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 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 [].