What is the prognosis for malignant lacrimal gland tumors?

From the Guidelines

The prognosis for malignant lacrimal gland tumors is generally poor, with overall survival rates significantly influenced by tumor size, clinical stage, histology, and treatment quality, as evidenced by studies on similar salivary gland tumors 1. When considering the prognosis for malignant lacrimal gland tumors, several key factors come into play, including:

• Tumor size and clinical stage at diagnosis, which are critical in determining the overall prognosis and guiding the therapeutic approach
• Histology, with low-grade tumors generally having better outcomes than high-grade ones
• The quality of surgical excision, highlighting the importance of complete removal of the tumor with clear margins Given the aggressive nature of these tumors and the complexity of the orbital region, early detection and aggressive multimodal treatment, including complete surgical excision and adjuvant therapies like radiation and chemotherapy, are crucial for improving survival rates. The importance of these factors is underscored by studies such as the one published in the British Journal of Cancer 1, which emphasizes the role of tumor size, clinical stage, histology, and treatment quality in determining prognosis. Regular, long-term follow-up is also essential due to the potential for late recurrences and metastases, particularly in tumors like adenoid cystic carcinoma.

From the Research

Prognosis for Malignant Lacrimal Gland Tumor

The prognosis for malignant lacrimal gland tumors varies depending on several factors, including the type and stage of the tumor, as well as the treatment approach used.

• Larger tumor size and predominant basaloid histology seem to be associated with higher risk of local-regional recurrence and distant metastasis 2.
• The traditional treatment for lacrimal gland carcinoma is orbital exenteration followed by radiation therapy, but this does not prevent distant relapse and death, and some patients experience local-regional recurrence after exenteration 2.
• More recently, eye-sparing surgery and adjuvant radiation therapy and chemotherapy have gained popularity in the treatment of lacrimal gland carcinoma, with preliminary studies showing reasonable local control rates and ocular toxicity profiles 2.
• Recent studies suggest promising results for neoadjuvant intra-arterial chemotherapy followed by orbital exenteration and adjuvant intravenous chemotherapy, but only in patients with an 'intact lacrimal artery' 2.
• Globe-sparing surgery followed by adjuvant proton radiation therapy or concurrent chemoradiation may provide good local control, reasonable rates of eye preservation, and low risk of serious ocular toxic effects 2.
• In selected patients with lacrimal gland carcinoma, eye-sparing surgery with eye-sparing radiation therapy offers preservation of visual function with good local control and minimal radiation-induced ocular toxic effects 2.
• The development of targeted drugs may offer palliation for patients with unresectable or metastatic disease in lacrimal gland carcinoma 3.
• Mortality rates for malignant lacrimal sac tumors depend on tumor stage and type, with a mean rate of 38% 4.
• Correct diagnosis and appropriate therapy require a multidisciplinary management approach, with treatment of malignant epithelial tumors being first and foremost complete surgical removal with wide excision 4.
• Lacrimal gland tumors are rare and constitute a wide spectrum of different entities ranging from benign epithelial and lymphoid lesions to high-grade carcinomas, lymphomas, and sarcomas with large differences in prognosis and clinical management 5.
• The symptoms and findings of a lacrimal gland lesion are a growing mass at the site of the lacrimal gland, including displacement of the eyeball, decreased motility, diplopia, and ptosis, with pain being the cardinal symptom of an adenoid cystic carcinoma 5.
• Radiological findings characteristically include an oval, well-demarcated mass for benign lesions whereas malignant lesions typically display calcifications, destruction of bone, and invasion of adjacent structures 5.
• The diagnosis ultimately relies on histology, as does the choice of treatment and the prognosis 5.
• Treatment of benign epithelial lesions is surgical excision whereas carcinomas often require adjuvant radiotherapy and/or chemotherapy 5.
• In contrast, the cornerstone in management of lymphoid lesions is chemotherapy, often including a monoclonal antibody 5.
• Eye-sparing surgery for adenoid cystic carcinoma leaving minimal or no tumor residual in the orbit followed by adjuvant radiation therapy or chemoradiotherapy may provide good local control and long-term survival outcomes 3.
• Intra-arterial chemotherapy has been found to decrease recurrence and improve survival in adenoid cystic carcinoma and can also be used as part of an eye-sparing treatment strategy 3.
• The adjunctive use of intra-arterial cytoreductive chemotherapy for the management of adenoid cystic carcinoma is one of the most important advancements on the management of these aggressive tumors 6.
• Carcinoma ex pleomorphic adenoma of the lacrimal gland is subclassified into noninvasive carcinoma, with an excellent prognosis after complete excision and invasive carcinoma for which the prognosis is still guarded despite adjunctive radiotherapy 6.