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Show PHOTO ESSAY Accelerated Growth of an Orbital Schwannoma During Pregnancy Nobuo Sugo, MD, Kyousuke Yokota, MD, Masaaki Nemoto, MD, Tsutomu Hatori, MD, Toshiyuki Kano, MD, Syozo Goto, MD, and Yoshikatsu Seiki, MD FIG. 1. Three months before pregnancy, T2 axial MRI ( top left) shows a right intraconal mass of mixed signal intensity that partially enhances on postcontrast T1 axial MRI ( top right). Immediately after delivery, T2 axial MRI ( bottom left) shows a marked increase in the cystic mass with slight enhancement of its wall on T1 axial MRI ( bottom right). Abstract: An incidentally diagnosed unilateral orbital mass in a 34- year- old woman grew at an accelerated rate during pregnancy with deterioration of visual function. Removed early after delivery by a transcranial approach, the tumor was histologically diagnosed as a richly vascularized cystic schwannoma containing red blood cell components. The accelerated tumor growth was attributed to intratumoral hemorrhage. Although acoustic schwannomas have been reported to enlarge during pregnancy, this phenomenon has only been described once for orbital schwannomas. Because this type of tumor may enlarge during pregnancy and threaten visual function, surgery should not be delayed. ( J Neuro- Ophthalmol 2007; 27: 45- 47) Department of Neurosurgery ( NS, KY, NM, TK, SG, YS), Department of Pathology ( TH), Toho University Omori Medical Center, Omori- nishi, Ota- ku, Tokyo, Japan. Address correspondence to Nobuo Sugo, MD, Department of Neurosurgery, Toho University Omori Medical Center, 6- 11- 1, Omori- nishi, Ota- ku, Tokyo, Japan; E- mail: nsugo@ med. toho- u. ac. jp A 3 4- year- old woman with head trauma underwent brain CT that incidentally showed a right orbital mass. There were no skin findings suggestive of neurofibromatosis. The 24 X 14 X 13 mm mass showed high signal intensity at the periphery and irregular mixed intensity signal in the center on T2 MRI; it was hypointense on Tl MRI and displayed partial enhancement ( Fig. 1). J Neuro- Ophthalmol, Vol. 27, No. 1, 2007 45 J Neuro- Ophthalmol, Vol. 27, No. 1, 2007 Sugo et al Cerebral angiography revealed no abnormalities in the The patient had an uneventful postoperative course arterial or venous phase. The patient had normal visual with improvement of the exophthalmos and preservation acuity and a normal visual field. She had only mild of visual function. Intraocular pressures were 16 mm Hg in exophthalmos with intact extraocular movements. After the right eye and 16 mm Hg in the left eye. Visual acuity discussing the problem with the patient, we decided not had improved to 20/ 30 bilaterally. The central scotoma to operate on the tumor. Five months after the first was markedly reduced. After surgery, she developed a mild examination, she was found to be 9 weeks pregnant. At 32 right abduction deficit. The postoperative ptosis disap-weeks of pregnancy, the right exophthalmos became peared 2 months later. rapidly aggravated ( 20 mm on the right and 14 mm on the Among all orbital tumors, orbital schwannomas left), and right abduction was reduced to 70%. Visual are relatively rare ( 1- 3). There has been only one reported acuity and visual field remained normal. Intraocular case of an orbital schwannoma with rapid growth during pressures were 16 mm Hg in the right eye and 16 mm Hg pregnancy ( 4). On the other hand, there have been many in the left eye. reports of acoustic schwannomas enlarging during preg- Within days of a normal delivery at 37 weeks of nancy, resulting in the appearance and aggravation of gestation, MRI showed marked interval growth of the symptoms ( 5- 8). Whether pregnancy- related hormonal tumor to 34 X 27 X 29 mm; the fluid in the cystic mass changes are directly involved in the mechanism of had markedly increased ( Fig. 1). Nearly complete right schwannoma growth acceleration is controversial ( 4,9). ophthalmoplegia subsequently developed. Intraocular pres- Chang et al ( 4) suggested progesterone receptors as a sures were 25 mm Hg in the right eye and 16 mm Hg in the mechanism of the rapid growth of the orbital schwannoma left eye. Visual acuity had deteriorated to 20/ 200 in the in their patient. Immunohistochemical studies in our patient right eye. Right visual field examination disclosed a central showed that the tumor was negative for progesterone and scotoma. estrogen receptors, which suggests that another factor, such To preserve visual function, the orbital cystic lesion as intrarumoral hemorrhage, accounted for the growth of was removed by a microsurgical transcranial approach on the tumor. The increased vascularity of these tumors makes the 15th day after delivery. Orbital unroofing by a fronto- them more vulnerable to intrarumoral hemorrhage ( 7). temporal approach revealed a tumor mainly in the In this patient, serial MRI showed increased cystic fluid intraconal space. The fluid obtained by puncturing the accumulation, and the cystic fluid obtained during surgery cystic mass was yellowish and serous and contained red contained red blood cell components. In addition, patho-blood cell components on cytologic examination. Because logic examination showed that the wall of the tumor was the tumor was firmly adherent to the optic nerve, it was richly vascularized. These findings suggest that repeated resected subtotally It was difficult to identify the nerve intrarumoral hemorrhage resulted in tumor growth. Be-from which the tumor arose. cause orbital schwannomas developing during pregnancy The histologic diagnosis was schwannoma with threaten visual function by rapid growth, surgery should not Antoni A and B features ( Fig. 2A). The tumor was richly be delayed, vascularized and displayed scattered aggregates of vessels ( Fig. 2B). Immunohistochemical studies showed that the tumor was positive for S- 100, negative for epithelial REFERENCES membrane antigen, and negative for progesterone and L Rootman J, Goldberg C, Robertson W. Primary orbital schwarmomas. estrogen receptors. Br J Ophthalmol 1982; 66: 194- 204. 46 © 2007 Lippincott Williams & Wilkins Orbital Schwannoma J Neuro- Ophthalmol, Vol. 27, No. 1, 2007 2. Lam DS, NG JS, To KF, et al. Cystic schwannoma of the orbit. Eye 1997; 11: 798- 800. 3. Tokugawa J, Nakao Y, Mori K, et al. Orbital cystic neurinoma. Acta Neurochir ( Wien) 2003; 145: 605- 6. 4. Chang BY, Moriarty P, Cunniffe G, et al. 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