FIGURE 1. Standardized A-scan echography showing (left) 7.6-mm-wide optic nerve diameter (white arrows) in primary gaze position, which decreases to 5.4 mm (white arrows) in lateral gaze position (right), pathognomonic for the presence of increased subarachnoidal fluid attributable to the presence of intracranial hypertension. REFERENCES 1. Alloway JA, Mitchell SR. Sulphasalazine neurotoxicity: a report of aseptic meningitis and a review of the literature. J Rheumatol 1993;20:409 –411. 2. Rogers AH, Rogers GL, Bremer DL, McGregor ML. Pseudotumor cerebri in children receiving recombinant human growth hormone. Ophthalmology 1999;106:1186 –1190. 3. Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998;105:1686 –1693. 4. Friedman DI. Pseudotumor cerebri. Neurosurg Clin N Am 1999;10:609 –621. FIGURE 2. B-Scan echography showing the so-called flying bat appearance (arrow) indicating increased subarachnoidal fluid attributable to intracranial hypertension. is loss of vision.2 Headaches have been reported frequently within 3 months of beginning therapy with sulfasalazine. The headaches resolve when dosage is lowered.1 Our case suggests that in some of these patient, headaches may be related to benign intracranial hypertension. Studies with large patient samples should be conducted to confirm our findings and establish the mechanism by which this drug might produce benign intracranial hypertension. We believe that the temporal relationship between the administration of mesalazine and the onset of symptoms, as well as the response to rechallenge with mesalazine, suggest a drug-related condition. The delay in diagnosis contributed to our patient’s severe vision loss, and we recommend following up these patients with serial ophthalmoscopic or echographic examinations of the optic nerves if decreased vision, headaches, or neck stiffness are present. VOL. 136, NO. 1 Spermatic Cord Leiomyosarcoma Metastatic to the Orbit Sophie J. Bakri, MD, Gregory B. Krohel, MD, George B. Peters III, MD, and Martha G. Farber, MD PURPOSE: To report a case of spermatic cord leiomyosarcoma metastatic to the orbit. DESIGN: Case report. METHODS: A 78-year-old man presented with progressive redness, proptosis, and decreasing vision of the left eye of 3 weeks’ duration. Computed tomography revealed an intraconal mass (1.5 ⴛ 2 cm) inferior to and displacing the left optic nerve. RESULTS: A left lateral orbitotomy was performed, and a Accepted for publication Dec 19, 2002. From the Department of Ophthalmology, Albany Medical College, Albany, New York. Inquiries to Gregory B. Krohel, MD, 2200 Burdett Avenue, Suite #206, Troy, New York 12180; fax: (518) 271-6394; e-mail: sbakri@nycap.rr.com BRIEF REPORTS 213 FIGURE 1. Axial 1.5-mm contrast-enhanced computed tomographic scan. An intraconal mass measuring 1.5 ⴛ 2 cm, inferior to and compressing the left optic nerve, is shown. FIGURE 2. Spindle-cell tumor stained with hematoxylin-eosin, original magnification ⴛ200. Mitotic figures are present. The insert shows the well-circumscribed nature of the tumor (hematoxylin-eosin, original magnification ⴛ20). Immunocytochemical staining confirmed the diagnosis of leiomyosarcoma. well-demarcated mass was removed from the surrounding tissue. A follow-up computed tomography of the chest and abdomen was consistent with metastatic disease to the lungs and liver. 214 AMERICAN JOURNAL Spermatic cord leiomyosarcoma is a rare cause of metastatic lesions to the orbit. (Am J Ophthalmol 2003;136:213–215. © 2003 by Elsevier Inc. All rights reserved.) CONCLUSIONS: OF OPHTHALMOLOGY JULY 2003 S PERMATIC CORD LEIOMYOSARCOMA IS A RARE MALIG- nancy.1 We are unaware of any previous reports of this lesion metastasizing to the orbit and could find no reference to it in a computer search of MEDLINE from 1966 to 2002 using the search terms spermatic cord leiomyoscarcoma and orbit. A 78-year-old man presented with a 3-week history of progressive redness, proptosis, and decreasing vision of the left eye. The patient had undergone a left radial orchiectomy 4 years previously for a testicular mass. A pathologic diagnosis of spermatic cord leiomyosarcoma was made at the Armed Forces Institute of Pathology. A metastatic evaluation at that time, including a bone scan and computed tomography (CT) of the abdomen and chest, was negative. Ocular examination revealed a best-corrected visual acuity of 20/30 in the right eye and 20/40 in the left eye. There was left upper eyelid blepharoptosis, a left afferent pupillary defect, and limitation of supraduction and infraduction in the left eye. Hertel exophthalmometry revealed 6 mm of proptosis in the left eye. Fundus examination was remarkable for optic nerve edema in the left eye. Mild cataracts were present in both eyes. A Humphrey visual field 30 –2 showed a large central scotoma in the left eye. An orbital CT scan (Figure 1) revealed an intraconal mass measuring 1.5 2 cm inferior to and displacing the left optic nerve. A preoperative chest x-ray showed multiple parenchymal nodules, and a follow-up CT of the chest was consistent with metastatic disease to both the lungs and liver. A left lateral orbitotomy was performed to decompress the optic neuropathy and to perform an excisional biopsy. At the time of surgery, the mass was found to be well demarcated from surrounding tissues and was removed in its entirety using a cryoprobe to assist the delivery. Two months postoperatively, the patient’s vision was 20/50 in the left eye. The proptosis resolved, but there was optic nerve pallor in the left eye. Gross pathology revealed a well-circumscribed oval white firm mass measuring 25 mm at its greatest diameter. Microscopic examination of the mass revealed a basophilic spindle cell tumor with one to five mitotic figures per high-power field (Figure 2). There were bundles and fascicles of spindle-shaped cells throughout the tumor and rare cellular atypia in some areas. The tumor stained VOL. 136, NO. 1 positively for vimentin and actin. Alpha-one antitrypsin stain was positive in focal areas. Stains for CD34 and CD68, desmin, S-100, and cytokeratin stains AE1/AE3 were negative. Based on this tumor morphology and immunocytochemical staining, a diagnosis of leiomyosarcoma was made. This case is 1 of only 20 reported orbital leiomyosarcomas, 3 of which occurred after radiation for retinoblastoma,2 4 of which spread from the paranasal sinuses, and 8 of which were primary orbital tumors. The primary sites of the five reported cases that metastasized to the orbit were the uterus (two cases), buttock, abdominal skin, and venous extremities.2 The most common primary sites of leiomyosarcoma are the uterus and gastrointestinal tract.3 They can usually be removed surgically because of their cohesive growth pattern and minimal infiltrative behavior. Even a few mitoses in leimyosarcomas carry a poor prognosis.4 Orbital leiomyosarcomas, in contrast, are usually friable and unencapsulated and hence may be difficult to remove entirely.5 This tumor appeared well circumscribed and was completely surgically excised. In summary, spermatic cord leiomyosarcomas can metastasize to the orbit, although this is an infrequent occurrence. Such lesions can be successfully resected. Our patient is currently undergoing chemotherapy for systemic metastases. REFERENCES 1. Puts JJ, Boerema JB, van Haelst UJ. Leiomyosarcoma of spermatic cord. Urology 1984;23:187–193. 2. Folberg R, Cleasby G, Flanagan JA, Spencer WH, Zimmerman LE. Orbital leiomyosarcoma after radiation therapy for bilateral retinoblastoma. Arch Ophthalmol 1983;101:1562– 1565. 3. Conlon MR, Rubin PA, Samy CN, Albert DM. Metastatic orbital leiomyosarcoma: a clinicopathological study. Can J Ophthalmol 1994;29:85–89. 4. Jakobiec FA, Howard GM, Rosen M, Wolff M. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol 1975;80: 1028 –1042. 5. Minkovitz JB, Dickersin GR, Dallow RL, Albert DM. Leiomyosarcoma metastatic to the orbit. Arch Ophthalmol 1990;108: 1525–1526. BRIEF REPORTS 215