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Show PHOTO ESSAY Bilateral Orbital Metastases from a Neuroendocrine Tumor Vasuki Sivagnanavel, BSc, MRCOphth, Paul Riordan- Eva, FRCS, FRCOphth, Jo Jarosz, MRCP, FRCR, Bernard Portmann, MD, FRCPath, and Muriel Buxton- Thomas, MSc, FRCP FIG. 1. Axial ( A) and coronal ( B) CT scans show enlargement of extraocular muscles. Coronal precontrast T- 1 MRI ( C) shows low signal intensity within the enlarged extraocular muscles with speckled areas of high signal. Coronal ( D) post-contrast T- 1 MRI shows speckled enhancement and axial postcontrast T- 1 MRI ( E) shows ovoid areas of low signal within the enhancing extraocular muscle tissue. Abstract: When neuroendocrine tumors metastasize to the orbit, they usually do so as solitary lesions, sometimes involving an extraocular muscle. A 70- year- old woman with a known neuroendocrine tumor had bilateral painful pro-ptosis, orbital soft tissue swelling, and ophthalmoplegia. Imaging showed masses within all the extraocular muscles. Orbital biopsy disclosed metastatic neuroendocrine tumor cells within the connective tissue. ( JNeuro- Ophthalmol 2004; 24: 240- 242) Departments of Ophthalmology ( VS, PRE), Neuroradiology ( JJ), and Nuclear Medicine ( MBT), and Institute of Liver Studies ( BP), King's College Hospital, London, UK. Address correspondence to Paul Riordan- Eva, FRCOphth, King's College Hospital, Department of Ophthalmology, London SE5 9RS, England; E- mail: paul. riordan- eva@ kingsch. nhs. uk A70- year- old diabetic woman presented with a two-week history of diplopia, increasingly prominent eyes, and bilateral periocular pain. Visual acuity was 20/ 30 OU with normal color vision. The eyelids and conjunctiva were injected and edematous. There was bilateral proptosis. Eye movements were limited in all directions. Orbital computed tomography showed enlargement of all extraocular muscles bilaterally, particularly the medial and inferior recti and the left superior oblique ( Figs. 1A and B). On precontrast orbital magnetic resonance imaging, the enlarged muscles had intermediate signal on Tl- weighted images with foci of higher signal. After contrast, multiple ovoid areas within the extraocular muscles enhanced less than the surrounding tissue ( Figs. 1C through E). Six months previously, the patient had presented with abdominal pain. Liver biopsy had shown deposits of a 240 J Neuro- Ophthalmol, Vol. 24, No. 3, 2004 Bilateral Orbital Metastases JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 neuro- endocrine tumor but no primary tumor could be identified. Two months before onset of the orbital symptoms, [ 1HIn] octreotide and [ 123I] meta- iodobenzylguanidine ( mlBG) scans ( Fig. 2) had shown uptake in the abdomen and in both orbits. Full- thickness biopsy of the belly of the right medial rectus muscle showed only interstitial edema and nonspecific inflammation. Adjacent orbital connective tissue showed cuboidal cells whose cytoplasm stained strongly with chromogranin ( Fig. 3) and more weakly with synapto-physin, a pattern identical to that found in the earlier liver biopsy. Orbital metastases from neuroendocrine tumors are rare, accounting for no more than 5% of orbital metastatic disease, and usually arising from gastrointestinal primary tumors whereas uveal metastases are associated with a broncho- pulmonary source ( 1). Orbital disease may be the initial manifestation of the systemic disease or become evident up to 20 years after diagnosis of the primary tumor ( 2,3). Usually the orbital metastasis consists of a unilateral solitary lesion, often involving an extraocular muscle, and resulting in proptosis, limitation of eye movements, and optic nerve compression ( 4). FIG. 3. Orbital biopsy from connective tissue shows cells that stain intensively with chromogranin, indicative of a neuroendocrine tumor. FIG. 2. ['•" In] Octreotide ( A) and [ 123l] mlBG ( B) scans show abdominal and bilateral orbital uptake. 241 JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 Sivagnanavel et al A presentation with metastases arising in both lateral recti has been reported ( 5). Acute orbital inflammation is rare but may result from a solitary deposit ( 6). Disease arising simultaneously within all the extra- ocular muscles of both orbits and presenting as bilateral orbital inflammatory disease, as our case demonstrated, has not been reported previously. The imaging findings in our case were unusual in showing heterogeneous signal on Tl- weighted sequences; previous reports have noted isointense signal on Tl- weighted sequences. ( 7,8) [ 1HIn] octreotide and [ 123I] mlBG scans identify loci of neuroendocrine tumors, including orbital metastases, but they are not specific for neoplastic lesions ( 7,9). Orbital uptake of [ H1In] octreotide occurs in active dysthyroid eye disease and to some extent in normal orbits ( 10). Physiological orbital uptake of [ 123I] mlBG does not occur. Another surprising feature of this case is that orbital biopsy disclosed tumor cells in the connective tissue but not in the medial rectus muscle, despite the striking intramuscular abnormalities on imaging. REFERENCES 1. Shetlar DJ, Font RL, Ordonez N, el- Naggar A, Boniuk M. A clini-copathologic study of three carcinoid tumors metastatic to the orbit. Immunohistochemical, ultrastructural, and DNA flow cytometric studies. Ophthalmology 1990; 97: 257- 64. 2. Harris AL, Montgomery A, Reyes RR, Gazet JC, Smith IE. Carcinoid tumour presenting as an orbital metastasis. Clin Oncol 1981: 7: 365- 72. 3. Bardenstein DS, Char DH, Jones C, Crawford JB, Miller TR, Riekhof, FT. Metastatic ciliary body carcinoid tumor. Arch Ophthal 1990; 108: 1590- 4 4. Fan JT, Buettner H, Bartley GB, Boiling JP. 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