A 63-year-old man presented with diplopia, redness, and pain of his left eye of 4 weeks duration. He had a history of neuroendocrine tumor of the testicle treated by radical orchiectomy 13 years before presentation followed by metastasis to the ileum, which was treated with bowel resection. Visual acuity was 20/30 OD and 20/25 OS and exophthalmometry revealed 4 mm proptosis of the left eye with mild limitation of ocular motility in lateral gaze. An intraconal, well-circumscribed mass in the left orbit displacing the lateral rectus muscle laterally was noted on MRI []. Histopathologic evaluation following tumor excision revealed a low-grade atypical neuroendocrine tumor with positive staining for chromogranin, synaptophysin, neuron-specific enolase, and AE1/3 compatible with carcinoid metastasis. Between the next 4.5 years, the patient had three recurrences of the orbital carcinoid which were managed with EBRT, surgical excision, and eventually orbital exenteration after the MRI showed early erosion of orbital bones by the tumor and tumor extension into the maxillary bone [Figures and ]. Histopathology of the exenterated orbital lesion showed multinucleated cells with large hyperchromatic nuclei and seven mitotic figures in 10 high-power fields consistent with aggressive metastatic poorly differentiated neuroendocrine tumor. The patient also developed signs of carcinoid syndrome with carcinoid metastasis to the liver, kidney, and parotid gland. He was alive with systemic metastasis 4 years after initial presentation.\nThe clinical and pathological features as well as treatment methods and outcomes for each patient are summarized in .
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.