Outcomes in patients with adenoid cystic carcinoma of the lacrimal gland

PURPOSE: To evaluate the outcomes among patients with adenoid cystic carcinoma of the lacrimal gland treated at various stages of their disease at a tertiary care cancer center. METHODS: A retrospective case series of 20 patients with adenoid cystic carcinoma of the lacrimal gland treated at a single institution between 1952 and 2002. Clinical records were available for all 20 patients; histologic sections from 12 of the 20 patients were available for review. Disease-free survival was measured from the completion of treatment; overall survival was measured from the date of initial diagnosis. RESULTS: The study included 6 men and 14 women. The mean age at diagnosis was 39.5 years. The median follow-up time was 34 months (range, 6 to 264 months). The local/regional treatment modalities included exenteration with bone removal and radiation therapy (RT) in 5 patients, exenteration with RT (no bone removal) in 8 patients, exenteration (no RT or bone removal) in 1 patient, exenteration with bone removal (no RT) in 1 patient, local resection with RT in 3 patients, and local resection without RT in 2 patients. Overall, 16 patients had RT as part of their treatment regimen. Seven patients (35%) had local recurrence. Sixteen patients (80%) had distant metastasis during the study period. At the time of this report, 13 (65%) of the patients had died of disease. The median disease-free survival for the entire group was 18 months. Eight patients had a predominantly basaloid histologic pattern. Ten patients had verifiable histologic evidence of perineural invasion. CONCLUSIONS: This study underscores the generally grave prognosis for patients with adenoid cystic carcinoma of the lacrimal gland and the difficulty in making any conclusive recommendations for local therapy for this disease.     

Combined therapeutic approach to malignant lacrimal gland tumors.

The medical records of 13 patients with primary malignant lesions of the lacrimal gland revealed adenoid cystic carcinoma as the most common malignant tumor. The histologic cell type, neural invasion, and bony destruction were correlated with localized control and ultimate survival. Surgical removal of the lacrimal gland was adequate treatment for low-grade mucoepidermoid carcinoma confined to the gland itself. Combining a radical surgical removal of the area in planned sequence with high voltage radiation therapy offered the only reasonable hope for localized control in the more aggressive cell types or in tumors with neural invasion or bony involvement. A 40%-localized control and survival rate was achieved in the adenoid cystic carcinoma group of patients. Radiation therapy administered for cancer that obviously recurs after surgical resection was generally unsuccessful.     

Clinical analysis of the effect of intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma

PURPOSE: To determine the effect of intraarterial cytoreductive chemotherapy (IACC) as an adjunct to conventional surgery and radiation therapy for lacrimal gland adenoid cystic carcinoma (ACC). DESIGN: A retrospective, comparative, interventional case series. METHODS: setting: Institutional. patient population: Nine consecutive patients with lacrimal gland ACC were treated with IACC, followed by orbital exenteration and chemoradiotherapy. This case series was compared with a series of seven patients treated by conventional local therapies in the same institution. intervention procedure: Clinical records, imaging studies, histologic sections, and archival specimens from all 16 patients were reviewed. Information analyzed included site of disease, histologic characteristics, extent of disease, incidence of locoregional recurrence or distant metastases, and disease-free survival and overall survival time. main outcome measure: The effect of IACC was assessed by the radiographic and histologic response and survival outcome in comparison to a historical cohort of patients managed by conventional local therapies. RESULTS: The difference between the carcinoma cause-specific death rate of the study group versus conventional treatment was significant (P = .029, log rank test). The cumulative 5-year carcinoma cause-specific death rate in the IACC treated group was 16.7% compared with 57.1% in the conventional treatment group. The cumulative 5-year recurrence rate in the IACC treated group was 23.8% compared with 71.4% in the conventional treatment group. CONCLUSIONS: The preliminary data suggest that IACC as an integral component of a multimodal treatment strategy is potentially effective in improving local disease control and overall disease-free survival in lacrimal gland adenoid cystic carcinoma.     

泪腺上皮性肿瘤的外科治疗探讨

目的 探讨泪腺上皮性肿瘤的现代治疗方法。方法 对34例泪腺上皮性肿瘤进行分析。其中20例泪腺良性多形性腺瘤（复发性7例），11例腺样囊性癌（复发性９例），２例恶性多形性腺瘤（复发性2例）和1例黏液表皮癌。结果 前路开眶15例，改良外侧开眶15例，眶内容摘除术2例，内侧开眶1例，术后放射治疗17例（恶性14例，良性3例），随访28例，时间半年至1.5年，良性肿瘤16例均无复发，恶性肿瘤12例中2例复发。结论 泪腺上皮性肿瘤是常见的眶内肿瘤，复发率较高。改良外侧开眶是切除泪腺上皮性肿瘤最好的方法。     

Adenoid cystic carcinoma of the lacrimal gland: report of two cases and literature review

The adenoid cystic carcinoma of the lacrimal gland is a rare malignant orbital tumour, which has a generally poor prognosis. The therapy for adenoid cystic carcinoma of the lacrimal gland remains controversial. We present two cases of patients with adenoid cystic carcinoma of the lacrimal gland, who were treated with radical surgery in combination with radiotherapy. The therapeutic results are discussed with reference to relevant literature reports.     

泪腺肿瘤38例临床分析

目的：分析泪腺肿瘤的临床特点和治疗结果。方法：回顾性研究38例45眼泪腺肿瘤。结果38例45眼中，男17例17眼，女21例28眼；年龄5～63岁，平均40．6岁；其中泪腺癌5眼，腺样囊性癌5眼。恶性泪腺混合瘤5眼，良性泪腺混合瘤14眼，炎性假瘤13眼，慢性泪腺炎2眼，泪腺潴留性囊肿1眼。42眼行前路或侧壁开眶肿瘤摘除术，恶性肿瘤中4眼行眶内容物剜除。恶性肿瘤患者全部于术后1个月接受放疗。术后随访观察3个月～8年。术后症状完全缓解35眼，好转5眼，复发5眼。结论：泪腺肿瘤为眼眶常见肿瘤，结合病史，临床表现和影像学检查。绝大部分可以作出术前定性诊断，良性泪腺混合瘤预后较好，但易复发。恶性泪腺混合瘤及腺样囊性癌复发率、死亡率较高，应联合眶内容物剜除，放疗及化疗。     

泪腺腺样囊性癌生物标志物的研究进展

泪腺腺样囊性癌是泪腺最常见的恶性上皮肿瘤,具有易复发、易转移及预后差的特点,目前临床治疗方式主要有手术切除、放疗、化疗等,但其生存率仍低。因此,进一步研究泪腺腺样囊性癌的发病机制和寻找泪腺腺样囊性癌的生物标志物尤为迫切。本文将对泪腺腺样囊性癌的生物标志物的研究进展作一综述。     

Cystic adenocarcinomas of the lacrymal gland

Adenoid cystic carcinoma of the lacrimal gland. BACKGROUND: Adenoid cystic carcinoma is the most frequent epithelial tumour of the lacrimal gland. It represents 1.6 % of the orbital tumours. The aim of this study is to present a series of such patients who were followed up in our clinic and to discuss their clinical management in order to achieve a better life prognosis. MATERIALS AND METHODS: The cases we followed up in our department were presented, the treatment modalities were discussed, and conclusions were drawn. RESULTS: Six patients with this pathology were examined, 3 men and 3 women, aged 31 to 69 years. A large excision was performed in 5 patients (exenteration alone or extended exenteration with complementary radiotherapy) and in 1 patient with excision of the tumour followed by radiotherapy. One patient died due to carcinoma six years after the first surgery. The median follow-up period of our cases is 25 months. CONCLUSION: Adenoid cystic carcinoma of the lacrimal gland is a malignant tumour with a reserved prognosis. Various treatments were proposed to improve the mortality rate of the disease. At present radical surgery with or without complementary therapy seems to give the best chances of healing, at least with short or mid-term follow-up.     

An atypical presentation of adenoid cystic carcinoma of the lacrimal gland

PURPOSE: To report a case of lacrimal gland adenoid cystic carcinoma (ACC) with an atypical initial presentation and to postulate an anatomical explanation for this unusual biologic behavior. DESIGN: Interventional case report. METHODS: An orbital magnetic resonance imaging study of a 58-year-old man who complained of progressive diplopia and orbital discomfort disclosed a soft tissue mass in the left cavernous sinus and orbital apex. The left lacrimal gland and the contiguous bone appeared normal. RESULTS: Biopsy of the intracranial mass revealed ACC of unknown origin which was judged to be unresectable. He underwent two cycles of intraarterial cytoreductive chemotherapy followed by a left medial maxillectomy with sphenoethmoidectomy, orbital exenteration, and chemoradiation. ACC tumor cells were identified in nerve fiber bundles surrounding and within the lacrimal gland. CONCLUSIONS: In patients presenting with an infiltrative mass in the cavernous sinus or orbital apex, metastatic disease from an occult lacrimal gland primary should be considered, even with a normal-appearing lacrimal gland.     

Adenoid cystic carcinoma of the lacrimal gland]

A 38-year-old female presented with a left-sided peripheral N. VI-paresis and negative neuroradiologic work-up. After one year she developed an incomplete N. III paresis, numbness of her face correlating to N. V1 and V2, reduced lacrimal secretion as well as a palpable mass at the lateral orbital rim. Magnetic resonance imaging revealed an orbital tumor extending into the cavernous sinus. Biopsy disclosed an adenoid cystic carcinoma of the lacrimal gland with basaloid, cribriform and tubular pattern. Therefore, an exenteration with postoperative radiation therapy was performed. Immunohistochemical findings included positive reactions for keratin (KL-1 and AE 1 + 3), Vimentin and Egp 34. By determination of Ki 67 the proliferative activity of the tumor was found to be 15%. Our case report demonstrates that even in absence of an exophthalmus or pain a malignant orbital tumor may be assumed as the underlying cause for a combined monolateral oculomotor and trigeminal paresis. Immunohistochemical results support the notion, that the adenoid cystic carcinoma of the lacrimal gland may arise from precursor cells of the terminal duct system.     

E-cadherin和β-catenin在泪腺多形性腺瘤和腺样囊性癌中的表达

目的研究E-cadherin及β-catenin在泪腺多形性腺瘤和腺样囊性癌中的表达。方法选取经病理检查确诊为泪腺多形性腺瘤的患者11例11眼和腺样囊性癌的患者6例6眼,分别行E-cadherin和β-catenin免疫组织化学染色。选取泪腺脱垂患者的泪腺组织6例作为正常对照组。结果正常泪腺组织中E-cadherin呈强阳性表达,主要位于细胞膜;而在泪腺多形性腺瘤中E-cadherin呈弱阳性表达,主要位于细胞质。E-cadherin在腺样囊性癌中未见表达。3组中E-cadherin阳性表达的样本数差异有统计学意义（H=16.492,P=0.000）;正常泪腺组E-cadherin阳性表达的样本数明显高于多形性腺瘤组和腺样囊性癌组,差异均有统计学意义（U=-3.561,P=0.000;U=-3.108,P=0.002）,多形性腺瘤组中E-cadherin阳性表达的样本数明显高于腺样囊性癌组,差异有统计学意义（U=-2.147,P=0.032）。正常泪腺β-catenin细胞膜呈中度或强阳性表达,多形性腺瘤和腺样囊性癌中β-catenin的表达较正常泪腺组织表达阳性程度差异无统计学意义（P〉0.05）,腺样囊性癌组中β-catenin细胞核阳性的比例（33.3%,2/6）较多形性腺瘤组（72.7%,7/11）低。结论 E-cadherin表达的下降是参与泪腺上皮性肿瘤发生及癌变的重要因素,而E-cadherin的进一步下降甚至缺失,以及β-catenin的核转位可能是参与泪腺多形性腺瘤恶性转化的因素之一。     

Adenoid cystic carcinoma arising in the inferior orbit without evidence of lacrimal gland involvement

A 60-year-old woman sought treatment for right orbital fullness and intermittent headache. CT revealed an inferior orbital mass along the inferior rectus muscle. Incisional biopsy revealed an adenoid cystic carcinoma. Orbital exenteration followed by concurrent radiotherapy and chemotherapy was performed. The lacrimal gland was uninvolved by tumor microscopically. Although rare, adenoid cystic carcinoma must be considered in the differential diagnosis of an orbital tumor, because the unusual location of an orbital adenoid cystic carcinoma may make its early detection difficult.     

Basal cell adenocarcinoma of the lacrimal gland

PURPOSE: Basal cell adenocarcinoma is a recently recognized entity among malignant tumors of the salivary glands. This entity has not received enough attention among ophthalmologists and ophthalmic pathologists. We recommend that basal cell adenocarcinoma be included in the differential diagnosis of lacrimal gland tumors. DESIGN: Case report. METHODS: In 1988 a lacrimal gland tumor was incompletely removed from the left orbit of a 36-year-old woman and diagnosed as "solid basaloid adenoid cystic carcinoma". Soon after, an exenteration of the orbit was performed. In 1998, upon review of the initial histopathology, the diagnosis of solid adenoid cystic carcinoma was changed to basal cell adenocarcinoma. RESULTS AND MAIN OUTCOME MEASURE: The pathologic findings included nests of basaloid cells with minimal atypia and incomplete palisading around the periphery. The cells were of two types. The first type were large, pale cells with round or oval nuclei, scanty cytoplasm, and ill-defined borders. The other type were smaller cells with hyperchromatic nuclei situated mainly near the periphery of the nests. There was no necrosis or perineural invasion. Mitotic figures were present. Cysts within the nests showed Alcian blue negative contents. Immunohistochemistry showed a positive reaction to cytokeratin and a negative reaction with smooth muscle actin (SMA). CONCLUSIONS: The Alcian blue negative stain, the negative reaction to SMA, and the fact that the patient is still alive 10 years after exenteration favored the diagnosis of basal cell adenocarcinoma. It is essential to differentiate a basal cell adenocarcinoma of the lacrimal gland from the solid basaloid type of adenoid cystic carcinoma, because the former has a better prognosis. Patients with basal cell adenocarcinomas of the lacrimal gland should be closely monitored for local recurrences because this tumor has the tendency to show perineural invasion.     

眼眶泪腺癌26例的CT诊断价值

目的：探讨泪腺腺癌的CT诊断价值。 方法：回顾性分析26例26眼经病理证实眼眶泪腺腺癌患者的CT影像资料。 结果：泪腺腺癌26例均为单眼发病,右眼15例,左眼11例。CT表现：病变呈圆形、卵圆形15例,不规则形、分叶形11例。15例边界尚清楚,11例轮廓呈锯齿状。肿瘤密度不均匀,内见低密度灶或钙化。26例病变包绕并压迫眼球,14例沿眶外壁向眶尖区生长,与眼肌分界不清,并浸润视神经。眼眶骨质虫蚀样破坏21例,溶骨性破坏5例。 结论：泪腺癌有特异性CT征象,其诊断准确率高,是本病最主要影像检查方法。     

An unusual clinical course of adenoid cystic carcinoma of the lacrimal gland

The case of a 25-year-old woman with adenoid cystic carcinoma of the lacrimal gland is reported. Her complaints, including pain and localized swelling, first started six years earlier. She was diagnosed elsewhere as orbital pseudotumor based on clinical and computed tomographic features and was prescribed oral corticosteroids. The regimen completely relieved her signs and symptoms, which regularly recurred in the ensuing years. When she finally presented with rapidly worsening proptosis and pain, she underwent incisional biopsy followed by exenteration and 6000 cGy of radiotherapy. This patient is a rare example of adenoid cystic carcinoma of the lacrimal gland with an atypical clinical course of long duration during which corticosteroids completely suppressed the clinical signs and symptoms.     

An unusual clinical course of adenoid cystic carcinoma of the lacrimal gland

The case of a 25-year-old woman with adenoid cystic carcinoma of the lacrimal gland is reported. Her complaints, including pain and localized swelling, first started six years earlier. She was diagnosed elsewhere as orbital pseudotumor based on clinical and computed tomographic features and was prescribed oral corticosteroids. The regimen completely relieved her signs and symptoms, which regularly recurred in the ensuing years. When she finally presented with rapidly worsening proptosis and pain, she underwent incisional biopsy followed by exenteration and 6000 cGy of radiotherapy. This patient is a rare example of adenoid cystic carcinoma of the lacrimal gland with an atypical clinical course of long duration during which corticosteroids completely suppressed the clinical signs and symptoms.     

A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland

A retrospective study of 26 patients with the diagnosis of primary adenoid cystic carcinoma of the lacrimal gland was performed with use of randomly selected tissue from each tumor. An attempt was made to determine any associations among clinical presentation, survival, and tumor histologic patterns. We found that lower tumor grades were associated with a predominantly Swiss-cheese (cribriform) pattern. Both lower tumor grade and Swiss-cheese pattern were associated with longer survival. The basaloid (solid) pattern or bone invasion was not associated with a shorter survival. Between men and women, there were no differences in clinical presentation, histologic patterns, or survival. Survival was not affected by surgical or radiation therapy.     

Orbital exenteration: one size does not fit all

PURPOSE: To evaluate the clinical indications for orbital exenteration in a tertiary referral center and to compare clinicopathologic correlation and cosmetic outcome with previously reported data. DESIGN: Retrospective, nonrandomized, consecutive case series. METHODS: Review of Electronic Medical Record system, Orbital Clinic, Jules Stein Eye Institute, between January 1999 and December 2003. main outcome measures: Surgery type, clear margins histologically, survival, and wearing an eye patch. RESULTS: Thirty-four patients (mean age 67 years) underwent orbital exenteration; mean follow-up 1.2 +/- 1.5 years (6 months to 6 years). Diagnosis included orbital, ocular, and adnexal malignancies, with squamous and basal cell carcinoma being the most common. Twenty-one patients (62%) underwent total or extended orbital exenteration, and 13 patients (38%) underwent subtotal exenteration including tissue reconstruction. Clear surgical margins were obtained in 23 cases (68%), whereas positive margins were left in 11 cases (32%). Many of the patients preferred an eye patch to cover the surgical region regardless of surgical reconstruction. Only 4 patients (11.8%) who underwent subtotal exenteration with orbital prosthesis did not use a patch. During follow-up period 3 patients expired, only 1 of which was tumor-related. CONCLUSIONS: Clinical indications for orbital exenteration remain similar over the last four decades with a higher prevalence of squamous cell carcinoma in our institute. Orbital exenteration is considered curative in cases of basal or squamous cell carcinoma but not in cases of malignant infiltrative processes such as adenoid cystic carcinoma of the lacrimal gland. Patients are likely to wear an eye patch regardless of any attempt at surgical reconstruction.     

泪腺腺样囊性癌的分期及临床治疗进展

泪腺腺样囊性癌是泪腺最常见的恶性上皮性肿瘤,手术治疗效果不理想。近年来,随着多种放射治疗和化学治疗的应用,泪腺腺样囊性癌的治疗模式发生较大转变。一方面,临床分期逐步完善,以新的分期为基础的综合治疗得到规范化应用; 另一方面,在质子放疗、中子放疗、经动脉细胞减容化疗等新辅助治疗的支持下,保眼手术逐渐增多,局部复发率和转移率逐渐降低,无病生存期获得延长,但还需要长期观察。本文就近年来国际上泪腺腺样囊性癌相关方面的治疗进展进行综述,以提供新的参考依据。     

Space-occupying lesions of the lacrimal gland at one tertiary eye center in China: a retrospective clinical study of 95 patients

AIM: To investigate the treatment status and prognosis of space-occupying lacrimal gland lesions at one tertiary eye center in China. METHODS: A retrospective clinical study was performed on 95 patients with space-occupying lesions of the lacrimal gland surgically treated at the Eye & ENT Hospital of Fudan University from 2003 to 2007. The reviewed clinical data included age, gender, side of the lesion, duration of signs and symptoms, histopathological diagnosis, treatment modality, recurrence (local, regional, and distant metastasis) and survival. RESULTS: Of the 95 cases (99 eyes), pleomorphic adenomas were the most common lesions (43 cases), followed by lymphoid disorders (14), inflammatory pseudotumors (11), carcinoma ex-pleomorphic adenomas (11), and adenoid cystic carcinomas (ACC, 6). There were 8 patients with relapsed pleomorphic adenomas. Five of these 8 cases had malignant pathological changes. All patients with ACC had metastasis and three of them died during their follow-up. CONCLUSION: Our study indicated that the most common lacrimal gland lesions were pleomorphic adenomas. Multiple recurrence and surgical procedures may increase the risk of tumor progression. ACC had a high incidence of tumor metastasis and a poor prognosis.