title: High grade neuroendocrine neoplasm of the antrum and orbit.
abstract: Neuroendocrine malignancies-tumors characterized by the production of dense-core secretory granules-are most often encountered in the lungs and can also be found in extrapulmonary sites. Our patient had a primary neuroendocrine tumor of the antrum with an elusive cell of origin that secondarily invaded the inferior orbit. In the sinuses, neuroendocrine tumors may be confused with infectious sinusitis or squamous cell carcinoma. There are no known pathognomonic clinical or radiographic signs to distinguish these tumors from other conditions. Diagnosis depends on a biopsy with histopathologic and immunohistochemical analysis to identify biomarkers such as synaptophysin, chromogranin, CD56 and neuron specific enolase. Our patient's tumor defied precise immunohistochemical characterization because of its primitive character and erratic biomarker expression. The diagnosis oscillated between a neuroendocrine carcinoma and an ectopic esthesioneuroblastoma grade IV-hence the use of the more generic nosologic category of neuroendocrine neoplasm without specifying a neuronal or epithelial origin. Data to guide management are limited, particularly in the ophthalmic literature, and derive from experience with tumors of the sinonasal compartments. In the present case of a sino-orbital high grade neuroendocrine neoplasm, regional lymph node metastases developed shortly after presentation. The tumor has responded well to chemotherapy and radiation, but recurrence is often encountered within 2 years in this class of neoplasms.
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Peter W
authors.initials: PW
authors.lastname: MacIntosh
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; David G. Cogan Laboratory of Ophthalmic Pathology, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA. Electronic address: Fred_Jakobiec@meei.harvard.edu.
authors.forename: Frederick A
authors.initials: FA
authors.lastname: Jakobiec
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; David G. Cogan Laboratory of Ophthalmic Pathology, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Anna M
authors.initials: AM
authors.lastname: Stagner
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Sapideh
authors.initials: S
authors.lastname: Gilani
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Fay
mesh_terms: D000328:Adult; D018278:Carcinoma, Neuroendocrine / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D059248:Chemoradiotherapy; D005260:Female; D006801:Humans; D008444:Maxillary Sinus Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D060787:Neoplasm Grading; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed
keywords: esthesioneuroblastoma; immunohistochemistry; neuroendocrine carcinoma; orbit; orbital tumor; sinonasal carcinoma; sinusitis