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Cancer of the lacrimal gland
Last reviewed: 07.07.2025
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Lacrimal gland cancer is a rare, highly malignant tumor with a poor prognosis. According to its frequency of occurrence, it is divided into the following histological types: adenoid cystic, pleomorphic adenocarcinoma, mucoepidermoid, squamous cell.
Lacrimal gland cancer develops twice as often in women at any age, and is characterized by invasive growth already at the earliest stages of development. The tumor is characterized by significant cellular variability and grows rapidly. The history of the disease usually does not exceed 2 years, more often patients note an accelerated increase in all symptoms over several (up to 6) months. Often, the first symptoms of developing cancer are pain, discomfort, and lacrimation in the affected orbit. Uneven drooping of the upper eyelid appears early (ptosis initially develops in its outer third). The upper transitional fold becomes shallower. Exophthalmos develops with a downward and inward displacement of the eyeball, sometimes only downward. Myopic astigmatism develops as a result of mechanical deformation of the eye by the tumor. The palpable tumor is lumpy, practically does not shift in relation to the underlying tissues. Eye movements towards the tumor are limited, reposition is greatly hampered.
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Symptoms of Lacrimal Gland Cancer
Cancer of the lacrimal gland manifests itself in the 4th-6th decades of life. The anamnesis of the disease is shorter than with a benign tumor.
Pain is a sign of malignancy, but can also occur with inflammatory processes. Pleomorphic adenocarcinoma (malignant mixed-cell tumor) is represented by three main clinical variants:
- After incomplete removal of a benign pleomorphic adenoma, subsequent one or more relapses over several years and eventual malignant transformation.
- Long-standing exophthalmos (or enlargement of the upper eyelid) that suddenly begins to increase.
- Without prior history of pleomorphic adenoma as a rapidly growing mass in the lacrimal gland (usually over several months).
Signs of Lacrimal Gland Cancer
- A neoplasm in the area of the lacrimal gland that displaces the eyeball.
- Posterior extension involving the superior orbital fissure may result in conjunctival and episcleral congestion and ophthalmoplegia.
- Limitation of upward and outward eye movements (common symptom).
- Hypoesthesia of the lacrimal nerve innervation zone.
- Edema of the optic disc and choroidal folds.
Methods of studying cancer of the lacrimal gland
The diagnosis is established only after histological examination. A presumptive diagnosis can be established based on the analysis of clinical symptoms and the results of instrumental examination. Radiography reveals areas of bone destruction against the background of an enlarged orbit, most often in the upper outer, upper and outer walls of the orbit. Computed tomography allows us to determine the extent of the tumor shadow, the unevenness of its edges, the spread to the adjacent extraocular muscles and the unevenness of the contours of the bone wall of the orbit or its complete destruction, and ultrasound examination - only the presence of a tumor shadow and its density. A radioscintigram of the orbit in adenocarcinoma is characterized by an increase in the asymmetry coefficient, typical of malignant tumors. Remote thermography, especially with a sugar load, is informative. Preoperative fine-needle aspiration biopsy helps to clarify the diagnosis.
- CT reveals erosion of adjacent bone or bone ingrowth, calcifications are often visible;
- A biopsy is necessary to establish a histological diagnosis. Subsequent treatment depends on the extent of tumor invasion into adjacent structures, as determined by CT;
- A neurological examination is essential because adenoid cystic carcinoma that spreads perineurally may invade the cavernous sinus.
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Treatment of lacrimal gland cancer
Treatment of lacrimal gland cancer is a complex task. Along with the radical point of view, whose supporters demand mandatory exenteration of the orbit, there is an opinion on the possibility of organ-preserving treatment, combining local excision of the tumor with a block of adjacent healthy tissues and postoperative external irradiation of the orbit. The choice of one or another method of treatment depends on the size of the tumor and its prevalence. If preoperative detection of a violation of the integrity of the orbital bones, surgical treatment, including exenteration, is contraindicated.
- Radical removal can be performed in the form of orbital exenteration or midfacial resection. Unfortunately, it is rarely possible to completely remove the tumor tissue, which determines a poor life prognosis.
- Radiotherapy combined with local ablation can prolong life and reduce pain.
The prognosis for life and vision is poor, since the tumor is not only prone to recurrence with growth into the cranial cavity, but also to metastasis to the lungs, spine or regional lymph nodes. The time of occurrence of metastases varies from 1-2 to 20 years.