Dedifferentiated adenoid cystic carcinoma of the lacrimal gland

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.     

Ulcerated adenoid cystic carcinoma of the lacrimal gland--case presentation

The authors present the case of a 63 years old patient, admitted in the Neurosurgical Department, presenting exulcerated adenoid cystic carcinoma of the right lacrimal gland. The tumor was extended medially to the superolateral part of the posterior orbit and laterally in the temporal fossa. The patient presents one and a half years history of left exophthalmos, the tumor exulcerated in the left temporal region one year ago, meantime the tumor extended in the superior half of the posterior orbit. The tumor was completely removed using superolateral orbital approach, with very good postoperative course. Complete recovery of the visual function and satisfactory aesthetic aspect was obtained, without the risks of a craniotomy. Good outcome without recurrence in the past two years.     

泪腺腺样囊性癌的超微结构观察

目的观察泪腺腺样囊性癌(adenoid cystic carcinoma,ACC)与正常泪腺的超微结构,明确肿瘤基底膜(basement membrance,BM)改变与病理类型的关系。方法收集泪腺ACC和正常泪腺标本各6例,行透射电子显微镜检查,观察不同病理分型ACC超微结构特点和BM形态的改变。结果正常泪腺的腺泡由腺上皮细胞和肌上皮细胞组成,细胞的基底面与结缔组织间有均匀的BM相隔。病理分型为管状型和筛状型的ACC肿瘤细胞由腺上皮和肌上皮细胞组成,排列紊乱,细胞染色质分布不均,深染;BM明显增厚,呈多层,互相交织成网状或不规则折叠,肿瘤细胞可伸出伪足侵入BM,使其中断溶解。实性型ACC细胞间界限不清,细胞外堆积大量溶解的细胞外基质,完整BM结构少见。结论 ACC的BM增厚、结构紊乱、溶解和中断可能是肿瘤侵袭能力的体现。阻断肿瘤细胞对BM的降解或减少相关酶的活性,有可能成为治疗或延缓肿瘤侵袭的新靶点。     

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.     

泪腺腺样囊性癌的研究现状

泪腺腺样囊性癌是原发性泪腺上皮性肿瘤中最常见且恶性程度最高的肿瘤.近年来国内外对该病的组织学分类及病理学改变、主要临床表现、治疗手段有了新的认识;在细胞与分子生物学方面,对与泪腺腺样囊性癌的发生、侵袭性生长及转移、嗜神经性生长等特性相关的生物学指标的研究也取得了一些进展.就泪腺腺样囊性癌的研究现状做一综述.     

泪腺腺样囊腺癌的MRI特征分析

目的 探讨泪腺腺样囊腺癌（adenoid cystic carcinoma,ACC）的MRI特征,为临床早期诊断提供依据。设计 回顾性病例系列。研究对象 北京同仁医院经手术证实ACC患者26例,术前均行MRI扫描。方法 所有ACC患者常规MRI扫描;同时对动态增强磁共振成像（DCE-MRI）及磁共振扩散加权成像（DWI）图像进行后处理获得相应量化指标。主要指标 ACC动态增强的峰值强化指数（CIpeak）、峰值强化时间（Tpeak）、最大强化指数（CImax）、强化指数曲线（I型为持续上升型,II型为平台型,III型为流出型）、肿瘤平均表观扩散系数（ADC）值。结果  26例均起自泪腺眶部,20例形态不规则,14例边界不清,12例边界清晰,6例累及颅内,9例出现骨质破坏。T1WI呈等或略低信号,其中3例内部出现高信号区;T2WI呈等或稍高信号为主,增强后仅1例强化均匀,25例呈不均匀强化,其中16例伴有内部囊变、坏死区。泪腺ACC峰值强化指数（CIpeak）为0.9±0.41,峰值强化时间（Tpeak）为（109.3±58.5）s, 最大强化指数（CImax）为0.95±0.45。22例时间强化指数曲线中2例为I型,14例为II型,6例为III型。平均ADC值为（1.25±0.30×10-3）mm2·s－1。结论 MRI可以清晰显示泪腺ACC的部位、形态、范围、边界及骨质侵犯,DCE-MRI及DWI为肿瘤的诊断及鉴别诊断提供辅助量化特征。（眼科,2013, 22： 314-319）     

泪腺腺样囊性癌的组织病理学特征

目的 分析泪腺腺样囊性癌的组织病理学特征,明确肿瘤侵袭相关的组织病理学特征与分型的关系.设计回顾性病例系列.研究对象经病理诊断证实的泪腺腺样囊性癌标本49例.方法 观察组织学形态特征并以WHO标准分型,根据Szanto法分级.免疫组化检查选用的一抗有S-100和CD34.主要指标各种组织病理学分型的特征,与肿瘤侵袭相关的病理学特征在各分级绀的阳性率,包括侵犯神经、脂肪、肌肉、血管内瘤栓等.侵犯神经在有无疼痛症状患者间的阳性率.结果 泪腺腺样囊性癌组织学病理分型分为管状、筛状和实性型3种,Szanto Ⅰ级19例,Ⅱ级9例,Ⅲ级21例.S-100和CD34分别标记肿瘤内的神经纤维和血管内皮.肿瘤侵犯神经表现为两种显微镜下形态,共22例,占45%,其中Ⅰ级中1例,Ⅱ级中6例,Ⅲ级中15例.Ⅰ级和Ⅲ级间侵犯神经的例数有统计学差异(P=0.001);Ⅰ级和Ⅲ级间侵犯神经的例数有统计学差异(P=0.000).分别有12例和7例显微镜下可见到肿瘤侵犯脂肪和肌肉,但三级间均无统计学筹异.仅1例可见静脉血管内瘤栓.有疼痛者32例,无疼痛者17例.各有15例和7例显微镜下可见肿瘤侵犯神经,两者比较无统计学差异(P=0.703).结论 泪腺腺样囊件癌中Ⅲ级比例较高.侵犯神经是该肿瘤重要的侵袭方式,也是特征性病理表现,在高分级的肿瘤中发生率较高.侵犯脂肪、肌肉以及血管内瘤栓发生率较低.疼痛症状与镜下肿瘤侵犯神经无明显对应关系.     

泪腺腺样囊性癌CT表现的研究

目的 分析泪腺腺样囊性癌常见的CT图像特征,从而探讨CT(computed tomography)影像对于泪腺腺样囊性癌的诊断价值.方法 病历来源为天津医科大学眼科中心及天津医科大学第二医院,经病理确诊的眼眶腺样囊腺癌39例.其中原发肿瘤20例,复发肿瘤19例,均为单眼发病,所有患者均行眼眶CT检查,获取水平及冠状扫描图像.结果 泪腺腺样囊性癌中原发肿瘤形状多为厚扁平状,贴附于眶壁增长,在体层像上呈锥形者10例、不规则形5例;复发肿瘤形状为不规则型8例、厚扁平状,贴附于眶外上壁向后增长者8例.原发与复发泪腺腺样囊腺癌的好发位置位于眶外上方分别为18例与11例.结论 CT可清晰显示肿瘤的位置,形状,范围、眶骨壁改变,对于泪腺腺样囊性癌的诊断和制定治疗方案具有重要指导意义.     

泪腺腺样囊性癌CT表现的研究

目的 分析泪腺腺样囊性癌常见的CT图像特征,从而探讨CT(computed tomography)影像对于泪腺腺样囊性癌的诊断价值.方法 病历来源为天津医科大学眼科中心及天津医科大学第二医院,经病理确诊的眼眶腺样囊腺癌39例.其中原发肿瘤20例,复发肿瘤19例,均为单眼发病,所有患者均行眼眶CT检查,获取水平及冠状扫描图像.结果 泪腺腺样囊性癌中原发肿瘤形状多为厚扁平状,贴附于眶壁增长,在体层像上呈锥形者10例、不规则形5例;复发肿瘤形状为不规则型8例、厚扁平状,贴附于眶外上壁向后增长者8例.原发与复发泪腺腺样囊腺癌的好发位置位于眶外上方分别为18例与11例.结论 CT可清晰显示肿瘤的位置,形状,范围、眶骨壁改变,对于泪腺腺样囊性癌的诊断和制定治疗方案具有重要指导意义.

Abstract：

Objective To analyze imaging characteristics of adenoid cystic carcinoma(ACC)of lachrymal gland on CT for the diagnosis of ACC in clinic. Methods Thirty-nine patients with ACC consisting of 20 cases of primary tumor and 19 cases of recurred tumors confirmed by pathology were collected from The Eye Centre of Tianjin Medical University and The Second Hospital of Tianjin Medical University. All the patients were unilateral, and examined by CT. The imaging characteristics of ACC on CT were analyzed. Results The most common shape of primary ACC included 10 cases of conical-shape, 5 cases of irregular-shape. The recurred cases contained 8 cases of irregular-shape, 8 cases of conical-shape. The primary and recurred ACC usually existed on superior-external side, with which there were 18 cases and 11 cases respectively. Conclusions CT examination can exactly reveal the shape, size, position, and ossa orbitale destruction of ACC and played an important role in the diagnosis of ACC.     

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.     

泪腺腺样囊性癌临床治疗进展

泪腺腺样囊性癌(adenoid cystic carcinoma,ACC)是最常见的泪腺恶性上皮肿瘤,单纯手术切除疗效不满意,易复发,预后差。我们总结近年来国内外报道的相关泪腺ACC的临床综合治疗手段,包括改进手术入路、采取手术前后化学治疗、放射治疗等综合治疗方案,特别还报告了青年泪腺ACC患者的治疗策略,并分析了原发于泪腺以外ACC的诊断与治疗,为眼科临床医生提供更广阔的思路。     

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。     

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.     

Surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland

PURPOSE: To review our experience with multidisciplinary surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland. METHODS: We present a case series of 7 patients with lacrimal gland adenoid cystic carcinoma treated at our institution between June 2001 and October 2003. Clinical records, histologic sections, and radiographic images were reviewed. RESULTS: The study included 3 men and 4 women (mean age at diagnosis, 44 years). All 7 patients underwent an orbital exenteration with bone removal. Five patients had an orbitectomy through a craniotomy approach and 2 patients had an exenteration through a fronto-orbito-zygomatic approach, all with removal of the bone of the superior and lateral wall. Six patients underwent reconstruction of the socket through the use of a vascularized flap. The surgical approach involved a neurosurgeon, an oculoplastic or head and neck surgeon, and a plastic surgeon. Six patients received postoperative radiation therapy. One patient with a recurrent tumor had already received radiation therapy, which precluded additional radiation therapy after surgical resection. The radiation field included the orbit and the skull base because all patients had evidence of perineural invasion. As of this writing, there have been no local recurrences. Five patients had development of distant metastases and died of disease, at follow-up times from 12 to 32 months after surgery. Two patients are alive without evidence of disease, both at 24 months' follow-up. CONCLUSIONS: Orbitectomy with bone removal may be indicated for achieving local and regional control in advanced cases of adenoid cystic carcinoma of the lacrimal gland. This surgery does not decrease the risk of distant metastasis. The cases in our series highlight the locally invasive and metastatic behavior of this cancer.     

动脉介入化学疗法在视网膜母细胞瘤和泪腺腺样囊性癌治疗中的应用

动脉介入化学疗法是20世纪90年代发展起来的一种局部化疗技术,主要用于恶性肿瘤的局部给药治疗.该技术可集中高剂量化疗药物进行局部治疗,且全身副作用小.目前已被应用于眼部肿瘤尤其是视网膜母细胞瘤及泪腺腺样囊性癌的治疗研究,并显示出较好的效果.本文主要论述动脉介入化学疗法在视网膜母细胞瘤及泪腺腺样囊性癌治疗方面的应用及进展...     

眼眶泪腺腺样囊性癌手术联合放疗的疗效观察

目的：回顾总结20年间眼眶泪腺腺样囊性癌手术联合放疗的治疗效果。方法：对21例腺样囊性癌手术联合放疗后进行随访分析，女性12例，男性9例；年龄15－61岁，平均37．5岁。放射治疗采用^60Co，直线加速器（电子，光子），γ刀及后装近距离治疗＋外，放射野为正位，侧位或斜侧位。结果：10例随访3年无复发，其中6例随访5年无复发，1例10年以上无复发，平均随访3年。放疗的并发症：皮疹，脱发秃睫，放射性白内障。结论：手术联合放射治疗是恶性肿瘤综合治疗的重要手段之一。采用包括放射治疗在内的综合治疗，可提高患者的治愈率，保存患病器官的功能，改善生存质量。     

Unusual presentations of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland

Purpose: To report two atypical cases of pleomorphic adenoma and adenoid cystic tumours of the lacrimal gland. Methods: Two case reports are presented. The first is of a 65-year-old female with a long history of right hypoglobus with sudden recent worsening. Computed tomography (CT) showed a round, well-defined lesion in the fossa for the lacrimal gland with an anterior hypodense exten- sion suggestive of possible malignancy in a pleomorphic adenoma. The tumour in the second case, a 35-year-old male, was diagnosed after presentation following a relatively minor periorbital injury. The smooth rounded mass on CT scan was suggestive of a benign lacrimal gland tumour. Results: The lesion in case 1 was excised with a diagnosis of haemorrhage within a pleomorphic adenoma. The lesion in case 2 was excised with a diagnosis of adenoid cystic carcinoma of the lacrimal gland with pseudocapsule. Conclusions: Haemorrhagic cyst developing beneath the pseudocapsule of a pleomorphic adenoma should be considered in the differential diagnosis of secondary development of malignancy in a pleomorphic adenoma. Adenoid cystic tumours of the lacrimal gland can present with a pseudocapsule.     

Unusual presentations of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland.

PURPOSE: To report two atypical cases of pleomorphic adenoma and adenoid cystic tumours of the lacrimal gland. METHODS: Two case reports are presented. The first is of a 65-year-old female with a long history of right hypoglobus with sudden recent worsening. Computed tomography (CT) showed a round, well-defined lesion in the fossa for the lacrimal gland with an anterior hypodense extension suggestive of possible malignancy in a pleomorphic adenoma. The tumour in the second case, a 35-year-old male, was diagnosed after presentation following a relatively minor periorbital injury. The smooth rounded mass on CT scan was suggestive of a benign lacrimal gland tumour. RESULTS: The lesion in case 1 was excised with a diagnosis of haemorrhage within a pleomorphic adenoma. The lesion in case 2 was excised with a diagnosis of adenoid cystic carcinoma of the lacrimal gland with pseudocapsule. CONCLUSIONS: Haemorrhagic cyst developing beneath the pseudocapsule of a pleomorphic adenoma should be considered in the differential diagnosis of secondary development of malignancy in a pleomorphic adenoma. Adenoid cystic tumours of the lacrimal gland can present with a pseudocapsule.