A 62-year-old man had a neuroendocrine tumor of the rectum treated by colon resection and radiation therapy. Three years later, he experienced diplopia and right periorbital swelling and also was found to have metastasis to the liver, lung, and groin. Indium-111 OctreoScan demonstrated a mass in the right orbit. Visual acuity was no light perception in the right eye (OD) and 20/30 in the left eye (OS). There was right eye proptosis of 10 mm on exophthalmometry and decreased ocular motility in all gazes. Magnetic resonance imaging (MRI) of the orbits revealed a 30-mm well-circumscribed intraconal mass adjacent to the medial rectus muscle displacing the optic nerve medially. The lesion was isointense on T1-weighted images with moderate enhancement after contrast. Enucleation and anterior orbitotomy were performed for the painful blind eye and tumor resection, respectively. Histopathology of the resected lesion showed metastatic neuroendocrine tumor with the tumor cells staining positive for chromogranin, synaptophysin, and neuron specific enolase. At 2 years follow-up, there was no orbital recurrence of the tumor; however, there was progression of the liver, lung, and skin metastases. Despite chemotherapy (carboplatin and TP-16) and external beam radiotherapy (EBRT) of extraorbital tumors, the patient died 3 years after orbital surgery.
A 45-year-old female patient presented with weight loss, mild proptosis, and decreased vision in both the eyes. She was evaluated to have multiple lymphnodes and biopsy from mediastinal lymphnode showed metastatic neuroendocrine tumour. She was referred for whole body Ga-68 DOTANOC PET/CT for detection of primary site. Whole body PET/CT showed an intense uptake in the left lung nodule, mediastinal, and retroperitoneal lymph nodes and also uptake in the EOM lesions (arrows) []. A diagnosis of primary carcinoid lung with nodal and extrocular muscle metastasis was made and she was treated with Lu-177 DOTATATE (7.4GBq). Whole body Lu-177 images [] showed intense uptake in lymphnodes and bilateral EOM metastasis (arrows). Symptomatically, she had improvement with weight gain and normal vision in both eyes on clinical examination.