Patient 3 is a 63-year-old female who was initially diagnosed with a well differentiated metastatic ileal NET in 1994. She was found to have G3 (Ki-67 22%) disease on later biopsies. She had stable disease controlled on octreotide until 2014 when she had disease progression which required everolimus, multiple hepatic artery embolizations and debulking of bulky adenopathy in her right paratracheal region. She underwent her first 68Ga-DOTATATE PET-CT in April 2015 which revealed bilateral extraocular masses in her right medial and left infraorbital rectus muscles. She was asymptomatic initially from these lesions; however, during her initial visit at VUMC in June 2018 for consideration of 177Lu-DOTATATE PRRT, due to persistent CS, she mentioned worsening diplopia and visual acuity. A pre-treatment 68Ga-DOTATATE PET-CT was repeated which revealed increased SSTR avidity in the recti at the site of the previously known lesions (Fig. a and b). Although she was planned for earlier, in the setting of disease-related complications, her anticipated start date for 177Lu-DOTATATE PRRT is December 2018.
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 .