Lacrimal gland cancer

Definition

Lacrimal gland tumors are a group of neoplasms of the lacrimal gland, mainly of epithelial origin. They are localized in the outer part of the upper eyelid and are painless. Benign tumors of the lacrimal gland develop over many years and, in the process of growth, may cause exophthalmos and thinning of the ocular wall. Malignant neoplasms rapidly progress, disturb the mobility of the eyeball, provoke pain, and increase intraocular pressure. Sprouting of surrounding tissues and distant metastasis is possible. Diagnosis is made based on ophthalmologic examination and data from instrumental studies. 

General information

Lacrimal gland tumors are a group of lesions of the lacrimal gland of heterogeneous structure. They originate from glandular epithelium and are represented by epithelial and mesenchymal components. They belong to the category of mixed neoplasms. They are rare, diagnosed in 12 out of 10000 patients. They account for 5-12% of the total number of orbital tumors. The question of the degree of malignancy of such neoplasms is still debatable. Most specialists conditionally divide lacrimal gland tumors into two groups: benign and malignant, resulting from the malignization of benign neoplasms. In practice, both “clean” and transitional variants can occur. Benign processes are more often detected in women. Cancer and sarcoma are diagnosed with equal frequency in both sexes. Treatment is carried out by specialists in oncology and ophthalmology.

Benign tumors of the lacrimal glands

Pleomorphic adenoma is a mixed epithelial tumor of the lacrimal gland. It accounts for 50% of this organ’s total number of neoplasms. Women suffer more often than men. The age of patients at the time of diagnosis can vary from 17 to 70 years; the most significant number of cases (more than 70%) falls on 20-30 years. It arises from the cells of epithelial ducts. Some experts suggest that the source of the neoplasm is abnormal embryonic cells.

It is a nodule of lobular structure covered with a capsule. The tissue of the lacrimal gland tumor on the section is pink with a grayish color. It consists of two tissue components: epithelial and mesenchymal. Epithelial cells form chondral and mucus-like foci located in a heterogeneous stroma. The initial stages are characterized by very slow progression; the period from the onset of the lacrimal gland tumor to the first visit to the doctor can vary from 10 to 20 or more years. The average time interval between the onset of the first symptoms and seeking medical help is about seven years.

For some time, the lacrimal gland tumor exists without causing much inconvenience to the patient, and then its growth accelerates. Inflammatory edema appears in the eyelid area. Due to the pressure of the growing node, exophthalmos and displacement of the eye to the inside and downward develop. The upper outer part of the orbit thins. The mobility of the eye is limited. In some cases, the tumor of the lacrimal gland can reach a giant size and destroy the wall of the eye socket. A fixed, painless, dense, smooth nodule is detected on palpation of the upper eyelid.

A review radiograph of the orbit shows an increase in the size of the eye socket due to displacement and thinning of its upper outer part. Ultrasound of the eye indicates the presence of a dense nodule surrounded by a capsule. CT scan of the eye allows you to more clearly visualize the neoplasm’s boundaries and assess the capsule’s continuity and the state of the bony structures of the orbit. Treatment is operative – excision of the lacrimal gland tumor together with the capsule. The prognosis is usually favorable, but patients should remain under dispensary observation throughout their life. Recurrences can occur even several decades after the removal of the primary node. More than half of patients show signs of malignization already at the first recurrence. The shorter the remission period, the greater the likelihood of malignization of the recurrent tumor.

Malignant tumors of the lacrimal glands

Because of the rapid metastasis of lacrimal gland cancer, therapeutic interventions can be pretty lengthy. In general, several basic techniques are used:

• Surgical intervention, during which the neoplasm and adjacent areas affected by metastases are eliminated;
• Radiotherapy – necessary for effective rehabilitation to maximize the destruction of cancer cells;
• Chemotherapy – using innovative drugs (administered intravenously or orally) that improve the quality of treatment;
• Radiation therapy for lacrimal gland cancer – used after surgery;
• Gamma knife radiosurgery is used if the metastases have affected the orbital part of the eye. In this case, the tumor is removed by irradiation with a special device – an ultra-precise linear gas pedal;
• Hormone therapy is used in combination with other methods of combating the disease.

Adenocarcinoma is a mixed epithelial neoplasm with several morphologic variants and an identical clinical course. It is almost evenly distributed in different age groups and is characterized by faster growth compared to benign tumors of the lacrimal gland. Usually, patients consult an ophthalmologist a few months or 1-2 years after the appearance of symptoms. The first manifestation of the disease is often neurological pain in the forehead due to the impact of the neoplasm on the branches of the trigeminal nerve.

A typical sign of this lacrimal gland tumor is early onset and rapidly increasing exophthalmos. The eye is displaced to the inside and downward; its movements are limited. Astigmatism develops, and there is an increase in intraocular pressure. Folding appears in the area of the eye fundus. The phenomena of a stagnant disk are noted. A rapidly growing mass is detected in the upper outer corner of the eye socket. The tumor of the lacrimal gland sprouts nearby tissues, spreads deep into the orbit, penetrates the cranial cavity, and metastasizes to regional lymph nodes and distant organs.

Treatment: Exenteration of the eye socket or organ-preserving surgery combined with postoperative radiation therapy. Surgical intervention is ineffective in cases of ocular bone sprouting. The prognosis is unfavorable due to a high propensity for recurrence and distant metastasis. Lacrimal gland tumors usually metastasize to the spinal cord and lungs. The time period between the appearance of the primary tumor and the occurrence of distant metastases can range from 1-2 to 20 years. Most patients fail to meet the five-year survival threshold.

Cylindroma (adenocystic cancer) is a malignant tumor of lacrimal glands of adenomatous-alveolar structure. Clinical manifestations are similar to the symptoms of adenocarcinoma. There is less aggressive local growth, a more pronounced tendency to hematogenous metastasis, the possibility of prolonged existence of metastases, and a slow increase in the size of secondary tumors. The spinal cord is most often affected; the second place in prevalence is occupied by metastases to the lungs—tactics of treatment of adenocystic tumor of the lacrimal gland – as in adenocarcinoma. The prognosis in most cases is unfavorable. About 50% of patients die within 3-5 years after diagnosis. The cause of the lethal outcome is distant metastases or penetration of the neoplasm into the cranial cavity.

All these treatment options are available in more than 400 hospitals worldwide (https://doctor.global/results/diseases/lacrimal-gland-cancer). For example, Chemotherapy for eye cancer can be performed in following countries:

China in 3 clinics

Germany in 21 clinics

Israel in 8 clinics

Turkey in 12 clinics

United States in 8 clinics.