Patient 5 is a 61-year-old female who was diagnosed with a well differentiated G2 (Ki-67 5%) metastatic small intestine (not otherwise specified) NET in February 2010. She remained symptom free until April 2014 when she was started on monthly octreotide. In the setting of progressive disease, she subsequently received sunitinib and everolimus. She began to develop progressive diplopia and right ocular pain in August 2016. This prompted an orbital MRI which revealed bilateral recti masses in the right lateral and superior left medial muscles. She was evaluated by radiation oncology shortly thereafter and was treated with IGRT to 52Gy, which completed in October 2016. Although her right ocular pain improved, she had persistent diplopia. Her post-treatment orbital MRI in January 2017 revealed a mild increase in size of her right lateral rectus mass (Fig. a). She was then started on capecitabine in January 2017 on a two week on, one week off regimen schedule. Patient also established care with ophthalmology at this time. She achieved stable disease in her orbits with symptomatic improvement and did not demonstrate any evidence of visual field deficits. She continues capecitabine and her last MRI in July 2018 revealed ongoing shrinkage of her right lateral rectus mass (Fig. b); her recent 68Ga-DOTATATE PET/CT from October 2018 shows residual SSTR avidity within her bilateral recti muscles (Fig. c and d).
A 64-year-old woman experiencing diplopia for 6 months was found to have a right orbital tumor on MRI. Three years before onset of eye symptoms, she had a colon carcinoid tumor treated by colectomy. Visual acuity was 20/20 OD and 20/40 OS. Ocular motility of the right eye was slightly limited in up and down gaze. MRI revealed a right orbital mass infiltrating the inferior rectus muscle and the resected lesion showed intermediate to large mononuclear cells with scant cytoplasm, irregular nuclei, and finely granular chromatin on histopathology, with positive staining for chromogranin, synaptophysin, pancytokeratin, and CD10, compatible with diagnosis of neuroendocrine tumor. MRI of the chest and abdomen revealed liver and lung lesions. She died 24 months after initial evaluation due to brain metastasis.