Orbital tumors: a systematic review: part II

Abstract

Part II of this orbital tumor survey reviews orbital neurogenic tumors, idiopathic orbital inflammation, lacrimal gland tumors, lymphomas, rhabdomyosarcoma, granulocytic sarcoma, histiocytic tumors, secondary tumors and metastatic tumors. In the recent years, there has been a trend toward treating optic nerve sheath meningiomas with stereotactic radiotherapy at initial diagnosis. For optic gliomas demonstrating growth and aggressive behavior, BRAF inhibitors were tried but the initial results are disappointing. With regard to inflammatory orbital processes, IgG4 was found to have a causal role in certain types of idiopathic orbital inflammation and chronic inflammation of systemic sites. For lacrimal gland adenoid cystic carcinomas, recent evidence suggests that neoadjuvant cytoreductive chemotherapy decreases local tumor recurrence and improves survival. Finally, for periocular basal cell and squamous cell carcinomas demonstrating orbital invasion, targeted biologic treatment with vismodegib and cetuximab, respectively, has yielded promising results in patients who are not good candidates for extensive surgery.

Keywords:

• adenoid cystic carcinoma
• basal cell carcinoma
• benign
• dacryoadenitis
• granulocytic sarcoma
• histiocytic tumors
• idiopathic orbital inflammation
• malignant
• meningioma
• metastatic tumors
• neurofibroma
• ocular adnexal lymphoma
• optic glioma
• orbit
• pleomorphic adenocarcinoma
• pleomorphic adenoma
• rhabdomyosarcoma
• schwannoma
• secondary tumors
• squamous cell carcinoma
• tumors

Financial and competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Key issues

• In the recent years, there has been a trend toward treating optic nerve sheath meningiomas with stereotactic radiotherapy at initial diagnosis without waiting for the tumor to exhibit growth and further vision loss.

• For optic gliomas demonstrating growth and aggressive behavior, BRAF inhibitors were tried but the initial results are disappointing. Currently, chemotherapy agents are successfully used.

• IgG4 was found to have a causal role in certain types of idiopathic orbital inflammation and chronic inflammation of systemic sites. The relationship between the presence of IgG4 disease and development of subsequent orbital lymphoma still remains elusive.

• For lacrimal gland adenoid cystic carcinomas, recent evidence suggests that neoadjuvant cytoreductive chemotherapy decreases local tumor recurrence and improves survival.

• There is an increasing evidence for the role of chronic infection/antigen exposure in the development of ocular adnexal lymphomas.

• Orbital rhabdomyosarcoma patients treated with external beam radiotherapy as part of their initial treatment have lower recurrence rates compared with those who did not; however, overall survival is not adversely affected by lack of EBRT administration initially.

• Among histiocytic tumors, eosinophilic granuloma is the most frequently seen entity and many cases can be managed by surgical excision and curettage initially without chemotherapy if there is no systemic involvement.

• For periocular basal cell and squamous cell carcinomas demonstrating orbital invasion, targeted biologic treatment with vismodegib and cetuximab, respectively, have yielded promising results in patients who are not good candidates for extensive surgery.

Notes