Diagnostic and Therapeutic Management of Primary Orbital Neuroendocrine Tumors (NETs): Systematic Literature Review and Clinical Case Presentation.

NETs diagnosis multidisciplinary team neuroendocrine tumor orbit primary orbital treatment

Journal

Biomedicines
ISSN: 2227-9059
Titre abrégé: Biomedicines
Pays: Switzerland
ID NLM: 101691304

Informations de publication

Date de publication:
06 Feb 2024
Historique:
received: 01 12 2023
revised: 25 01 2024
accepted: 30 01 2024
medline: 24 2 2024
pubmed: 24 2 2024
entrez: 24 2 2024
Statut: epublish

Résumé

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. 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. 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. 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.

Sections du résumé

BACKGROUND 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 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 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 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.

Identifiants

DOI: 10.3390/biomedicines12020379 PMID: 38397981
pubmed: 38397981
pii: biomedicines12020379
doi: 10.3390/biomedicines12020379
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

Auteurs

Giulia Arrivi (G)

Oncology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, Grottarossa Street 1035-1039, 00189 Rome, Italy.
Department of Medical and Surgical Sciences and Translational Medicine, Faculty of Medicine and Psychology, PhD School in Translational Medicine and Oncology, Sapienza University of Rome, 00189 Rome, Italy.
ORCID: 0000-0003-0541-415X

Monia Specchia (M)

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 (E)

Anatomia Patologica Unit, Department of Clinical and Molecular Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy.
ORCID: 0000-0002-7110-9172

Maria Rinzivillo (M)

European Neuroendocrine Tumor Society (ENETS) Center of Excellence, Digestive Disease Unit, Sant'Andrea University Hospital, 00189 Rome, Italy.

Damiano Caruso (D)

Radiology Unit, Department of Medical Surgical Sciences and Translational Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy.
ORCID: 0000-0001-9285-4764

Curzio Santangeli (C)

Radiology Unit, Department of Medical Surgical Sciences and Translational Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy.

Daniela Prosperi (D)

Nuclear Medicine Unit, Sant'Andrea University Hospital, 00189 Rome, Italy.

Anna Maria Ascolese (AM)

Radiotherapy Oncology Unit, Department of Surgical Medical Sciences and Translational Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, 00189 Rome, Italy.

Francesco Panzuto (F)

European Neuroendocrine Tumor Society (ENETS) Center of Excellence, Digestive Disease Unit, Sant'Andrea University Hospital, 00189 Rome, Italy.
European Neuroendocrine Tumor Society (ENETS) Center of Excellence, Department of Medical-Surgical Sciences and Translational Medicine, Sapienza University of Rome, 00189 Rome, Italy.
ORCID: 0000-0003-2789-4289

Federica Mazzuca (F)

Oncology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, Grottarossa Street 1035-1039, 00189 Rome, Italy.

Classifications MeSH