Malignant Orbital Tumors
Malignant orbital tumors represent a broad spectrum of tumors which include primary tumors, secondary tumors (extension from adjacent structures), and metastatic tumors. In addition, orbital inflammation and infection may clinically simulate an orbital ne
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Bhupendra C. K. Patel
Introduction
Esthesioneuroblastoma
Malignant orbital tumors represent a broad spectrum of tumors which include primary tumors, secondary tumors (extension from adjacent structures), and metastatic tumors. In addition, orbital inflammation and infection may clinically simulate an orbital neoplasm (Chap. 7). In a recent survey of 1264 consecutive patients with suspected orbital tumor referred to an ophthalmic oncology center, 36% were malignant tumors [1]. The percentage of malignant tumors increases with age, due to higher incidence of lymphoma and metastasis in the older age groups [1]. Malignant tumors of vascular (Chap. 8), neural (Chap. 11), fibrous, and osseous origin are rare in the orbit. Rhabdomyosarcoma is the most frequent primary malignant orbital tumor in children (Chap. 17), and lymphoproliferative disorders including lymphoma are most frequent in older adults (Chap. 15). Malignant orbital tumors may also arise from the lacrimal gland (Chap. 13) and lacrimal sac (Chap. 14). The details of clinical examination (Chap. 1), clinical evaluation (Chap. 2), and imaging techniques (Chap. 5) supplement contents of this review. Malignant orbital tumors not covered under other chapters are reviewed herein.
Introduction Esthesioneuroblastoma is a tumor of neural crest origin that arises from the sensory olfactory epithelium and can invade the cribriform plate, the ethmoid sinuses, and the orbit. Most esthesioneuroblastomas seen in the orbit have invaded the orbit secondarily [2]. Approximately 25% of newly diagnosed esthesioneuroblastomas will present with orbital extension. The peak incidence is in the second to third decades. They are frequently mistaken for other small-cell tumors. Males and females are equally affected and have age peaks at 20 and 50 years.
Clinical Features When confined to the nasal cavity or paranasal sinus, patients will have nasal obstruction, bloody nasal discharge, and headache. Three-quarters of patients with olfactory esthesioneuroblastoma have ophthalmic symptoms such as periorbital pain, epiphora, decreased vision, and diplopia. The most common ophthalmic signs include eyelid edema and proptosis. Ptosis and cranial nerve palsies may also be present.
B. C. K. Patel (*) Division of Facial Cosmetic and Reconstructive Surgery, University of Utah, Salt Lake City, UT, USA e-mail: [email protected] © Springer Nature Switzerland AG 2019 C. J. Hwang et al. (eds.), Clinical Ophthalmic Oncology, https://doi.org/10.1007/978-3-030-13558-4_16
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Diagnostic Evaluation CT scans show an isodense homogenous tumor in the nasal cavity and ethmoid sinus, often with orbital extension [3]. Esthesioneuroblastomas are classified according to their location: Group A tumors are confined to the nasal cavity, group B tumors affect the nasal cavity and one or more paranasal sinuses, and group C tumors extend into the cranium or orbit. Histologically, the hallmark of diagnosis is the presence of rosettes of the neuroblastoma cells, but histol
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