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An Unusual Case of Severe Bilateral Extraocular Muscle Enlargement

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 47-year-old woman with a history of metastatic neuroendocrine carcinoid tumor (NET) status in remission after liver transplant was hospitalized for workup of a new pancreatic mass concerning for recurrence. Several days into her hospital course, she developed acute-onset severe bilateral periorbital swelling, chemosis, and ophthalmoplegia as well as occasional flushing and palpitations. Her best-corrected visual acuity was 20/70 in both eyes. Pupils, intraocular pressure, and color vision were normal. She had bilateral proptosis to 20/21 mm by Hertel exophthalmometer, −3 restriction of extraocular movements in all directions of gaze, diffuse punctate epithelial erosions, and mechanical lower lid entropion secondary to severe chemosis in both eyes (Figure 1A). Anterior segment examination and dilated ophthalmoscopy results were otherwise unremarkable.

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Recurrent NET metastatic to the EOMs

C. Biopsy the EOMs

NETs are rare neoplasms that arise in the intestine, pancreas, or lungs and can cause hormone hypersecretory syndromes. NETs most commonly metastasize to the liver, followed by bone and lung1; metastasis to the EOMs is uncommon. Most reports describe unilateral involvement; rarely is there bilateral symmetric enlargement of all muscles as seen in this patient.2,3 Patients may develop proptosis, diplopia, motility limitations, eyelid swelling, and optic disc edema.4 Treatment options include excision, exenteration, radiotherapy, hormonal therapy, and chemotherapy.5

The next best step is EOM biopsy (choice C) to confirm neoplastic involvement and eliminate alternative etiologies such as myositis or orbital inflammatory disease. Thyroid eye disease often shows tendon-sparing EOM enlargement, but given the extent of involvement and negative thyroid serologies, this diagnosis is unlikely (choice A). Furthermore, corticosteroids may affect diagnostic yield and should be avoided prior to biopsy.6 PET/CT imaging (choice B) may identify additional metastases and areas of disease activity more accessible for biopsy, but would not provide tissue diagnosis and is not the next best step. Treatment with octreotide for NET metastases (choice D) is appropriate only after disease recurrence is confirmed.

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Article Information

Corresponding Author: Katherine M. Lucarelli, MD, Department of Ophthalmology, University of Illinois at Chicago, 1855 W Taylor St, Chicago, IL 60612 (klucar2@uic.edu).

Published Online: May 5, 2022. doi:10.1001/jamaophthalmol.2022.0265

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Riihimäki  M , Hemminki  A , Sundquist  K , Sundquist  J , Hemminki  K .  The epidemiology of metastases in neuroendocrine tumors.   Int J Cancer. 2016;139(12):2679-2686. doi:10.1002/ijc.30400PubMedGoogle ScholarCrossref
2.
Mehta  JS , Abou-Rayyah  Y , Rose  GE .  Orbital carcinoid metastases.   Ophthalmology. 2006;113(3):466-472. doi:10.1016/j.ophtha.2005.10.051PubMedGoogle ScholarCrossref
3.
Fan  JT , Buettner  H , Bartley  GB , Bolling  JP .  Clinical features and treatment of seven patients with carcinoid tumor metastatic to the eye and orbit.   Am J Ophthalmol. 1995;119(2):211-218. doi:10.1016/S0002-9394(14)73875-9PubMedGoogle ScholarCrossref
4.
Matsuo  T , Ichimura  K , Tanaka  T , Takenaka  T , Nakayama  T .  Neuroendocrine tumor (carcinoid) metastatic to orbital extraocular muscle: case report and literature review.   Strabismus. 2010;18(4):123-128. doi:10.3109/09273972.2010.525779PubMedGoogle ScholarCrossref
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Kamaleshwaran  KK , Joseph  J , Upadhya  I , Shinto  AS .  Image findings of a rare case of neuroendocrine tumor metastatic to orbital extraocular muscle in Gallium-68 DOTANOC positron emission tomography/computed tomography and therapy with Lutetium-177 dotatate.   Indian J Nucl Med. 2017;32(2):125-127. doi:10.4103/0972-3919.202236PubMedGoogle ScholarCrossref
6.
Mombaerts  I , Rose  GE , Verity  DH .  Diagnosis of enlarged extraocular muscles: when and how to biopsy.   Curr Opin Ophthalmol. 2017;28(5):514-521. doi:10.1097/ICU.0000000000000395PubMedGoogle ScholarCrossref
7.
Sanli  Y , Garg  I , Kandathil  A ,  et al.  Neuroendocrine tumor diagnosis and management: 68Ga-dotatate PET/CT.   AJR Am J Roentgenol. 2018;211(2):267-277. doi:10.2214/AJR.18.19881PubMedGoogle ScholarCrossref
8.
Kim  SJ , Kim  JW , Han  SW ,  et al.  Biological characteristics and treatment outcomes of metastatic or recurrent neuroendocrine tumors: tumor grade and metastatic site are important for treatment strategy.   BMC Cancer. 2010;10:448. doi:10.1186/1471-2407-10-448PubMedGoogle ScholarCrossref
9.
Shah  MH , Goldner  WS , Benson  AB ,  et al.  Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology.   J Natl Compr Canc Netw. 2021;19(7):839-868. doi:10.6004/jnccn.2021.0032PubMedGoogle ScholarCrossref
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