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 1 is a 72-year-old female who was initially diagnosed in 1997 with a well differentiated G3 (Ki-67 20%) metastatic jejunal NET. Her primary tumor was resected after diagnosis and a liver transplant was performed in 1998 for bulky symptomatic liver metastases. She has received a series of therapies including octreotide, lanreotide, and everolimus for her carcinoid syndrome (CS) and disease control. In May 2017, a 68Ga-DOTATATE PET-CT revealed somatostatin receptor (SSTR) avidity in bilateral orbits although she was asymptomatic from a visual symptom standpoint (Fig. a and b). In March 2018, the patient was hospitalized for preseptal cellulitis. During the hospitalization, she underwent a CT scan which revealed focal thickening of the right medial rectus and left lateral rectus muscles. Opthalmology evaluated her and felt the cellulitis was unrelated to her orbital masses. Once her infection resolved she underwent biopsy of a rectus muscles mass which confirmed orbital metastases from her NET primary. Patient did not have any worsening visual symptoms or signs (limited extraocular motility, visual field deficits or proptosis).\n177Lu-DOTATATE PRRT was initially recommended; however, this was not pursued due to the patient’s poor renal function. Radiation therapy was then considered but given the proximity of the lesions to other critical structures and her lack of symptoms, this too was deferred in favor of close surveillance. In July 2018, she started on capecitabine and temozolomide for better systemic control of her NET, which also involved her liver, pancreas, bone and intra-abdominal lymph nodes. On a follow-up orbital MRI in July 2018, her lesions were noted to be stable.