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Keywords:

  • lacrimal gland;
  • lacrimal duct;
  • lacrimal sac;
  • radiation therapy;
  • ophthalmology

Abstract

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES

BACKGROUND:

Malignant epithelial neoplasms of the lacrimal apparatus are rare and are typically treated with surgery and occasionally adjuvant radiation therapy (RT). The purpose of this study was to assess treatment outcomes by type of surgery (orbital exenteration vs eye-sparing surgery) and clarify the role of adjuvant RT for this rare disease.

METHODS:

Forty-six patients with malignant epithelial neoplasms of the lacrimal apparatus were treated at a single institution from 1945 through 2008. Twenty-seven patients (59%) were treated with orbital exenteration and 19 (41%) with eye-sparing surgery; 64% of the orbital exenteration group and 83% of the eye-sparing surgery group also received adjuvant RT (median dose, 60 grays). Median follow-up time for all patients was 38 months (range, 3-460 months).

RESULTS:

For the orbital exenteration and eye-sparing surgery groups, the 5-year overall survival (OS) rates were 59% and 62%, and the 5-year disease-free survival (DFS) rates were 49% and 39%, respectively (P = .56, P = .35). Tumor status (T1-2 vs T3-4) was associated with OS (P = .02), and tumor size (<3.5 vs >3.5 cm) with DFS (P = .015). Median time to locoregional recurrence was 85 months for orbital exenteration, and 123 months for eye-sparing surgery. All patients who did not receive RT experienced local recurrence, and RT extended time to locoregional recurrence (median 460 vs 30 months, P = .009). Seven grade ≥3 complications were experienced after adjuvant RT.

CONCLUSIONS:

For appropriately selected patients, an eye-sparing surgery for lacrimal apparatus tumors can achieve similar survival outcomes to those in patients treated with an orbital exenteration. Adjuvant RT should be considered for all patients presenting with these rare tumors. Cancer 2011. © 2011 American Cancer Society.

Primary epithelial malignancies of the lacrimal apparatus, comprising the lacrimal gland, duct, and sac, are extraordinarily rare tumors, with an estimated yearly incidence of 0.072 per 100,000 people.1 Common presenting symptoms include epiphora, palpable mass, change in visual acuity, and eye pain and can easily be attributed to a more benign etiology. Thus, delays in diagnosis are common in many cases. The most common tumor histology is adenoid cystic carcinoma, particularly for tumors in the lacrimal gland; however, other histologic types include carcinoma ex pleomorphic adenoma and adenocarcinoma. Five-year overall survival (OS) rates for individuals with epithelial lacrimal apparatus tumors vary from 40% to 80% depending upon the study reviewed, the tumor histology, and the site of disease.2-11 Furthermore, because of the rarity of this disease, current clinical practice is based largely on retrospective case series, and the most appropriate management strategy remains controversial.

Traditionally, lacrimal apparatus malignancies have been treated by surgical resection sometimes followed by adjuvant radiotherapy, with local control rates of approximately 50% at 5 years.4, 7-9, 12 In 1 retrospective analysis, Wright and colleagues suggested that adding radiotherapy to local resection for adenoid cystic carcinoma of the lacrimal gland delayed tumor recurrence and may have extended survival,6 but the number of patients in that study was small. More recent findings suggest that extensive surgical resection can improve local control, at least for patients with adenoid cystic carcinoma of the lacrimal gland.2 Brada and Henk reported a series of 33 patients with primary malignant lacrimal gland tumors that suggested a trend toward improved local control with the inclusion of postoperative radiotherapy to at least 60 grays (Gy).12

The relatively poor prognosis of malignant tumors of the lacrimal apparatus has led several groups to add chemotherapy to the treatment regimen.11, 13, 14 One approach has used intra-arterial platinum-based chemotherapy to decrease the size of the primary tumor before surgical resection and postoperative radiotherapy.11, 14 In 1 such study, Tse et al compared the outcomes of 9 patients treated with intra-arterial chemotherapy followed by extensive surgical resection and adjuvant chemoradiotherapy with those of 7 patients treated at the same institution with resection with or without postoperative radiotherapy.11 Local control and disease-free survival (DFS) were improved in the group given intra-arterial chemotherapy. However, this experience has yet to be validated in a larger group of patients.

Available data specifically regarding malignant tumors of the lacrimal sac and duct, as opposed to the lacrimal gland, are even more scant. The treatment of these lesions again has usually involved surgical resection and sometimes adjuvant radiotherapy.7-10, 15, 16 Both local control and survival rates are variable, but seem to be similar to those seen for patients with the same histologic types of tumor in the lacrimal gland. Given the limited data available regarding the appropriate management of malignant tumors of the lacrimal apparatus, we reviewed all cases treated at The University of Texas MD Anderson Cancer Center to determine the outcomes of treatment and to better characterize the most effective therapy for these rare tumors.

MATERIALS AND METHODS

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES

We identified and reviewed the medical records of 46 patients diagnosed with primary epithelial malignancies of the lacrimal apparatus treated with curative intent at The University of Texas MD Anderson Cancer Center between 1945 and 2008. Patients with distant metastases at diagnosis were excluded, as were those with lymphoma or melanoma of the lacrimal gland or sac. Lacrimal gland tumors were staged according to the seventh (2010) edition of the American Joint Committee on Cancer (AJCC) staging manual.17 Because no staging system has been established for tumors of the lacrimal sac, these tumors were classified in a similar manner to those of the lacrimal gland for analysis. The most common tumor classification was T4, and all but 1 patient had lymph node-negative disease. The most common tumor histology, present in nearly half of the patients, was adenoid cystic carcinoma.

Typically, the treatment field encompassed the postoperative bed only, with no prophylactic nodal coverage. However, 4 patients with tumors of nonadenoid cystic pathology underwent irradiation of the ipsilateral parotid gland and cervical lymph nodes, including the 1 patient who presented with nodal metastasis. Before 2002, radiation was delivered by conventional techniques, most often with 6 MV photons or appositional electron fields, but occasionally with helium ion therapy (1 patient) or orthovoltage radiotherapy (1 patient). Since 2002, 14 patients were treated with 6 MV intensity-modulated radiation therapy (IMRT), and 1 patient was treated with proton radiotherapy.

The primary outcomes assessed were OS, DFS, distant metastasis-free survival, and locoregional control (LRC). DFS was defined as freedom from disease at any site, whereas LRC was defined as freedom from disease in the orbit, postoperative bed, or ipsilateral lymphatics. Visual acuity was also recorded from clinical examinations performed on follow-up. Snellen fractions were normalized to 20/20 and grouped by time after treatment. Differences between groups were compared with the chi-square statistic. The Kaplan-Meier method was used to determine the probabilities of LRC, DFS, and OS, with groups compared by using log-rank statistics. Multivariate analysis was performed using forward stepwise Cox regression. Only 2 variables were used in any multivariate analysis secondary to low numbers of patients per group. P values <.05 were considered significant. SPSS software (version 16.0; SPSS Inc., Chicago, Ill) was used for the statistical analyses.

RESULTS

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES

Patient Population

Characteristics of the study population and each surgical group are presented in Table 1. Treatment characteristics are shown in Table 2. Specifically, equal numbers of men and women were treated, and the median age at diagnosis was 46.5 years. Thirty-three (72%) patients were treated for a primary tumor of the lacrimal gland, and 13 (28%) patients were treated for a primary tumor of the lacrimal sac or duct.

Table 1. Patient Characteristics
Number of patientsAll PatientsOEESSP
No.%No.%No.%
  1. OE indicates orbital exenteration; ESS, eye-sparing surgery; SD, standard deviation.

  2. Percentages are calculated by row. P values represent comparisons between OE and ESS for each patient characteristic.

Age at diagnosis, median y (±SD)46.5 (±15.36)      
Follow-up time, median mo (range)38 (3-460)      
Sex
 Male231001565.2834.8.37
 Female231001252.21147.8 
Race
 White351002365.71234.3.25
 Black2100150150 
 Hispanic3100266.7133.3 
 Asian5100120480 
 Other1100001100 
Date of diagnosis
 1945-19552100150150 
 1956-19652100002100 
 1966-19758100675225 
 1976-19855100120480 
 1986-19954100250250 
 1995-2005161001381.3318.8 
 2005-20089100444.4555.6 
Tumor laterality
 Right231001565.2834.8.37
 Left231001252.21147.8 
Site
 Lacrimal gland3371.72060.61339.4.17
 Lacrimal sac/duct1328.3753.8646.2 
Stage
 T1510.9240360.0.08
 T2919.6777.8222.2 
 T3000000.0 
 T42554.31768832.0 
 TX715.2114.3685.7 
 N04597.82657.81942.2 
 N112.2110000.0 
Tumor size3 cm 3.3 cm 2.9 cm .2
Tumor histology
 Adenoid cystic carcinoma23501252.21147.8.23
 Adenocarcinoma817.4787.5112.5 
 Mucoepidermoid48.7250250 
 Transitional cell carcinoma12.2110000 
 Poorly differentiatedcarcinoma24.3210000 
 Squamous cell carcinoma715.2342.9457.1 
 Undifferentiated12.2001100 
Margin status
 Positive1941.31052.6947.4.03
 Negative2145.71676.2523.8 
 Unknown613116.7583.3 
Perineural invasion
 Negative48.7250250.034
 Positive2043.51680420 
 Unknown2247.8940.91359.1 
Table 2. Treatment Characteristics
 N%
Type of Surgery  
 Orbital exenteration2758.7
 Eye-sparing resection1941.3
Postoperative Radiation  
 Yes3780.4
 No919.6
Radiation Method  
 Conventional  
  X-rays1335.1
  Cobalt616.2
 IMRT1437.8
 Other25.4
Treatment Sequence  
 Orbital exenteration48.7
 Orbital extenteration [RIGHTWARDS ARROW] radiation2350
 Eye-sparing resection510.9
 Eye-sparing resection [RIGHTWARDS ARROW] radiation1430.4
Radiation Dose  
 Median dose, Gy60 ± 7.918 
 Median dose per fraction, Gy2 ± 0.121 

All patients were treated with surgical resection consisting of either orbital exenteration (27 patients [59%]) or more limited eye-sparing surgery (19 patients [41%]). Thirty-seven patients (81%) were treated with postoperative radiotherapy, 23 (85%) in the orbital exenteration group and 14 (74%) in the eye-sparing surgery group. The median radiation dose was 60 Gy (range, 18-70 Gy).

Three patients in this study received induction chemotherapy, 1 of whom also received chemotherapy during radiation. A fourth patient was treated with concurrent chemoradiotherapy after an eye-sparing surgery. Of patients treated with induction chemotherapy, 1 was treated with eye-sparing surgery and 2 with orbital exenteration. Median follow-up time from diagnosis was 38 months (range, 3-460 months).

Survival

Five-year OS and DFS rates for the entire group were 61% and 45% (Fig. 1). On univariate analysis, tumor classification (T1-2 vs T3-4) correlated with improved OS (P = .02), and smaller tumor size (<3.5 vs >3.5 cm) was associated with improved DFS (P = .015). Tumor location (lacrimal gland vs sac), histopathology (adenoid cystic vs other), margin status, type of surgery (OS vs eye-sparing surgery), and use of postoperative radiotherapy did not correlate with OS or DFS. Multivariate analysis was limited secondary to low numbers of patients in the study; however, when tumor size and type of surgery performed were analyzed in this fashion, no significant association between type of surgery and OS (P = .72) or DFS (P = .36) was seen. Similarly, when tumor category and type of surgery were included in a multivariate analysis, again no effect of type of surgery was seen on OS (P = .98) or DFS (P = .75). Specifically, the 5-year OS rates were 62% in the orbital exenteration group and 59% in the eye-sparing surgery group (P = .56), and the 5-year DFS rates were 49% in the orbital exenteration group and 39% in the eye-sparing surgery group (P = .35) (Fig. 2). Postoperative radiotherapy was not associated with improved OS (P = .89), but may have been associated with improved DFS (P = .08) (Fig. 3).

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Figure 1. (A) Overall survival, (B) disease-free survival, (C) locoregional recurrence (LRR), and (D) distant metastasis (DM)-free survival curves for all 46 patients in the current study are shown.

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Figure 2. (A) Overall survival, (B) disease-free survival, (C) locoregional recurrence (LRR), and (D) and distant metastasis (DM)-free survival curves are shown for 27 patients treated with ocular exenteration (OE) and for 19 patients treated with eye-sparing surgery (ESS). No significant differences in outcome were found between groups.

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Figure 3. (A) Overall survival, (B) disease-free survival, (C) locoregional recurrence (LRR), and (D) and distant metastasis (DM)-free survival curves are shown for 37 patients who received postoperative radiotherapy (XRT) and for 9 patients who were observed. Locoregional control was better among patients treated with postoperative radiation (P = .009); an apparent trend toward improved disease-free survival was not statistically significant (P = .08).

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Distant Metastasis

Eighteen patients developed metastatic disease, with the most common sites being bone (8 [44%]), lungs (5 [28%]), and subcutaneous tissue (1 [6%]); the other 4 patients presented with multiple sites of metastases. The 5-year distant metastasis-free survival rate for the entire population was 64% (Fig. 1). On univariate analysis, development of metastases was associated with higher tumor classification (T3-4 vs T1-2, P = .049) and larger tumor size (≥3.5 vs <3.5 cm, P = .013), but not tumor location (lacrimal gland vs sac), histopathology (adenoid cystic vs other), margin status, type of surgery (orbital exenteration vs eye-sparing surgery), and use of postoperative radiotherapy. Surgical type was also not significantly associated with distant metastasis-free survival on multivariate analysis, using a model containing type of surgery and either tumor size (P = .78) or category (P = .34). Five-year distant metastasis-free survival rates were no different between the orbital exenteration group (66%) and the eye-sparing surgery group (61%) (Fig. 2).

Locoregional Control

Overall, 18 of the total 46 patients had had a locoregional relapse at a median 85 months (range, 1-460 months). The 5-year actuarial LRC rate for the population was 60%, with no observed difference between the orbital exenteration group (60%) and eye-sparing surgery group (60%) (Fig. 3). On univariate analysis, having a smaller tumor (<3.5 vs 3.5 cm) (P = .016) and receiving postoperative radiation treatment (P = .017) were associated with improved LRC. No correlations were detected between LRC and tumor classification, location (lacrimal gland vs sac), histopathology (adenoid cystic vs other), margin status, or type of surgery. Surgery performed was not significantly associated with LRC on multivariate analysis, using a model containing type of surgery and either tumor size (P = .78) or treatment with postoperative radiation (P = .34). On multivariate analysis using a model containing tumor size and radiotherapy, treatment with postoperative radiotherapy was significantly associated with LRC (P = .004). For those patients who did not receive postoperative radiotherapy, regardless of initial surgery, the 5-year LRC rate was 22% compared with 73% for patients who received postoperative radiotherapy (P = .009) (Fig. 3). Of the 9 locoregional recurrences in patients treated with postoperative radiotherapy, 5 (28%) were in the radiation field or at the field margin, 3 (17%) were out of field, and 1 (6%) occurred in a patient whose initial treatment field could not be determined (Table 3). LRC rates were no different for patients treated with IMRT versus those treated with other radiotherapy methods.

Table 3. Patterns of Treatment Failure
 N%
  1. LRR indicates loco-regional recurrence; DM, distant metastases.

  2. Percentages represent percent of total number of LRR and DM respectively. All nodal failures were ipsilateral to the primary tumor and all occurred concurrent with failure in the primary site.

Total number of patients with LRR18 
Sites of initial LRR  
 Postoperative bed1161.1
 Parotid gland211.1
 Nasopharynx211.1
 Ptygeroid space15.6
 Maxilla15.6
 Dermis15.6
 In field527.8
 Out of field316.7
 Unknown15.6
 Observation950
Nodal failures  
 Level Ib125
 Level II125
 Retropharygneal125
 Intraparotid125
Initial therapy for patients with LRR  
 Orbital exenteration422.2
 Orbital exenteration [RIGHTWARDS ARROW] radiation633.3
 Eye-sparing resection527.8
 Eye-sparing resection [RIGHTWARDS ARROW] radiation316.7
Total number of patients with DM18 
Sites of initial DM  
 Bone844.4
 Lungs527.8
 Subcutaneous tissue15.6
 Multiple sites422.2

Most initial local failures appeared in the postoperative bed or orbit (61%). No isolated nodal failures were seen. Four patients, 2 in the orbital exenteration group and 2 in the eye-sparing surgery group, experienced nodal failure in addition to local failure or distant metastasis (Table 3). Whereas 3 of the 4 patients had received postoperative radiotherapy, no patient received prophylactic nodal irradiation.

Visual Acuity After Radiation

Information on visual acuity was available for 26 patients treated with radiation of the 46 patients in the study (Fig. 4). No significant difference in corrected visual acuity was found in the untreated eye at any of the time points measured compared with baseline, with the exception of an apparent decrement at 28 to 33 months that may have resulted from the large variance among patients. Of the 19 patients treated with eye-sparing surgery, 15 had an intact globe for >2 years after treatment. Of those 15 patients, 12 had received adjuvant radiotherapy as part of the primary treatment. Long-term (>2 years) information on visual acuity in the ipsilateral eye was available for 6 patients, 5 of whom had received postoperative radiotherapy. Of those 5 patients, 2 could recognize upheld fingers on exam only, and visual acuity in the remaining 3 was >20/200 at 2 years after radiation treatment. Average baseline visual acuity in the ipsilateral eye for those 3 patients was 20/30.

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Figure 4. Visual acuity in the untreated eyes of patients who received postoperative radiotherapy (XRT) is shown. For the 26 patients for whom visual acuity data were available, clinically determined Snellen fractions were normalized to 20/20 vision. Normalized values were then grouped into baseline and time points after radiation. Mean visual acuity is shown ± standard deviation. *Significantly different from baseline (P < .05). CL VA indicates contralateral visual acuity.

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Toxicity

Three patients developed severe (grade ≥3) acute toxicity during radiation treatment (Table 4), 1 with mucositis, 1 with emesis and dehydration requiring hospitalization, and 1 with dermatitis that required surgical intervention. The average total dose received by these patients was 60 Gy. None of the patients who developed acute toxicity developed severe long-term radiation-related complications.

Table 4. Radiation Associated Acute and Late Effects
 N%
≥Grade 3 Acute Effects  
 Dermatitis12.7
 Mucositis12.7
 Emesis12.7
≥Grade 3 Late Effects  
 Ipsilateral glaucoma12.7
 Temporal necrosis38.1
 Ipsilateral entropion12.7
 Ipsilateral radiation keratopathy12.7
 Radiation-associated sarcoma12.7

Seven patients, 6 of whom received 60 Gy, developed severe (grade ≥3) long-term complications after radiotherapy. One patient experienced exacerbation of previously diagnosed glaucoma at 22 months after completion of eye-sparing surgery and postoperative radiotherapy; another patient treated similarly developed radiation-induced keratopathy in the ipsilateral eye 5 months after radiation. Both of these complications necessitated enucleation. A third patient developed entropion 14 months after completing radiotherapy to the intact ipsilateral eye, which required surgical correction but resulted in eye preservation. One patient developed a radiation-associated sarcoma almost 19 years after initial treatment. Three patients developed symptomatic temporal lobe necrosis requiring surgical correction at 6, 10, and 38 months after completing radiotherapy.

DISCUSSION

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES

This report represents 1 of the largest single-institution studies of primary malignancies of the lacrimal apparatus. We found that the extent of surgical resection, specifically eye-sparing surgery versus orbital exenteration, did not affect OS, and that in most cases in which the goal was ocular preservation, that could be accomplished without compromising 5-year locoregional and distant disease control. We further found that postoperative radiotherapy was necessary to prevent locoregional recurrence regardless of which type of surgery had been used.

Traditionally, management of tumors of the lacrimal apparatus has involved some form of surgery, either local excision or more extensive resection, with consideration of adjuvant radiation based on the pathologic findings.2-10, 15, 18-20 However, it has been difficult to generate evidence-based treatment recommendations because of the rarity of this disease. Data are somewhat conflicting regarding the optimal surgical therapy for malignant tumors of the lacrimal apparatus, with at least 1 study showing no benefit from extensive surgical resection compared with an eye-sparing approach.6 However, more recently, Ahmad et al showed significant benefit in terms of local control with orbital exenteration, at least in patients with adenoid cystic carcinoma.2 However, this benefit was limited to patients with T3-T4 tumors. In the current study, more extensive surgery (orbital exenteration as compared with eye-sparing surgery) had no significant benefit in terms of survival or local control. One possible explanation for this difference between studies is that we examined patients with a variety of tumor histologies, whereas the aforementioned study involved primarily adenoid cystic carcinoma. Furthermore, we included tumors of the lacrimal sac in our analysis. Tumors of the lacrimal gland or sac are often viewed as separate entities, because they are often of different histologies and are derived from different embryologic origins; however, we found no significant differences in survival outcomes or recurrence based on tumor location. Because of this, as well as the rarity of these 2 entities and the similarities in their management, we believe it is reasonable to analyze these tumors as a single group. Finally, the finding that patients with larger tumors and higher tumor categories were more likely in this study to undergo orbital exenteration than eye-sparing surgery probably reflects the difficulty of ocular sparing in cases of extensive, infiltrating tumor, as well as surgical bias toward treating more aggressive disease with more extensive surgery. Therefore, consideration of an eye-sparing surgery approach for smaller tumors may be appropriate without compromising long-term outcomes and with acceptable morbidity. As with all studies of tumors of the lacrimal apparatus, this study is limited by small patient numbers and possible bias because of its nonrandomized nature. In addition to the above differences between arms, a small, but significant difference was seen in regard to margin status and perineural invasion. However, this difference may have been biased by the comparatively large number of unknown statuses in the eye-sparing surgery group. In regard to the development of metastatic disease, long-term follow-up is necessary in this population, because distant metastasis was observed >5 years after treatment.

Interpreting the potential benefit of adjuvant radiotherapy in much of the available literature is difficult because of small numbers of patients, inconsistencies in reporting of dose, and the variety of radiation modalities used. However, in at least 1 study, conventional external beam radiotherapy as a single modality has been shown to be inferior to surgery,6 and thus most of the available literature focuses on postoperative radiotherapy. Other modalities for postoperative radiation treatment that have been used include brachytherapy, stereotactic radiosurgery, and neutron radiotherapy.21-23 However, those studies are limited by extremely small numbers of patients, and their findings are difficult to extrapolate to larger patient populations. Thus, the vast majority of the available literature examines the utility of conventional radiotherapy in the postoperative setting.4, 7-9, 12 In the current study, all patients, regardless of tumor site, histology, or surgical therapy, who did not have postoperative radiotherapy eventually developed locoregional recurrence. Conversely, slightly less than 25% of patients who received postoperative radiotherapy developed locoregional recurrence. This difference seems to provide a compelling argument for the addition of adjuvant radiotherapy to postoperative treatment for most patients with this condition. Conclusions regarding the role of chemotherapy in this disease are difficult to elucidate, as only 4 patients, split evenly between the orbital exenteration and eye-sparing surgery groups, received systemic therapy.

The nodal drainage of the lacrimal apparatus was defined by Rouviere.24 Generally, the primary echelon for nodal drainage of the lacrimal apparatus is the intraparotid and preauricular lymph nodes. A secondary nodal drainage pattern for the lacrimal sac and nasolacrimal duct is the submaxillary nodal basin. These were the locations of nodal failures found in this study. Thus, if elective nodal radiation is to be considered, coverage of these nodal basins should be given priority. In this study, no specific factor could be linked to the development of nodal metastasis. In fact, only 9% of the patients developed nodal failure, and none of these failures was an isolated event. This would, in our view, argue against routine prophylactic nodal irradiation.

Acute toxicity during radiotherapy for the patients in this study was acceptable, with only 3 patients developing serious (grade ≥3) toxicity. However, approximately 20% of the patients treated with postoperative radiotherapy developed long-term complications. Because many of these complications were related to an attempt to preserve the ipsilateral eye, the potential for complications was higher. Attempts to preserve the ipsilateral eye were usually successful, as >80% of patients treated with eye-sparing surgery maintained visual acuity for at least 2 years after radiation. The lack of consistent clinical documentation in the early years of this retrospective study limits conclusions regarding long-term visual acuity; however, current prospective documentation will allow us to report long-term outcomes in a future report. Our long-term follow-up information on visual acuity in the contralateral eye indicated no clinically relevant decreases after radiation treatment. Thus, postoperative radiotherapy appears to be safe for the contralateral eye and, for appropriately selected patients, offers the possibility of sparing the ipsilateral eye.

Although not the primary focus of this study, we did find that the recently published AJCC seventh edition staging system for tumors of the lacrimal gland was predictive for outcome.17 Specifically, we found that lower tumor classification (≤T2) was associated with improved OS and freedom from metastases. Other recently published findings also indicated predictive value for the sixth (2002) AJCC staging system for adenoid cystic carcinoma of the lacrimal gland2; however, ours is the first study of which we are aware that has validated the predictive value of the most recent update of the TNM staging system for tumors of the lacrimal apparatus as a whole.

Treatment Recommendations

On the basis of the results of this study and others,2, 6 we recommend consideration of eye-preserving surgery for patients with tumors of the lacrimal apparatus, particularly for tumors that are small. We further found that postoperative radiotherapy is necessary to minimize the risk of locoregional recurrence. However, the risk of radiation-related complications to the ipsilateral eye after adjuvant radiotherapy must be considered and may be minimized through the use of meticulous radiotherapy planning and delivery. In appropriately selected patients with lacrimal apparatus tumors, eye-sparing surgery can provide excellent functional outcomes after adjuvant radiotherapy.

CONFLICT OF INTEREST DISCLOSURES

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES

This research was supported in part by the National Institutes of Health through The University of Texas MD Anderson Cancer Center's Cancer Support Center Grant CA016672.

REFERENCES

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. CONFLICT OF INTEREST DISCLOSURES
  7. REFERENCES
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    Rootman J, White VA. Changes in the 7th edition of the AJCC TNM classification and recommendations for pathologic analysis of lacrimal gland tumors. Arch Pathol Lab Med. 2009; 133: 1268-1271.
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    Esmaeli B, Golio D, Kies M, DeMonte F. Surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland. Ophthal Plast Reconstr Surg. 2006; 22: 366-370.
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    Lee D, Campbell R, Waller R, Ilstrup D. A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland. Ophthalmology. 1985; 92: 128-134.
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    Wright J. Factors affecting the survival of patients with lacrimal gland tumours. Can J Ophthalmol. 1982; 17: 3-9.
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