Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases.
OBJECTIVE
To report clinical and histopathologic features and biologic behavior of orbital myxomas and angiomyxomas.


METHODS
Noncomparative retrospective case series.


METHODS
Patients with histopathologic diagnoses of orbital myxoma or angiomyxoma.


METHODS
Clinical metadata and features were obtained from the medical record. Neoplasms were studied by routine histopathology, special stains, and immunohistochemistry.


METHODS
Final diagnosis, based on histopathology, special stains, and immunohistochemistry, and clinical course from analysis of metadata and clinical features.


RESULTS
Three myxomas and 3 angiomyxomas were identified in 5 males and 1 female. Median age at presentation was 56 years (range, 4-69), with a follow-up ranging from 6 months to 8 years. Two angiomyxomas occurred in children ages 4 and 7 years whose tumors were locally aggressive and recurred. Recurrence also complicated one case of myxoma after incomplete excision. Pathologically, the tumors were poorly circumscribed. Histopathology showed them to be hypocellular, containing stellate and spindled cells in an abundant, loose, myxoid stroma rich in hyaluronic acid. Small blood vessels were rare in myxomas but abundant in angiomyxomas. Tumor cells were frequently immunoreactive for vimentin, CD34, and factor XIIIa.


CONCLUSIONS
Myxomas rarely involve orbital tissue, and no angiomyxomas of the region have been previously reported. Angiomyxomas in children may be aggressive. Vascularity and bone involvement appear to be important prognostic features for recurrence. Complete resection with a margin of healthy tissue appears to be the treatment of choice. Tumor cell immunopositivity for vimentin, CD34, and factor XIIIa may assist in the histopathologic diagnosis.
Hidayat
Ahmed A
AA
Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.
Flint
Andrew
A
Marentette
Lawrence
L
Torczynski
Elise
E
Al-Qahtani
Jerman M
JM
Ahl
Norman C
NC
Elner
Victor M
VM
D000368:Aged; D018952:Antigens, CD34 / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D026122:Factor XIIIa / Q000032:analysis; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009232:Myxoma / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D009918:Orbital Neoplasms / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014746:Vimentin / Q000032:analysis
D018952:Antigens, CD34; D014746:Vimentin; D026122:Factor XIIIa