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Impressum
Atypical orbital leiomyoma - successful treament with Gonadotropinreleasing hormone agonists
1Gösele S., 1Dithmar S., 2Schiesser M., 3Knauth M., 1Kolling G. H., 1Völcker H. E.
1Augenklinik, 2Frauenklinik, 3Abt. Neuroradiologie, Univ.-Klinik Heidelberg
Purpose: Leiomyomas are smooth muscle tumors that are extremely
rare within the orbit. Surgical removal is the usual treatment for orbital
leiomyomas causing symptoms. This is the first report of an unusual leiomyoma
which could successfully be treated with hormone therapy.
Case report: A 66-year-old female presented with a painless diffuse
swelling of her right upper eyelid. The patient had noticed this lesion
for about three months. Her visual acuity was 20/20 OU. MR showed a tumor
of the right orbit with enhancement. The tumor could not be distinguished
from the bulbar muscles, from the sclera and the lacrimal gland. There
was no compression of the optic nerve, but the tumor surrounded the ophthalmic
vein. The enhancement continued into the retrobulbar soft tissue. There
was also a smaller tumor in the left orbit. This lesion was retrobulbar
next to the superior rectus muscle and showed exactly the same MR characteristics
as the tumor in the right orbit. A biopsy was taken from the tumor in
the right orbit and a leiomyoma was diagnosed. Because of its diffuse
growth pattern no complete surgical excision of the tumor was possible.
A therapy with a Gonadotropinreleasing hormone agonist (Goserelin, Zoladex®)
was started instead and resulted in a significant regression of the tumor.
9 months later MRT could not show any tumor in the right orbit. The tumor
of the left orbit was also indetectable. The patient underwent surgery
for a residual ptosis 3 years later. Multiple biopsies taken at that time
could not detect any residual leiomyoma tissue.
Conclusions: The here presented patient demonstrated a very unusual
expression of leiomyoma. The left orbital lesion represented probably
also leimyoma although not histologically proven. Gonadotropin-RH agonists
have been shown to reduce the circulating estrogen concentration. Goserelin
(is in use) can reduce the size of uterine leiomyomata. To our knowledge
this is the first report about the treatment of orbital leiomyoma with
Gonadotropin-RH agonists.