Primary cutaneous adenoid cystic carcinoma of the upper lid: a case report and literature review

To report on the diagnostic challenge of an adenoid cystic carcinoma arising from the eyelid. A 77-year-old male was referred to our center with a clinical diagnosis of upper eyelid chalazion for a lesion that had appeared 2 years before. A loss of cilia was observed over the cutaneous area of induration, but there was no reddening or ulceration. Incisional biopsy was performed and the specimen was submitted in formalin for histopathological examination. On light microscopy, the lesion was composed of basaloid epithelial and myoepithelial cells that were arranged in strands or nests and associated with cystic spaces that contained a deeply eosinophilic secretory substance and an Alcian blue-positive material, characteristic of adenoid cystic carcinoma. After histological diagnosis, tumor re-excision was performed to ensure adequacy of resection margins, as well as a sentinel lymph node procedure, resulting in complete excision of the malignant tumor. No recurrence was observed during the first 18 months after surgery. Adenoid cystic carcinoma is a rare and aggressive epithelial malignancy, which tends to grow slowly and should be considered in the differential diagnosis of eyelid tumors simulating chalazion.     

Adenoid Cystic Carcinoma Arising from the Lower Eyelid

Background

Primary cutaneous adenoid cystic carcinoma is one of the rarest eyelid tumors.

Case

A 39-year-old man presented with a small subcutaneous induration in his left lower eyelid close to the lower lacrimal punctum.

Observations

Under a clinical diagnosis of chalazion, the eyelid induration was extracted surgically via the palpebral conjunctiva. Histopathological examination of the extracted tumor revealed proliferation of basaloid cells with a prominent cribriform pattern containing Alcian blue-positive and periodic acid Schiff-positive material, leading to a diagnosis of adenoid cystic carcinoma. The patient subsequently underwent extended radical excision including the lacrimal sac. No recurrence was observed during 20 months after surgery.

Conclusions

Adenoid cystic carcinoma should be considered as a differential diagnosis of eyelid tumors simulating chalazion. Adenoid cystic carcinoma in the eyelid may have a more favorable prognosis than that in the lacrimal gland.?Jpn J Ophthalmol 2006;50:374–376 © Japanese Ophthalmological Society 2006     

Primary adenoid cystic carcinoma: an extremely rare eyelid tumor

Cutaneous adenoid cystic carcinoma is an extremely rare clinical entity. Among the few cases reported in the literature, most had contiguous involvement from the lacrimal gland. Primary adenoid cystic carcinoma is one of the rarest eyelid tumors. The authors report a case of adenoid cystic carcinoma arising from the lower eyelid.     

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.     

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 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.     

Reconstruction Following Subtotal Full-Thickness Upper Eyelid Resection with Preservation of the Lid Margin

Purpose: To describe a modification of a technique for upper eyelid reconstruction previously described by Patrinely et al. Methods: Radical excision of a large adenoid cystic carcinoma of the upper eyelid resulted in a subtotal full-thickness eyelid defect with preservation of 2 mm of eyelid margin. Reconstruction was performed with a free transconjunctival graft of maximum size from the contralateral upper eyelid, an overlying bipedicled skin-orbicularis oculi muscle flap and a free skin graft. Results: With this one-step technique, a good cosmetic and functional result could be obtained. Conclusion: If the eyelid margin can be spared, reconstruction of even very large upper eyelid defects with a modification of the technique reported by Patrinely et al. may yield a good result.     

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.     

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.     

常见原发性泪腺上皮性肿瘤的组织病理学特征与复发的关系

目的分析原发性泪腺上皮性肿瘤的组织病理学特征与复发的关系,为临床诊治提供参考。方法收集128例病理诊断为原发性泪腺上皮性肿瘤患者的石蜡标本,其中最常见的三种依次为良性混合瘤74例（57．8％）,腺样囊性癌22例（17．2％）,恶性混合瘤18例（14．1％）,分析其组织病理学特征与复发的关系。结果良性混合瘤、腺样囊性癌和恶性混合瘤的复发率分别为23．0％、18．2％和27．8％,其中良性混合瘤的病理类型和包膜完整性在肿瘤的复发中具有统计学意义。结论三种常见原发性泪腺上皮性肿瘤复发率高,了解其病理表现对肿瘤的诊断、治疗方式的选择和随访时间的确定具有参考意义。     

Metastatic Merkel cell carcinoma to the eye

Merkel cell carcinoma is an infrequent but highly aggressive cutaneous neoplasm. Previous reports of ocular involvement have included primary eyelid carcinomas, eyelid and orbital metastases, and a clinically diagnosed choroidal metastasis. The authors report the histopathologic documentation of a metastatic lesion to the ciliary body, confirming the intraocular metastatic potential of this tumor.     

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。     

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。     

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.     

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.     

Primary malignant neoplasms of the lacrimal gland.

The clinical characteristics and outcome of 50 primary malignant neoplasms of the lacrimal gland are reviewed: 38 (76%) adenoid cystic carcinomas, six (12%) carcinomas arising in pleomorphic adenoma, and six (12%) adenocarcinomas or other types of carcinoma. Most patients presented with a short history and pain, though pain tended to occur less often and later with adenocarcinoma than with adenoid cystic carcinoma. Pain was unrelated to the duration of symptoms, invasion of bone, loss of trigeminal nerve function, or the frequency and time of tumour recurrence. The estimated disease-free survival for patients with adenoid cystic carcinoma was significantly (p less than 0.01) reduced where half or more of the biopsy specimen showed basaloid differentiation. Eleven patients underwent extended cranio-orbital resection, and the others received a combination of total dacryoadenectomy adenectomy and/or radiotherapy. Survival after adenoid cystic carcinomas appears to be significantly (p less than 0.05) greater when tumour resection is combined with radiotherapy than after radiotherapy alone. At present, however, the rate of disease-free survival after treatment of adenoid cystic carcinoma appears unaltered by cranio-orbital resection, though these latter patients form a relatively greater proportion of those surviving for more than 10 years. Further long-term follow-up is needed to see if this technique does influence survival.     

泪腺恶性肿瘤—附27例报告

本文报告泪腺恶性肿瘤27例(其中腺样囊性癌12例,恶性多形性腺瘤9例,其它腺癌6例)。讨论和分析了临床表现、影象诊断特征、诊断、鉴别诊断、治疗和予后问题。     

Primary mucinous carcinoma of eyelid: A rare clinical entity

Primary cutaneous mucinous carcinoma of the eyelid, a rare pathologic entity, is an adenocarcinoma of the eccrine glands. Though it has low metastatic potential, it does have a significant recurrence rate. We present the occurrence, clinical and histological features, and management of this tumor in a 62-year-old male who presented with a recurrent, firm, nodular left lower lid lesion. He underwent excision with a 5 mm margin and the defect was repaired with a Mustarde''s cheek rotation flap. A full oncological screening, including whole-body Positron Emission Tomography scan, excluded the presence of primary mucinous carcinoma elsewhere and any metastatic spread. This case underscores the importance of considering this tumor in recalcitrant eyelid lesions and highlights the pathology of this tumor.     

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.     

Pathology of eyelid tumors

The eyelids are composed of four layers: skin and subcutaneous tissue including its adnexa, striated muscle, tarsus with the meibomian glands, and the palpebral conjunctiva. Benign and malignant tumors can arise from each of the eyelid layers. Most eyelid tumors are of cutaneous origin, mostly epidermal, which can be divided into epithelial and melanocytic tumors. Benign epithelial lesions, cystic lesions, and benign melanocytic lesions are very common. The most common malignant eyelid tumors are basal cell carcinoma in Caucasians and sebaceous gland carcinoma in Asians. Adnexal and stromal tumors are less frequent. The present review describes the more important eyelid tumors according to the following groups: Benign and malignant epithelial tumors, benign and malignant melanocytic tumors, benign and malignant adnexal tumors, stromal eyelid tumors, lymphoproliferative and metastatic tumors, other rare eyelid tumors, and inflammatory and infections lesions that simulate neoplasms.