|Year : 2015 | Volume
| Issue : 2 | Page : 217-219
A case report of adenoid cystic carcinoma of lacrimal gland
Rajeshwari S Vhora1, Sanjay S Vhora2, Anand D Katkar3, Prajakta S Ghate3, Vishwanath B Kamatagi3
1 Department of Emergency Medicine, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, India
2 Consultant Neurosurgeon, Chairman & Founder Director of NTU & EMS & Director of Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India
3 Resident Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India
|Date of Web Publication||13-Mar-2015|
Rajeshwari S Vhora
D3 Clover Garden, 4 Naylor Road, Off Mangaldas Road, Pune - 411 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Adenoid cystic carcinoma (ACC) is a rare malignant tumor that can exist in many different body sites. It is most commonly found in the areas of the head and neck, (mainly salivary glands). It is also reported in the breast, lacrimal glands of the eye, lung, brain, sinuses. It is characterized by a prolonged clinical course and fatal outcome. It was first described as `cylindroma' by Billroth in 1859. The long natural history and its tendency for local recurrence is well known. Patients may survive for years with metastases because this tumor is generally well differentiated and slow growing.
We report a case of ACC of lacrimal gland. They are rare malignant tumors in a patient presenting with an orbital mass. They account for 1.6% of all orbital tumors. Despite their rarity, they are the second most frequent epithelial neoplasms occurring in the lacrimal gland after pleomorphic adenomas.
Keywords: Adenoid cystic carcinoma, lacrimal gland, orbital tumour
|How to cite this article:|
Vhora RS, Vhora SS, Katkar AD, Ghate PS, Kamatagi VB. A case report of adenoid cystic carcinoma of lacrimal gland. Med J DY Patil Univ 2015;8:217-9
| Introduction|| |
Adenoid cystic carcinoma (ACC) is a malignant tumor arising from glands. In the maxillofacial region, lacrimal glands are less frequently affected. The therapy of choice has to be resection of the primary tumor with safety margins. The resection status is important for determining the local control. The resection of the related efferent lymphatics has to be included in the therapeutic concept in patients suspected of having metastasis of the regional lymph nodes. An excellent prognosis is only in ACC which is detected early and radically resected. 
This surgery can prove challenging in the head and neck region due to this tumor's tendency to spread along nerve tracts. Adjuvant or palliative radiotherapy is commonly given following surgery. In some cases, where surgery is not a reasonable option, radiation used alone may provide an effective treatment
| Case Report|| |
A 52-year-old female, known hypertensive and hypothyroid, was admitted with the complaints of swelling in the left eye. She also complained of protrusion of the left eye ball over a period of 6 months. She had diminished vision in the left eye, diplopia, left sided headache of 15 days duration. The patient gave no complaints of nasal discharge or nasal obstruction which suggested no extension through naso lacrimal duct to nose. On examination the patient had ptosis of the left eye. The visual acuity was 6/18 in the right eye unaided (as she could not wear spectacles due to proptosis and swelling) and perception of light (PL+) in the left eye. The extraocular movements were normal in the right eye but restricted in the left eye. The right fundus was normal and left showed optic atrophy. No abnormality was detected in the ear, nose and throat. The patient had significant proptosis of the left eye, which was pushed downward and outward, however proptometry could not be done [Figure 1].
MRI revealed [Figure 2] an intra-orbital growth measuring 14.7 (RL) × 28.9 (SI) × 40.5 (AP) mm causing severe mass effect on the left optic nerve. Extra-orbital component was extending intra-cranially through the orbital apex along the lateral aspect involving the lateral orbital wall, frontal, zygomatic, squamous temporal bone and greater wing of the sphenoid bone on the left side. The lateral rectus was displaced medially and involvement of the muscle fascia of the left lateral rectus was found. The lacrimal gland on the left eye appeared to be involved in the disease process. The post contrast study showed [Figure 3] enhancement of the left cavernous sinus and meningeal thickening along the left fronto-temporal convexity. The image morphology was more in favour of lymphoma.
|Figure 3: MRI left orbit T1 contrast showing intraorbital enhancing tumor with meningeal thickening and cavernous sinus enhancement|
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A lateral orbitotomy with orbital decompression, excision biopsy and excision of the involved lateral orbital and zygomatic bone was carried out. The histopathology report was suggestive of an adenocystic carcinoma (solid basaloid type) arising from the left lacrimal gland with perineural and bony invasion [Figure 4]. The patient was planned for intensive modulated radiotherapy after a planning CT and MRI, immobilization was done, the planning was done using Brain Lab Planning system. The patient was given radiotherapy through 600 CD variant linear accelerator. The dose being delivered was 54 Gy/30 fractions at l80 cGy'fr to 90% isodose level. Immediate post op. pain and swelling reduced. After removal of sutures the patient was posted for Radiation. Post radiation vision testing and MRI was planned after 6 months.
|Figure 4: Histopathology showing adenoid cystic carcinoma of lacrimal gland|
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| Discussion|| |
Adenoid cystic carcinoma is notorious for growing slowly and recurring despite therapy. Controversy remains regarding the appropriate local therapy for adenoid cystic carcinoma of the lacrimal gland. Some authorities advocate conservative surgical therapy followed by external-beam radiation therapy or proton-beam therapy. Others believe that radical surgery may result in better local control of disease and possibly better long-term survival. ,
It has a slightly higher incidence in females. The peak incidence is in 5th to 7th decade. Blood borne metastasis occurs in 40% to 60% of cases, in which lung metastasis occurs in 40% of the cases. They are slow growing tumors, which tend to spread to adjacent structures and occasionally metastasize via hematogenous spread to lungs, , brain and bone in decreasing order of frequency. ACC in general has a slow biological growth and tends to have a protracted course with a poor outcome, with a reported , survival rate of less than 50% at 5 years and 20% at l0 years. A primary ACC usually is treated with radical surgery. The rarity of lymph node metastasis suggests that radical node dissection is unnecessary. While radiation therapy has a 96% response rate, it also has a 94% incidence of subsequent local recurrence and is thus a poor treatment when used alone. Planned combinations of surgery with preoperative or more commonly postoperative radiotherapy have shown improvement in both local control and survival. The value of adjuvant chemotherapy after surgery has not been explored fully.
The clinician should have a very high index of suspicion about this lesion when a mass develops in the lacrimal fossa over a period 3 to 6 months, is accompanied by pain, causes diplopia, and on CT scanning shows a globular configuration in coronal and axial projections along with bone changes in the lacrimal region (observed in 80% of cases). Sensations in the distributions of the trigeminal nerve around the orbit should be checked, since numbness in the distribution of V-2 would suggest involvement of the cavernous sinus, and in all likelihood an inoperable lesion. The combination of MRI and CT-scanning is helpful, because posterior extension into the orbit of a lacrimal fossa lesion suggests spread through the superior orbital fissure toward the middle cranial fossa. Once the clinical suspicion is high, a biopsy should be performed through the eyelid, differentiating the various types of histologic patterns exhibited by adenoid cystic carcinoma, namely solid basaloid, basaloid with central necrosis (comedocarcinoma), sclerosing cribriform (Swiss cheese pattern), tubular, and undifferentiated or anaplastic. The prognosis differs, as basaloid or comedocarcinoma patterns have worst prognosis, whereas the tubular, the rarest, has the best prognosis; the cribriform pattern has an intermediate prognosis.
| Conclusion|| |
There is need of high index of suspicion, rapid and accurate diagnosis of adenoid cystic carcinoma, histological differentiation into subtypes, clinical or radiographic evidence of spread beyond the orbit, prompt further treatment in the form of surgery and radiation for a better outcome.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]