disease.


METHODS
Interventional case report.


METHODS
An 83-year-old woman with Paget's disease who experienced progressive proptosis of the left eye.


METHODS
Fine-needle aspiration biopsy of the orbital mass.


RESULTS
Cytologic examination revealed round to oval malignant cells with wispy cytoplasm, consistent with the diagnosis of sarcoma. The patient died shortly after institution of orbital radiation therapy and systemic steroid therapy.


CONCLUSIONS
Proptosis related to Paget's disease is uncommon, and only rarely is it a result of sarcomatous transformation. Sarcomatous transformation of the skull involving the orbit should be included in the differential diagnosis of progressive proptosis in patients with Paget's disease.
Goldberg
S
S
Department of Ophthalmology and Visual Sciences, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, USA.
Slamovits
T L
TL
Dorfman
H D
HD
Rosenbaum
P S
PS
D000368:Aged; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D002471:Cell Transformation, Neoplastic; D003907:Dexamethasone / Q000627:therapeutic use; D005094:Exophthalmos / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000628:therapy; D017809:Fatal Outcome; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D010001:Osteitis Deformans / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D012516:Osteosarcoma / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed
D005938:Glucocorticoids; D003907:Dexamethasone