A 73-year-old woman presented with drooping of her right upper lid. Twelve years prior she had undergone resection of neuroendocrine tumor in the upper lobe of her right lung and a metastasis to the ninth thoracic vertebra, which was treated with EBRT. Subsequent 5-HIAA study and Indium-111 OctreoScan were within normal limits.\nVisual acuity was 20/20 OD and 20/40 OS. There was 1 mm proptosis and 3 mm blepharoptosis on the right side. MRI of the orbit revealed a heterogeneous lesion with contrast enhancement in the superior right orbit measuring 13 × 18 × 14 mm and a smaller lesion adjacent to the lacrimal gland []. Fine needle aspiration biopsy of the orbital tumor was consistent with neuroendocrine tumor with positive staining for chromogranin, AE1/AE3, synaptophysin, and CD56 but negative staining for CD45 consistent with atypical carcinoid. Excisional biopsy revealed a low-grade non-small cell neuroendocrine tumor. Residual orbital tumor was treated with EBRT. Within the next 2–6 years, she developed recurrent lesions in both lungs and multiple metastatic lesions in the thoracic vertebra, breast, scalp, and cavernous sinus which were treated with a combination of surgical excision, chemotherapy (carboplatin, etoposide, and paclitaxel), and EBRT. Six years after initial presentation, the patient was alive with no orbital tumor recurrence [].
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.