A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. Lacrimal gland tumors occur in about 1 in 1,000,000 individuals per year and constitute 525% of all orbital malignancies 2, 3, 5. Cureus primary isolated lacrimal gland amyloidosis. Adenoid cystic carcinoma of lacrimal glands radiology. The age of presentation of lacrimal gland tumors depends on the specific subtype of tumor, but overall these tumors can occur at any age. Cancer of the lacrimal gland competently about health on. Other rare causes of lacrimal gland masses include tuberculosis, amyloidosis, thyroidassociated orbitopathy, granulomatosis with polyangiitis. To the best of our knowledge, this is the first documented case of isolated lacrimal gland amyloidosis without immunoglobulin ig light chain restriction on in situ hybridization testing with a report of mri findings. Primary adenocarcinomas of the lacrimal gland rarely display sebaceous differentiation. Ct was superior to mr imaging in characterizing expansion versus erosion of the nasolacrimal bony canal. A, a 41yearold male presented with a slight fullness of the right upper eyelid arrow and displacement of the right eye developing over 6 months. The anamnesis of the disease is shorter than in a benign tumor. Feb 03, 2020 malignant tumors of the lacrimal gland include adenoid cystic carcinoma, adenocarcinoma, squamous cell carcinoma, mucoepidermoid carcinoma, and malignant lymphomas.
With a yearly incidence of one in one million, they represent only 10% of orbital spaceoccupying lesions. According to a clinical series from the wills eye hospital, lacrimal gland tumors represent almost 10% of the space occupying orbital lesions, with. Ductal adenocarcinoma ex pleomorphic adenoma of the lacrimal. Lacrimal gland tumors tumors of the lacrimal gland. Tumors of the lacrimal gland are rare in clinical practice. Overview of benign and malignant lacrimal gland tumors. The left lacrimal gland tumor was suspected, and tumorectomy was performed. Benign mixed tumors pleomorphic adenomas are the most common intrinsic lacrimal gland tumors, ac counting for approximately 50% of the epithelial tumors.
Pleomorphic adenoma of the lacrimal gland usually manifests as a slowly progressive, painless superolateral orbital mass. The symptoms and findings of a lacrimal gland lesion are. These findings were most consistent with adenoid cystic carcinoma of the lacrimal gland. Pleomorphic adenocarcinoma malignant mixedcell tumor is represented by three main clinical options. Pdf an update on tumors of the lacrimal gland researchgate.
Net includes many other sections about the medical and emotional aspects of a lacrimal gland tumor, both for the person diagnosed and. Another characteristic of adenoid cystic carcinoma is that it also invades local nerves causing pain. The main lacrimal gland is located in the upper temporal part of the orbit fig. Excision of a pleomorphic adenoma or lacrimal mass can often be completed using the anterior approach the technique is outlined below. Examination of the interface between the tumor and the globe will assist in differential diagnosis. Patients usually present between 20 and 40 years old with unilateral and painless proptosis. An overview find, read and cite all the research you need. Lacrimal gland excluding lymphoma tnm data seerrsa. Adenoid cystic carcinoma pleomorphic adenoma lacrimal gland solitary fibrous tumor original magnification these keywords were added by machine and not by the authors. And third, a patient who presents with rapid onset of symptoms, including pain, and aggressive bone destruction is found. Lacrimal gland tumor capt ayinun nahar trainee in ophthalmology cmh,dhaka 2. Lacrimal gland pleomorphic adenoma usually presents with painless gradual swelling of upper eyelid. Histopathological examination with immunostaining revealed it to be positive for igg and. If you see a test question about a tumor of the lacrimal gland, it is going to be one of those three conditions probably.
To see other pages, use the menu on the side of your screen. Inflammation of the lacrimal glands is called dacryoadenitis. In an exceptionally rare circumstance, this case showed acute orbital inflammation, and imaging findings of orbital inflammation mostly focused around the cystic space of the lacrimal gland tumor. Benign epithelial tumors include lacrimal ductal cyst dacryops and pleomorphic adenoma pa. Anterior and posterior lacrimal crests form the lacrimal sac fossa anterior medial. Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. They generally measure less than 3 cm in size and can be removed comfortably via a lateral orbitotomy approach. Carcinoma of the lacrimal gland is a rare malignancy with an estimated incidence of less than one case per million per year with no standard guidelines on optimal treatment of advanced disease. These tumors can be classified as benign epithelial, malignant epithelial, lymphoid, mesenchymal, secondarymetastatic, and tumorlike conditions. Adenoid cystic carcinoma is the most common malignant lacrimal gland tumor, comprising 50% of malignant tumors of lacrimal gland and 25% of all lacrimal gland tumors.
Second is a patient with a longstanding, indolent lacrimal gland tumor who suddenly experienced an acute expansion with pain and swelling of the upper eyelid. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Contrary to previous belief, careful biopsy of the lacrimal gland prior to excision does not appear to increase the risk of recurrence in cases with suspected pleomorphic adenoma. However, a nonreducible or tender mass merits workup for a lacrimal gland tumor or inflammatory process. Lacrimal gland involvement has also been reported in patients with sickle cell disease, which presents as lacrimal gland edema, with glands appearing enlarged and homogeneously enhancing or hyperenhancing on ct and mri.
Apr 05, 20 second is a patient with a longstanding, indolent lacrimal gland tumor who suddenly experienced an acute expansion with pain and swelling of the upper eyelid. When lacrimal gland cells change and grow uncontrollably, they form a mass called a tumor. Pleomorphic adenoma typically presents in patients with a mean age of 40 years, but have been described in children as well as in patients up to 80 years of age font et al. Lacrimal gland tumours are generally limited anteriorly, in which case the anterior approach is sufficient, but they may have mid.
Lacrimal gland tumors are rare and constitute a wide spectrum of different entities ranging from benign epithelial and lymphoid lesions to highgrade carcinomas, lymphomas, and sarcomas with large differences in prognosis and clinical management. However, no abnormality was observed on serum protein. You will read about how to cope with challenges in everyday life after a diagnosis of a lacrimal gland tumor. Adenoid cystic carcinoma cylindroma 25% if epithelial lacrimal gland tumors most common highly malignant tumor of lac gland pain from bone destruction, perineural invasion, rapid course differentiates from benign mixed. Lacrimal gland adenocarcinoma responding to checkpoint. When an adenoid cystic carcinoma of the lacrimal gland grows, it typically pushes the eye down, towards the nose and forward. Lacrimal gland tumors can be harmless or cancerous. Malignant tumors of the lacrimal gland include adenoid cystic carcinoma, adenocarcinoma, squamous cell carcinoma, mucoepidermoid carcinoma, and malignant lymphomas. Anaplastic carcinoma of the lacrimal gland presenting with. Dacryoadenitis is an inflammation of the lacrimal gland.
In any case, there are 3 major tumors that affect the lacrimal gland. Tumours of the lacrimal gland may be found in patients of all ages, but are primarily a disease of middle. The following sources were used in the development of this schema seer extent of disease 1988. Lacrimal gland is located in the superotemporal part of the orbit. Clinical presentation it often presents with orbital pain and paresthesia, since this type of tum. Keep in mind that in patients with marked herniation of fat, lacrimal gland prolapse might not be detectable except during surgical exploration. Lacrimal gland secretion is primarily under neural control, which is achieved through a neural reflex arc.
Pleomorphic adenoma of the lacrimal gland in a teenager. Lacrimal gland masses can be classified into two broad groups inflammatory 50% and neoplastic, either lymphoma 25% or salivary gland type tumors 25%. Lacrimal glandlacrimal sac is used to discriminate between lacrimal gland, staged by tnm, and lacrimal sac, not staged by tnm. From that sac, the tears drain through the lacrimal duct into the nose. This schema is based on the uicc chapter carcinoma of lacrimal gland, pages 303. A rare case of extramedullary plasmacytoma emp arising from the lacrimal gland of a 33yearold woman is presented. Adenoid cystic carcinoma adcc is the most common epithelial orbital cancer, accounting for approximately 60% of malignant lacrimal gland tumors. Lacrimal gland tumor an overview sciencedirect topics. Spontaneous rupture of lacrimal gland pleomorphic adenoma. Primary ductal adenocarcinomas of the lacrimal gland are very rare.
A case of extramedullary plasmacytoma arising from the. Pdf on jun 29, 2018, maya hada and others published lacrimal gland tumors. Lacrimal gland tumors lgt are well known neoplasm affecting both the nictitating membrane and the main lacrimal glands in dogs, however extensive histological and. He underwent orbital exenteration with lymph nodes dissection and ipsilateral parotidectomy. It was unclear whether this tumor was derived from the primary lacrimal gland or an accessory lacrimal gland. Patient a 53 yrs lady was evaluated for left eye proptosis for 6 months. Net includes many other sections about the medical and emotional aspects of a lacrimal gland tumor, both for the person diagnosed and their family members and friends. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses. Rule out lacrimal gland prolapse in blepharoplasty. The tumor typically presents with a superotemporal mass with inferonasal displacement of the globe.
Primary malignant neoplasms of the lacrimal gland british journal. An otherwise negative workup by hematologyoncology confirmed a diagnosis of primary isolated lacrimal gland amyloidosis. Sickle cell disease is reported to cause lacrimal gland edema 4. Palpation will reveal a nontender, sharply bordered, and easily reducible mass, he explained.
Adenoid cystic carcinoma of the lacrimal gland simulating. Ductal adenocarcinoma ex pleomorphic adenoma of the. Ct of the inferomedial orbit and the lacrimal drainage. It usually occurs in patients between 3040 years of age, but has been reported in children as young as 6 years old. Erosion of the orbital plate frontal bone by a benign. Other less common benign lesions also exist, as outlined in the image below. While 915% of orbital tumors arise in lacrimal gland, inflammatory or infiltrative diseases such as sarcoidosis, wegeners granulomatosis, igg4 disease, or other nonspecific dacryoadenitis comprise twothirds of lacrimal gland masses and can present with signs and symptoms similar to tumors. The following case illustrates the interdisciplinary approach to a rare tumor entity and the considerations made for preserving the orbital structures functionally. Verbist key points computed tomography and magnetic resonance imaging are in the critical pathway for evaluating the signs andor symptoms that are related to a lacrimal glandorigin neoplasm. Pediatric orbital tumors and lacrimal drainage system.
The youngest patient in our series was 16 years old. Lacrimal gland tumors are considered one of the challenging types of tumor which are difficult to study because of their rare incidence as they represent almost only 10% of the space occupying orbital lesions. Here, we present a case of a 55yearold moroccan man with lacrimal gland adenocarcinoma. Methods a retrospective multicenter cohort study of 118 cases of epithelial neoplasia was. Pain is a sign of malignancy, but can also be in inflammatory processes. The supraorbital location, encapsulation, and residual serous glands suggest that this mass was a lowgrade adenocarcinoma of the lacrimal gland. Malignant epithelial tumors of the lacrimal gland account for 2% of all orbital eye socket tumors. January human lacrimal gland gene expression vinay kumar aakalu 0 1 sowmya parameswaran 0 mark maienscheincline 0 neil bahroos 0 dhara shah 0 1 marwan ali 0 1 subramanian krishnakumar 0 0 editor. C695 lacrimal gland excluding lacrimal sac, lacrimal duct note 1. May 31, 2016 the tumor displayed cribriform, cystoid, mucinous, and basaloid features. Lowdose radiation 4 gy in two 2gy fractions appears to be effective and well tolerated in indolent nonhodgkin lymphoma of the ocular adnexa with high local control rate.
Residual glands were serous, and the majority of tumor cells were negative for mucin. Erosion of the orbital plate frontal bone by a benign tumor. Pa is the most common benign tumor of the lacrimal gland, constituting 65% of all epithelial tumors. The lacrimal gland is located under the outer part of each eyebrow. Intoduction lacrimal gland tumors are rare, representing only 518% of orbital spaceoccupying lesions they have an estimated incidence of 1 per 1 million people per year they are more prevalent in elderly. Adenoid cystic carcinoma acc of the lacrimal glands is an extraconal malignancy usually originating from the orbital lobe of the lacrimal gland. Benign mixed tumor of the palpebral love of the lacrimal gland.
An anaplastic lacrimal gland neoplasm manifesting this feature was excised from a 64yearold man who initially appeared with recurrent painless subconjunctival hemorrhages. This neoplasm shares some histological and immunohistochemical similarities with salivary duct carcinoma. Adenoid cystic carcinoma of the lacrimal gland simulating a. Pleomorphic adenoma is the most common epithelial neoplasm of the lacrimal gland. Adenoid cystic carcinoma of the lacrimal gland new york eye. Ashok kumar, wayne state university school of medicine, united states 1 lacrimal cell biology laboratory, university of illinois at chicago, department of ophthalmology and visual sciences. Noncaruncular or caruncular tumor with involvement of quadrants tumors of nonbulbar conjunctiva, nos with or without corneal extension. We found no other reports in the literature of malignant mixed tumor of the lacrimal gland in the pediatric age group. Lacrimal gland lesions a variety of neoplasms and related lesions can arise in the lacrimal gland 1,2,3,4,5,6,7,8,9,10,11,12. Malignant lacrimal gland tumors have recurrence rates of 52% to 63% and a metastasis rate of 26% to 50%. Of the malignant epithelial lesions, adenoid cystic carcinoma acc comprises approximately 60%. Imaging features of malignant lacrimal sac and nasolacrimal. The most common benign epithelial lesion in the lacrimal gland is the pleomorphic adenoma pa, also called benign mixed tumor. Orbital adenocarcinoma of lacrimal gland origin in a dog.
Globe displacement and tumorinduced hyperopia were later findings. Recent findingsdebulking of the orbital lobe of the lacrimal gland may offer improved control rates in dacryoadenitis without compromise of. Pdf lacrimal gland tumors are rare and constitute a wide spectrum of different entities ranging from benign epithelial and lymphoid lesions to. Objectiveto analyze 120 lacrimal gland masses collected during a 23year period in the ophthalmic pathology laboratory at the cullen eye institute, houston. It consists of two parts, palpebral lobe, which is visible on eversion of the eyelid and orbital lobe. On ct, the tumor typically appears as a wellcircumscribed unilateral mass that involves the orbital lobe of the lacrimal gland figs. Malignant mixed tumor of the lacrimal gland in a teenager. It secretes proteins, electrolytes and water, which helps to nourish and protect the ocular surface. Extra lacrimal masses that occur in the superolateral orbit should be considered, e. The lacrimal gland produces tears which then flow into canals that connect to the lacrimal sac.
More than 30 different subtypes were described in the 2005 who classification of tumours 4, 5. Adenoid cystic carcinoma is the most common primary malignant tumor of the lacrimal gland, but it accounts for less than 4% of all lacrimal gland lesions. Adenoid cystic carcinoma of the lacrimal gland is a malignant neoplasm that is generally found in adults and is usually managed by orbital exenteration and supplemental external beam irradiation or chemotherapy. Primary lacrimal gland tumors are of epithelial origin and are on a statistical basis, evenly divided into benign and malignant neoplasms. The word survivorship means different things to different people. These tumors can be classified as benign epithelial, malignant epithelial, lymphoid, mesenchymal, secondarymetastatic, and tumor like conditions. Computed tomography demonstrates a right lacrimal gland tumor which is confined to the orbital lobe and looks like a marble pushing on the globe arrow, a characteristic of epithelial tumors.
A recent report has suggested that the tumor may have a. An unusual presentation of lacrimal gland pleomorphic. Anatomists divide the gland into two sections, a palpebral lobe, or portion, and an orbital lobe or portion. Miscellaneous neoplastic causes of lacrimal gland masses include rhabdomyosarcoma and solitary fibrous tumor. Most cases are seen in the third decade of life with a second bimodal peak in the teenage years. Objective to apply the updated epithelial salivary gland classification scheme to a large cohort of lacrimal gland tumors so as to provide an updated lacrimal gland tumor classification scheme. Diffuse large b cell lymphoma dlbcl is the most common histologic subtype of nonhodgkin lymphoma nhl accounting for 25% of nhl cases with an incidence of 7 cases per 100 000 persons per year. Case of adenoid cystic carcinoma of lacrimal gland treated. Histopathologically myown 10 lacrimal gland tumours were distributed like the extraorbital mixed tumours. Aug 18, 2018 pa is the most common benign tumor of the lacrimal gland, constituting 65% of all epithelial tumors. Epithelial lesions of the lacrimal gland tend to be round tumors which push on the globe, like a marble or a ball fig.
Having no signs of a tumor after finishing treatment. Lacrimal gland lesions have many similarities to those that occur in the major salivary glands. The chronically expanding tumor typically produces a wellcorticated fossa in the orbital rim, but true bone destruction is rare. In most cases, tumor involved both the lacrimal sac and nasolacrimal duct at initial diagnosis. Sep 11, 2017 primary ductal adenocarcinomas of the lacrimal gland are very rare. Lacrimal gland primary epithelial tumors introduction.
Among all of them, the most common epithelial tumor is the lacrimal gland pleomorphic adenoma. Secretions of gland form the aqueous layer of the tear film. This process is experimental and the keywords may be updated as the learning algorithm improves. A lacrimal gland tumor is a tumor in one of the glands that produces tears. Cancer of the lacrimal gland is manifested in 4 6 decades of life. The lacrimal gland is an almondshaped, bilobed, eccrine gland embedded in the superolateral aspect of the orbital wall and is composed of small lobules separated by connective tissue 14.
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