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).
Patient 2 is a 68-year-old male who was initially diagnosed with a well-differentiated G2 (Ki-67 10%) metastatic ileal NET in 2012. Post-resection of his primary, the patient began treatment with octreotide. Eventually, he enrolled on a clinical trial with 177Lu-DOTATATE PRRT. During a hospitalization in November 2017, while admitted for abdominal pain, he developed left peri-orbital swelling. This prompted an orbital MRI which demonstrated bilateral extraocular masses in his recti muscles (Fig. a). A subsequent biopsy confirmed metastatic NET. To manage his acute periorbital swelling, he was first treated with corticosteroids and later completed image-guided radiation therapy (IGRT) to 44 Gy to bilateral orbits in December 2017. The patient had a post-treatment MRI scan which demonstrated a decrease in signal abnormality and enhancement in the previously visualized lesions (Fig. b). Post-radiation, patient continued octreotide until his death in February 2018 due to progressive disease.