Primary Adenoid Cystic Carcinoma Presenting as an Orbital Apex Tumor

Primary adenoid cystic carcinoma occurring in the orbital apex is rare. We present the clinical features of a patient who initially presented with the clinical and radiologic features of an orbital pseudotumor. He developed features of orbital apex syndrome and repeat imaging showed a tumor of the orbital apex with intracranial invasion. He underwent radical skull base surgery and pathologic examination revealed adenoid cystic carcinoma in the orbital apex with a normal lacrimal apparatus. He received post operative radiation and the outcome in the light of a review of available literature is being discussed.     

Primary cutaneous adenoid cystic carcinoma of the eyelid

PURPOSE: To report a case of primary cutaneous adenoid cystic carcinoma involving the eyelid. METHODS: We examined a 70-year-old woman with an 18-month history of a gradually enlarging, blue-colored mass in the medial third of the right upper eyelid. Excisional biopsy and additional resection of the medially infiltrated margin resulted in tumor-free edges. Superior cervical and orbital evaluations were performed. RESULTS: Histopathologic evaluation revealed a primary cutaneous adenoid cystic carcinoma. No gland pathology was documented. No tumor recurrence has been noted. CONCLUSION: Primary cutaneous adenoid cystic carcinoma, an uncommon tumor of the skin, should be added to the differential diagnosis of eyelid tumors.     

Adenoid cystic carcinoma with orbital and cranial metastases: case report.

Adenoid cystic carcinoma of the paranasal sinuses was demonstrated in a patient who subsequently developed a metastatic lesion to both orbits resulting in total blindness. While there are reports of extension of adenoid cystic carcinoma of the lacrimal gland to the adjacent orbit, this patient is an example of orbital extension of an adenoid cystic carcinoma of minor salivary gland originating in a paranasal sinus. The frequency and classification of this tumor also is reviewed.     

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.     

Adenoid cystic carcinoma of the superonasal conjunctival fornix

The case of a 32-year-old female with a mass in the superonasal conjunctival fornix is described. Histological examination revealed an adenoid cystic carcinoma, which we consider to be most likely derived from an accessory lacrimal gland. The clinical presentation, histological features, management and follow-up are described. A literature review of orbital adenoid cystic carcinoma arising outside the main lacrimal gland is presented.     

Adenoid cystic carcinoma of the superonasal conjunctival fornix

The case of a 32-year-old female with a mass in the superonasal conjunctival fornix is described. Histological examination revealed an adenoid cystic carcinoma, which we consider to be most likely derived from an accessory lacrimal gland. The clinical presentation, histological features, management and follow-up are described. A literature review of orbital adenoid cystic carcinoma arising outside the main lacrimal gland is presented.     

Choroidal metastasis from adenoid cystic carcinoma of the lung

PURPOSE: We report a case of a choroidal metastasis from an adenoid cystic carcinoma of the lung.Interventional case report. DESIGN: A 40-year old man, 9 months' status postresection of a bronchial adenoid cystic carcinoma, was diagnosed by clinical evaluation and fine-needle aspiration biopsy and treated with palladium-103 ophthalmic plaque brachytherapy. RESULTS: This unusual patient with uveal metastasis from adenoid cystic carcinoma presented with decreased vision in the right eye and a diaphanous amelanotic choroidal tumor in the superotemporal macula. Ultrasound revealed a dome-shaped tumor that measured 4.0 mm in apical height and 11 x 10 mm in basal diameter. Fluorescein angiography revealed a double circulation and late intense subretinal fluorescence. Plaque brachytherapy provided local control and preservation of the eye. CONCLUSION: Bronchogenic adenoid cystic carcinoma can metastasize to the choroid.     

Plaque radiotherapy for selected orbital malignancies: preliminary observations: the 2002 Montgomery Lecture,part 2

PURPOSE: To describe the principles and preliminary results of plaque brachytherapy for selected orbital malignancies. METHODS: A custom-designed Iodine-125 plaque, designed to deliver a target dose of 50 Gray, was placed surgically in the region from which an orbital malignancy was partially resected. The mean dose to the target area was 50 Gray. The initial and follow-up patient data were reviewed. RESULTS: Of the 8 patients, the diagnosis was adenoid cystic carcinoma of the lacrimal gland (4 cases), orbital invasion by basal cell carcinoma (2), orbital extension of conjunctival melanoma (1), and metastatic carcinoma (1). Of the 4 with adenoid cystic carcinoma, there was microscopic residual tumor after excision and orbital exenteration was considered. Three have tumor control with follow-up of 1, 3, and 6 years. One patient required exenteration for recurrence separate from the field of brachytherapy and is free of tumor after 10 years. All 4 patients are alive and well with tumor control. Of the 2 patients with orbital extension of basal cell carcinoma, tumor control without recurrence has been achieved in both after 2 years. The patient with orbital metastasis responded to plaque radiotherapy, with no orbital recurrence, but died of systemic metastasis. The patient with orbital melanoma had local orbital recurrence separate from the area of irradiation and is currently being treated for systemic metastasis. CONCLUSIONS: Based on preliminary observations, plaque radiotherapy appears to be a reasonable alternative to exenteration and external irradiation for selected orbital malignancies.     

Two cases of orbital adenocarcinoma treated with heavy charged carbon particle irradiation

Background Orbital adenocarcinoma is a relatively rare, primary orbital malignant epithelial tumor, and shares the poor prognosis of orbital adenoid cystic carcinoma. We report the cases of two patients with orbital adenocarcinoma who were treated with heavy charged carbon particle irradiation and followed up for more 6 years.Method Two patients with orbital adenocarcinoma, 62 and 74 years old, received 57.6 GyE of heavy charged particle irradiation therapy.Results In both cases, the size of the tumor gradually decreased after carbon ion irradiation therapy. No recurrences or metastasis of the tumor were found for more than 6 years.Conclusion Orbital adenocarcinoma has a poor prognosis in general. Two patients with orbital adenocarcinoma were treated with heavy charged carbon particle irradiation therapy and had a relatively good outcome and good prognosis. We believe that heavy charged carbon particle irradiation therapy is a promising therapy for orbital adenoid cystic carcinoma and adenocarcinoma.     

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。     

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.     

超声、CT、MRI对泪腺腺样囊性癌诊断价值比较

目的：探讨泪腺腺样囊性癌X线、超声、CT及MRI的影像学特征,以及各种检查方法对疾病诊断价值的比较。方法：回顾性分析1977－1999年经病理组织学确诊的泪腺腺样囊性癌46例,对X线（8例）、B超（39例）、CDI（7例）、CT（37例）、MRI（4例）的特征进行比较。结果：X线可以发现眼眶密度增高及眶骨破坏;B超可以发现眶内占位病变,且对病变内部的钙斑、液化腔等组织结构的显示良好;CDI可以提供肿瘤内部血液供应情况;CT可以揭示病变范围、生长方式、肿物与眶内正常结构的关系、眶骨及眶周结构的改变;MRI对于显示肿瘤颅内和颞凹蔓延,优于以上检查方法。结论：联合应用多种影像学检查方法,可以提高术前诊断率。     

眼眶腺样囊性癌临床病理分析

目的：探讨眼眶腺样囊性癌（adenoid cystic carcinoma ACC)的临床特征及病理组织学特点。方法：回顾性分析20例ACC患者的临床及病理资料。结果：ACC主要表现为眶内包块（20/20），眼球突出移位（14/20），眼球运动障碍（14/20），眶周疼痛（13/20）等；病理组织学分5型；筛状型，管状型，实体型，粉刺型和硬化型。结论：ACC的临床特征有助于其诊断，最终确诊有赖于病理组织学检查。     

Salivary duct carcinoma metastatic to eyelid and orbit—a case report

BACKGROUND: Salivary duct carcinoma is a rare, extremely aggressive malignant tumor, demonstrating invasive growth with early regional and distant metastasis. We describe a case of parotid gland salivary duct carcinoma metastatic to the eyelid and orbit, as confirmed by immunohistochemical analysis. To the best of our knowledge, this is the first such case reported in the literature. METHODS: A 43-year-old male had left lower eyelid and left inferior fornix/anterior orbital masses. The patient underwent left lower eyelid and anterior orbital biopsies for histopathological evaluation. Immunohistochemical analysis for markers like androgen receptors, gross cystic disease fluid protein-15, cytokeratins, HER-2/neu, epithelial membrane antigen, S-100 proteins, progesterone receptors, and estrogen receptors were performed to establish diagnosis. RESULTS: Specimens from the eyelid and orbit revealed lobules of tumor cells exhibiting solid, micopapillary, and glandular appearance with central necrosis-comedo patterns. The tumor cells showed immunohistochemical reactivity to androgen receptor proteins, pankeratin, HER-2/neu and epithelial membrane antigen. Focal reactivity to gross cystic disease fluid protein-15 was also present. Immunoreactivity to S-100 proteins, progesterone receptors, and estrogen receptors were negative. Diagnosis was metastatic paratoid duct carcinoma to the left lower eyelid and left inferior orbit. CONCLUSIONS: Although metastatic SDC of the eyelid and orbit is a rare phenomenon, the possibility of this extremely aggressive entity should be included in the differential diagnosis when dealing with a patient with an eyelid and/or orbital tumor.     

Adenoid cystic carcinoma presenting as an orbital apex syndrome

Adenoid cystic carcinomas (cylindromas) are distinctive neoplasms characterized by slow growth, perineural spread, extensive local invasion, frequent recurrence, and high mortality. A case of paranasal sinus adenoid cystic carcinoma presenting as an orbital apex syndrome is described.     

眼眶腺样囊性癌的治疗与预后分析

目的 分析总结眼眶腺样囊性癌的治疗方法及影响预后的因素,为改善预后提供帮助.方法 采用回顾性系列病例研究,分析1991年1月至2006年7月就诊的75例眼眶腺样囊性癌患者的手术记录、病理分型及随访记录.相关数据采用χ2检验和Fisher精确检验进行统计分析.结果 眼眶实体型腺样囊性癌的2年复发率为85%(17/20)、5年复发率为100%(19/19),而腺样.管状型则分别为23.53%(8/34)和64.52%(20/31),差异有统计学意义(2年,χ2=19.14,P=0.000;5年,Fisher精确检验,P=0.003).前者发生局部蔓延和远处转移例数亦多于后者.肿瘤切除术后放射治疗的5年复发率为70%(14/20),低于单纯手术切除的复发率92.86%(13/14)(Fisher精确检验,P=0.198).首次手术行眶内容物剜除术的5年复发率为25%(1/4),低于复发后再行眶内容物剜除术的病例为75%(6/8)(Fisher精确检验,P=0.222),γ刀、粒子刀、化疗及生物治疗的效果不能确定.局部蔓延主要是至颅内、副鼻窦和颞窝,远处转移可到达肺、骨、肝、耳前淋巴结.5年远处转移率为25.71%(9/35),肺转移和骨转移各占33.33%(3/9).5年生存率74.29%(26/35),死亡率25.71%(9/35),无瘤生存率37.14%(13/35),10年无瘤生存率17.14%(6/35).最常见的死亡原因是颅内蔓延.肿瘤切除联合放射治疗可以使5年生存率提高到80%(16/20).结论 腺样囊性癌是高度恶性的眼眶肿瘤,复发率和死亡率均较高,病理分型、治疗方法均影响预后.采取综合治疗方法,可以减少复发,提高生存率.     

Metastatic adenoid cystic carcinoma of the eyelid

A 48-year-old man presented with an enlarging mass of the right upper eyelid over a 1-month period. Nine years prior, the patient was diagnosed with adenoid cystic carcinoma (ACC) of the right maxillary sinus and underwent a total maxillectomy followed by local radiotherapy. Over the previous year, the patient had been treated with chemotherapy and radiotherapy for multiple organ metastases, including the spine, chest, and oral cavity. Ophthalmic examination revealed a white, round mass on the tarsal conjunctiva of the right upper eyelid, measuring approximately 1×1cm, and 2 palpable subcutaneous masses near the inferior orbital rim. Excisional biopsies of the masses were performed, and the histopathological findings were consistent with ACC with solid and cribriform patterns. Herein, the authors describe a case of metastatic ACC of the eyelid, which has not been described in the literature to their knowledge.     

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。     

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.