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 [].
A 64-year-old woman experiencing diplopia for 6 months was found to have a right orbital tumor on MRI. Three years before onset of eye symptoms, she had a colon carcinoid tumor treated by colectomy. Visual acuity was 20/20 OD and 20/40 OS. Ocular motility of the right eye was slightly limited in up and down gaze. MRI revealed a right orbital mass infiltrating the inferior rectus muscle and the resected lesion showed intermediate to large mononuclear cells with scant cytoplasm, irregular nuclei, and finely granular chromatin on histopathology, with positive staining for chromogranin, synaptophysin, pancytokeratin, and CD10, compatible with diagnosis of neuroendocrine tumor. MRI of the chest and abdomen revealed liver and lung lesions. She died 24 months after initial evaluation due to brain metastasis.