Neuro-Ophthalmology, 2013; 37(5): 204–208 ! Informa Healthcare USA, Inc. ISSN: 0165-8107 print / 1744-506X online DOI: 10.3109/01658107.2013.809367

C ASE REPORT

Diplopia with a Cavernous Sinus Metastasis of a Remote Endometrial Stromal Tumour Nisha X. Jain1, Michael Morgan2, Joseph Corbo3, Aseem Sharma4, and Gregory P. Van Stavern1 1

Department of Ophthalmology and Visual Sciences, 2Department of Neurology, 3Department of Pathology, and 4 Department of Radiology/Mallinckrodt Institute of Radiology, Washington University in St. Louis, School of Medicine, St. Louis, Missouri, USA

ABSTRACT Structural lesions involving the visual pathways often require tissue diagnosis to guide therapy. However, pathology is not always definitive, and a biopsy showing poorly differentiated cells poses significant difficulty in identifying the primary tumour. We report a case of metastatic disease causing cavernous sinus syndrome, in which biopsy revealed poorly differentiated tissue. The patient reported a history of a resected uterine tumour, and it was only after obtaining slides from 7 years prior that the diagnosis was made. Keywords: Cavernous sinus, cranial nerve palsies, metastasis

INTRODUCTION

commonly in the pelvis and abdomen, and less frequently in the lung and vagina.4,6 We report a case of a recurrent endometrial stromal sarcoma in the carvernous sinus, in a patient 7 years post resection of its primary origin in the uterus.

Cavernous sinus syndrome (CSS) is a rare entity that can be caused by a variety of factors, such as tumours, trauma, thrombosis, inflammation, infection, and assorted other conditions. Among the most common aetiologies are tumour, trauma, and self-limited inflammation.1,2 The tumours may be primary, extending from nearby structures, or metastatic. Signs and symptoms classically involve orbital or facial pain with ocular motor nerve palsies due to the location of cranial nerves III, IV, VI, V1, and V2 passing through the cavernous sinus. Uterine sarcomas are rare and constitute only 3% of all uterine cancers. Within the group of adult soft tissue sarcomas, they account for roughly 7% of new cases.3 Endometrial stromal sarcomas are indolent tumours and their prognosis depends largely on the grade of the tumour. Tumour behaviour is characterised by late recurrences even in patients with stage I disease; thus, long-term follow-up is required. About one third of patients develop recurrences, most

CASE REPORT

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A 54-year-old woman presented with right ear pressure and hearing loss that had developed over the course of 14 months. Two weeks prior to her visit, she noted right temporal headaches and diplopia. She was also having night sweats and low-grade fevers over several months. A head computed tomography (CT) 4 months prior showed a mass in her sphenoid sinus, but magnetic resonance imaging (MRI) 2 months later was reportedly normal. The patient states that yet another imaging test revealed a mass, and at that point was referred to our institution. Her past medical history includes hypertension and a uterine tumour resected 7 years prior to this encounter.

Received 17 March 2013; revised 18 April 2013; accepted 19 April 2013; published online 19 September 2013 Correspondence: Gregory P. Van Stavern, MD, Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, 660 S. Euclid Ave, Box 8096, St. Louis, MO 63110, USA. E-mail: [email protected]

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Neuro-ophthalmic exam, on initial presentation, was remarkable for 25% normal right abduction. The patient’s visual acuity was 20/20, with normal color plates. On external exam, the patient did not show ptosis or proptosis, and she had no resistance to retropulsion. Her pupils were round, equal and reactive to light, with no afferent pupillary defect. Alternate cover testing showed an esotropia of 15 prism diopters (PD) in primary gaze, increasing to 40–50 PD on right gaze and decreasing to 4 PD on left gaze. The remainder of the examination, including anterior segments, cranial nerves, and fundus, was normal. On follow up one month later, visual acuity continued to be 20/20, with normal color plates. External exam showed mild right sided ptosis, with

MRD1 = 3.0 mm OD, 4.0 mm OS. Levator function was reduced at 8 mm OD and normal at 16 mm OS. There was mild right enophthalmos. Motility exam showed full left ductions. She had 75% normal right abduction, and 50% normal right elevation, depression, and adduction. Alternate cover testing showed 20–25 prism diopters of exotropia and 14–16 prism diopters of right hypotropia in primary gaze. Visual field testing was fullOU. Pupils were equal with no relative afferent pupillary defect. Slit lamp exam showed reduced tear film OD and otherwise normal anterior segments OU. Fundus exam was non-remarkable. While her exam initially was significant for isolated

FIGURE 1 Head CT. Head CT bone (A) and tissue (B) windows show a sphenoid sinus mass with erosion of the wall and extension to the cavernous sinus.

FIGURE 2 Head MRI. Pre- (A) and post- (B) contrast MRI images show extension of the mass into the cavernous sinus with enhancement.

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206 N. X. Jain et al.

FIGURE 3 CT Angiogram and imaging reconstruction. CT Angiogram (A) and 3D digital reconstruction from CT (B) show right ICA aneurysm.

right abduction abnormalities, these findings now indicated right cavernous sinus involvement. Both CT and MRI show a sphenoid mass with bony erosion (Figure 1). The MRI also showed enhancing lymph nodes within the right parotid gland and in the upper cervical chain (Figure 2). Cerebral angiography showed an aneurysm in the cavernous segment of the right internal carotid artery (Figure 3). A cerebral angiogram initially showed a 5 mm  6 mm, relatively narrow neck aneurysm in the cavernous segment of the right internal carotid artery (Figure 3A). However, 4 months later it had grown to 13 mm  11 mm. The patient then underwent transsphenoidal biopsy. Pathology revealed a spindle cell sarcoma with staining positive for vimentin, CD34, and Smooth muscle actin (SMA), and negative for Human melanoma black -45 (HMB-45), S-100, p53, Epithelial membrane antigen (EMA), Progesterone receptor (PGR), desmin, Cytokeratin CAM5.2, Muscle specific actin (MSA) and pan keratin. These results were consistent with a mass of mesenchymal origin such as fibrosarcoma, leiomyosarcoma, and malignant glomangiopericytoma.5,7,9

FIGURE 4 Tumour pathology. H&E- (A) and vimentin- (B) stained sections from biopsy of the nasopharyngeal mass showing a spindle cell sarcoma.

Pathology slides obtained from the patient’s previous uterine tumour were crucial in revealing the final diagnosis. The results showed a spindle cell tumour staining positive for CD10 and B-cell leukemia/ lymphoma-2 onocogene (bcl-2) and negative for desmin, Wilms tumor gene-1 (WT-1), S-100, cytokeratins (AE1/AE3, CAM5.2, EMA, CK7, and CK19). The tumour was also less than 5% positive staining for estrogen (ER) and progesterone (PR). The mass in the sphenoid sinus was confirmed to be metastatic disease of an endometrial origin. (Figures 4, 5). With this diagnosis, the patient underwent transsphenoidal resection of the mass followed by radiation therapy. Despite efforts to control progression, 7 weeks into radiation the patient developed signs and symptoms of CN III, IV, V1, and VI involvement. Upon completion of the radiation and embolisation of the carotid aneurysm, she experienced subjective relief of her symptoms and improvement with her diplopia. Her exam showed only 4 PD esotropia on extreme right gaze. She began chemotherapy with imatinib; however, 6–7 months later she experienced recurrence of diplopia and pain. At that time she exhibited findings Neuro-Ophthalmology

Diplopia with Metastasis of Endometrial Stromal Tumour

FIGURE 5 Uterine pathology. H&E-stained section from a uterine tumour resected 7 years prior showing an endometrial stromal tumour permitting identification of the nasopharyngeal mass as a metastasis.

of CN III palsy with mild right ptosis, restricted eye movements to 50% adduction, 75% elevation, and 90% depression on the right and 20 PD exotropia at primary gaze. Her condition continued to deteriorate 8 weeks later to include severe ptosis and upper face dysthesias on her right. Her examination showed a fixed right pupil and a nearly frozen right globe, which was consistent with tumour progression visualised with MRI. Despite additional chemotherapy and radio surgery, she developed seizures, and in her right eye worsening acuity, complete ptosis, fixed pupil, and a total frozen globe. Her best acuity was 20/70 and she had a central scotoma, nasal field defect, and right relative afferent pupillary defect. Final MRI showed the tumour had progressed with extension into the right middle cranial fossa and suprasellar cistern. The patient died several months later secondary to complications of metastatic disease.

DISCUSSION We report a case of a 54-year-old woman who presented to our institution with initially an isolated 6th nerve palsy and a sphenoid/cavernous sinus mass. Medical history was unremarkable aside from hypertension and a remote history of uterine tumour, resected 7 years prior, but the nature of the prior uterine resection was not known initially. The definitive tissue diagnosis remained elusive until pathology slides from 7 years prior confirmed a diagnosis of metastatic endometrial stromal tumour to the right sphenoid sinus and cavernous sinus resulting in cavernous sinus syndrome and eventually leading to death. Uterine sarcomas are rare and constitute only 3% of all uterine cancers. Within the group of adult soft tissue sarcomas, they account for roughly 7% of new cases.3 Endometrial stromal sarcomas are indolent !

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tumours and their prognosis depends largely on the grade of the tumour. In general, most endometrial stromal sarcomas stain positively for CD10, estrogen receptor, progesterone receptor, bcl-2, and antismooth muscle antigen. These tumours are rarely stained with AE1/AE3.8 Markers such as p53 and S-100 can also be tested to determine tumour severity and prognosis. Several morphological variants exist with endometrial stromal tumours; therefore, immunohistochemistry may vary to a limited degree. Comparisons of slides from primary and metastatic disease therefore are crucial in determining the final diagnosis. The most frequent sites of metastasis for uterine sarcomas include the abdomen, lung, and bone, whereas metastasis to the brain may occur in less than 0.5% of very advanced case.10 Therefore, strong suspicion is needed to correlate an intracranial mass as a metastasis from this type of tumour. We present this case not only due to the interest of a stromal sarcoma metastasising to the sphenoid and cavernous sinus, but also to serve as a reminder to be cognizant of prior primary tumours and their potential to metastasise much later in life. Many such cases will present to the ophthalmologist or neuroophthalmologist early in the disease course. A specific tissue diagnosis is helpful in guiding therapy, and also in providing accurate prognostic information—which may spare patients unnecessary or futile treatments. Good communication between physicians across specialties was critical making the final diagnosis. If the pathology slides from 7 years prior for this patient had not been obtained, reviewed, and compared with the biopsy with our pathologists, the diagnosis may not have been made. This point is particularly important with this case given the diversity and heterozygosity of stromal sarcomas. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. This research was supported by DOVS Core Grant 5 P30 EY02687 and an unrestricted grant from Research to Prevent Blindness and NIH Core Vision Grant P30 EY02687. Note: Figures 2–5 of this article are available in colour online at www.informahealthcare.com/oph.

REFERENCES [1] Zelenak M, Doval M, Gorscak JJ, Cuscela DO. Acute cavernous sinus syndrome from metastasis of lung cancer to sphenoid bone. Case Rep Oncol 2012;5:35–42. [2] Keane JR. Cavernous sinus syndrome. Analysis of 151 cases. Arch Neurol 1996;53:967–971. [3] Reichardt P. The treatment of uterine sarcomas. Ann Oncol 2012;23:x151–x157.

208 N. X. Jain et al. [4] D’Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol 2010;116:131–139. [5] Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR. Primary uterine endometrial stromal neoplasms. A clinicopathologic study of 117 cases. Am J Surg Pathol 1990;14:415–438. [6] Huang MI, Debernardo RL, Rodgers M, Hart DJ. Endometrial stromal sarcoma metastasis to the lumbar spine and sphenoid bone. Rare Tumors 2011;3:e27. [7] Lan C, Huang X, Lin S, Cai M, Liu J. Endometrial stromal sarcoma arising from endometriosis: a clinicopathological

study and literature review. Gynecol Obstet Invest 2012;74: 288–297. DOI: 10.1159/000341706. [8] Bhargava R, Shia J, Hummer AJ, Thaler HT, Tornos C, Soslow RA. Distinction of endometrial stromal sarcomas from ‘hemangiopericytomatous’ tumors using a panel of immunohistochemical stains. Mod Pathol 2005;18:40–47. [9] Goldstein NS, Uzieblo A. Carcinomas is different from ovarian serous carcinomas. Am J Clin Pathol 2002;117: 541–545. [10] Rose PG, Piver MS, Tsukada Y, Lau T. Patterns of metastasis in uterine sarcoma. An autopsy study. Cancer 1989;63:935–938.

Neuro-Ophthalmology

Diplopia with a Cavernous Sinus Metastasis of a Remote Endometrial Stromal Tumour.

Structural lesions involving the visual pathways often require tissue diagnosis to guide therapy. However, pathology is not always definitive, and a b...
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