Diagnostic and Therapeutic Management of Primary Orbital Neuroendocrine Tumors (NETs): Systematic Literature Review and Clinical Case Presentation
Giulia Arrivi,
Monia Specchia,
Emanuela Pilozzi,
Maria Rinzivillo,
Damiano Caruso,
Curzio Santangeli,
Daniela Prosperi,
Anna Maria Ascolese,
Francesco Panzuto,
Federica Mazzuca
Affiliations
Giulia Arrivi
Oncology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, Grottarossa Street 1035-1039, 00189 Rome, Italy
Monia Specchia
Oncology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, Grottarossa Street 1035-1039, 00189 Rome, Italy
Emanuela Pilozzi
Anatomia Patologica Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy
Maria Rinzivillo
European Neuroendocrine Tumor Society (ENETS) Center of Excellence, Digestive Disease Unit, Sant’Andrea University Hospital, 00189 Rome, Italy
Damiano Caruso
Radiology Unit, Department of Medical Surgical Sciences and Translational Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy
Curzio Santangeli
Radiology Unit, Department of Medical Surgical Sciences and Translational Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy
Daniela Prosperi
Nuclear Medicine Unit, Sant’Andrea University Hospital, 00189 Rome, Italy
Anna Maria Ascolese
Radiotherapy Oncology Unit, Department of Surgical Medical Sciences and Translational Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy
Francesco Panzuto
European Neuroendocrine Tumor Society (ENETS) Center of Excellence, Digestive Disease Unit, Sant’Andrea University Hospital, 00189 Rome, Italy
Federica Mazzuca
Oncology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea University Hospital, Sapienza University of Rome, Grottarossa Street 1035-1039, 00189 Rome, Italy
Background: The ocular involvement of neuroendocrine neoplasms (NENs) is uncommon and mainly represented by metastases from gastrointestinal and lung neuroendocrine tumors. Primary orbital NENs are even less common and their diagnostic and therapeutic management is a challenge. Methods: A systematic review of the literature was conducted from 1966 to September 2023 on PubMed to identify articles on orbital NENs and to summarize their clinical–pathological features, diagnosis and therapeutic management. Furthermore, we presented a case of a locally advanced retro-orbital primary neuroendocrine tumor that was referred to the certified Center of Excellence of Sant’Andrea Hospital, La Sapienza University of Rome, Italy. Results: The final analysis included 63 records on orbital NENs and 11 records focused on primary orbital NENs. The localization was mostly unilateral and in the right orbit; proptosis or exophthalmos represented the initial symptoms. The diagnostic work-up and therapeutic management was discussed and a diagnostic algorithm for the suspicion of primary orbital NENs was proposed. Conclusions: A multidisciplinary approach is required for the management of primary orbital NENs, emphasizing the importance of early referral to dedicated centers for prompt differential diagnosis, tailored treatment, and an improved quality of life and survival.
