J Korean Ophthalmol Soc.  2019 Dec;60(12):1307-1311. 10.3341/jkos.2019.60.12.1307.

Metastatic Small Cell Neuroendocrine Carcinoma to the Orbit Manifesting as Total Ophthalmoplegia

• Lee JH
• Lee EC
• Lee HW
• Oh SY

• KMID: 2466187
• DOI: http://doi.org/10.3341/jkos.2019.60.12.1307

Abstract

PURPOSE
To report a case of primary ureteral small cell neuroendocrine carcinoma metastasis in the left orbital wall, with a review of the literature.
CASE SUMMARY
A 79-year-old male visited our clinic with a 10 day history of ocular pain, ptosis, and ophthalmoplegia in the left eye. He had been diagnosed with diabetes mellitus and was being treated for prostate adenocarcinoma. The corrected visual acuity was 0.8 in the right eye and 0.5 in the left eye. An extraocular movement test showed total ophthalmoplegia, mild exophthalmos, and ptosis in the left eye. Orbital computed tomography (CT) and magnetic resonance imaging showed an irregular mass-like enhancement in the superolateral orbital wall of the left eye, suggesting infectious and inflammatory orbital disease. After antibiotic treatment, high dose systemic steroids were administered. However, there was no improvement and the orbital CT was again performed, with no changes. A surgical biopsy for differentiating orbital tumors was performed and diagnosed as a metastatic orbital small cell neuroendocrine carcinoma. Using positron emission tomography, he was later diagnosed with a metastatic orbital small cell neuroendocrine carcinoma with multiple metastases.
CONCLUSIONS
When the patient has a mass-like lesion on imaging with ocular pain, ophthalmoplegia, or ptosis, a surgical biopsy should be performed to make a diagnosis and determine the optimal management.

Keyword

Exophthalmos; Ophthalmoplegia; Orbital metastasis; Small cell carcinoma

MeSH Terms

Adenocarcinoma
Aged
Biopsy
Carcinoma, Neuroendocrine*
Carcinoma, Small Cell
Diabetes Mellitus
Diagnosis
Exophthalmos
Humans
Magnetic Resonance Imaging
Male
Neoplasm Metastasis
Ophthalmoplegia*
Orbit*
Orbital Diseases
Positron-Emission Tomography
Prostate
Steroids
Ureter
Visual Acuity
Steroids

Figure

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  Figure 1. Clinical photograph showing severe ptosis, mild proptosis and conjunctival vessels engorgement in his left eye. −3.5 limitation of ocular movement in medial, superior and inferior gaze and −1.5 limitation of lateral gaze is noted in his left eye at initial visit.

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  Figure 2. Coronal and axial orbit computed tomography scans (axial [A] and coronal [B] views) showing irregular mass like soft tissue density of anterio temporal pole and superolateral orbital wall with well contrast enhancement and underlying bone enhancement at the left.

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  Figure 3. Brain magnetic resonance image (T2 Fluid Attenuated Inversion Recovery) showing lining pattern increased signal intensity with enhancement of dura at the left hemisphere. Axial (A) and coronal (B) views.

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  Figure 4. Microscopic examination showing infiltrating tumor cells of hyperchromatic round or oval nuclei and extremely scanty cytoplasm resembling lymphocyte in hematoxylin and eosin staining (A, ×400). The tumor cells were positive for synaptophysin (B, ×400).

Reference

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