Undiscovered Islands - So Close, Yet So Far (Slides)

Identifier walsh_2020_s3_c3
Title Undiscovered Islands - So Close, Yet So Far (Slides)
Creator Magdalena Wirth; Farahna Sabiq; Mehdi Agoumi
Affiliation (MW) Department of Ophthalmology, University of British Columbia, Vancouver, Canada; (FS) Department of Radiology, University of British Columbia, Vancouver, Canada; (MA) Department of Pathology, Surrey Memorial Hospital, Surrey, Canada
Subject Metastatic Carcinoma, Orbital Tumors, Proptosis, Tumor
Description The hepatic lesions were easily amenable to ultrasound-guided biopsy and three 18-gauge core biopsies of one of the lateral right lobe hepatic masses were obtained. The histopathological sections revealed a metastatic tumor composed of monomorphic cell proliferation, with insular growth pattern (Fig. 4). Based on these features, the tumor was diagnosed as a metastatic neuroendocrine tumor, grade 2. Given the positivity for CDX2 and the upper endoscopic findings of extrinsic compression of antrum/pylorus, the patient was diagnosed with a neuroendocrine carcinoma, with primary origin being pancreatic and widely metastatic (Fig. 5). Sadly, her visitor health insurance did not cover medical expenses for chemotherapy. Thus, she was transferred back to India for further care. Orbital metastases secondary to neuroendocrine carcinoma are exceedingly rare, with only 22 cases reported so far. Average age at diagnosis is reported to be 67.5 (range 42-79). Hence, this is an extremely unusual presentation for a 30-year old patient (1-8). Das et al. found that surgical biopsy of the orbital lesion is not necessary for diagnosis in the setting of a previously established diagnosis, systemic metastases and classic radiologic findings (2). In strong contrast to other primary malignancies with orbital metastases, patients with orbital involvement from neuroendocrine tumours carry an excellent prognosis with 10-year survival ratesof close to 40% (9).
History A 30-year old female, visiting Canada from India, presented to ophthalmology with a 2-day history of right periorbital swelling, painful proptosis, fevers and chills. Initial examination suggested a clinical diagnosis of post-septal cellulitis with mild motility restriction, without optic nerve compromise, and patient was admitted and started on IV broad-spectrum antibiotics. Serologic analysis revealed mildly elevated CRP and white blood cell count. CT orbits revealed two ovoid-shaped ring-enhancing lesions in the right lateral and superior rectus muscles (Fig.1) and clear sinuses, atypical for infectious post-septal cellulitis, raising other considerations of the differential diagnosis. ACE, ANA, ANCA, HIV, and TB testing were negative. The patient received a course of oral steroids. As an inpatient, she was noted to have bradycardic episodes. Holter-monitor was unremarkable. Antibiotic and steroid treatment resulted in clinical improvement. MRI orbits was performed for further characterization, confirming the presence of the lesions in the right lateral and superior rectus muscles and an additional lesion in the left superior rectus muscle (Fig. 2). The largest ring-enhancing lesion in the right lateral rectus muscle did not show restricted diffusion, arguing against an abscess. Orbital biopsy was discussed, but deferred due to non-compliance. Given the bilaterality of these findings, a parasitic process, i.e. orbital cysticercosis was suspected, despite low eosinophil count and negative taenia solium serology. A course of albendazole, along with steroids was initiated, which led to the resolution of her symptoms. 8 weeks after her initial presentation, she presented to the ER with a 3-day history of nausea, vomiting, diarrhea, fevers and weakness. Abdominal sonography and CT ordered for iron deficiency and elevated liver enzymes showed a 3cm mass, likely arising from the neck of the pancreas, along with numerous lesions in kidneys, liver, T12 vertebral body, and periaortic/retroperitoneal lymphadenopathy (Fig. 3). A diagnostic test was performed.
Disease/Diagnosis Orbital involvement of metastatic pancreatic neuroendocrine tumor, grade 2.
Date 2020-03
References 1. Borota et al. Carcinoid tumour metastatic to the orbit with infiltration to the extraocular orbital muscle, APMIS, 113(2):135-9,2005. 2. Das et al. The eye of the beholder: orbital metastases from midgut neuroendocrine tumors, a two institution experience, Cancer Imaging, 18(1):47, 2018. 3. Matsuo et al. Neuroendocrine tumor metastatic to orbital extraocular muscle: case report and literature review. Strabismus, 18(4):123-8, 2010. 4. Peixoto et al. Neuroendocrine tumor metastatic to the orbit treated with radiotherapy. World J Gastrointest Oncol, 5(8):177-80, 2013. 5. Riddle et al. Carcinoid tumors of the eye and orbit: a clinicopathologic study of 15 cases, with histochemical and electron microscopic observations. Hum Pathol, 13(5):459-69, 1982. 6. Turaka et al. A case series of neuroendocrine tumor metastasis to the orbit. Oman J Ophthalmol, 4(3):125-8, 2011. 7. Kozubowska et al. Neuroendocrine tumour metastasis to the orbit. Endokrynol Pol, 2019. 8. Halliday et al. Pseudocystic appearance of an orbital carcinoid metastasis. Orbit, 1-4, 2019. 9. Mehta et al. Orbital carcinoid metastases. Ophthalmology, 113(3):466-72, 2006.
Language eng
Format application/pdf
Format Creation Microsoft PowerPoint
Type Text
Source 2020 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of NANOS Annual Meeting 2020
Collection Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s65n1j0m
Setname ehsl_novel_fbw
ID 1551237
Reference URL https://collections.lib.utah.edu/ark:/87278/s65n1j0m