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 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.