Symptomatic ciliary body melanocytoma: melanocytomalytic glaucoma

CASE REPORT: A 33 year-old female with an asymptomatic pigmented mass in the iridocorneal angle of her right eye, arising from the ciliary body is presented. Ciliary body melanocytoma was suspected and conservative management recommended. After 36 months of follow-up the patient developed pain, inflammatory reaction and uncontrollable ocular hypertension, which was diagnosed as melanocytomalytic glaucoma. Tumor was removed by external iridecyclectomy and the histopathologic findings revealed necrotic melanocytoma. DISCUSSION: Ciliary body melanocytoma is a rare benign pigmented tumor that may present extension to the anterior chamber. Differential diagnosis mainly includes ciliary body melanoma, which carries a different prognosis and treatment.     

Diffuse melanocytic nevus of the iris and absolute glaucoma

BACKGROUND: Ferry et al. (8) showed that 35 % of eyes which have been enucleated because of a clinically suspected malignant iris lesion did not reveal a histologically malignant iris tumor. PATIENT: A 43-year old male presented with a blind, painful left eye, which has developed over a time period of 6 months. Ophthalmologic examination revealed the presence of pigmented iris stromal lesions associated with diffuse pigmentation of the chamber angle and the development of absolute glaucoma. A diffuse iridociliary malignant melanoma (ring melanoma) was suspected and the blind painful eye was enucleated. Histologic examination revealed the presence of a benign melanocytic lesion, i.e. a spindle cell nevus of the iris with surface plaques causing diffuse extension of the chamber angle, trabecular meshwork, Schlemm's canal, and the ciliary body. CONCLUSION: The malignant potential of iris lesions may be difficult to assess on clinical grounds only. Histologically, benign melanocytic proliferation may cause diffuse extension into the ciliary body as well as the chamber angle leading to an increase in intraocular pressure. In order to reach a final diagnosis a biopsy of the lesion is necessary.     

2010-2019年167例经局部切除术治疗的睫状体肿物的临床组织病理构成分析

目的 分析局部切除的睫状体肿物的临床组织病理类型构成。设计 回顾性病例系列。研究对象 2010-2019年北京同仁医院经局部切除术治疗的睫状体肿物167例。方法 回顾睫状体肿物的病理记录,包括一般临床信息和病理诊断。分析睫状体肿物的组织病理类型构成比。主要指标 组织病理类型构成比。结果 167例睫状体肿物中黑色素细胞瘤44例（26.3%）,无色素上皮腺瘤/腺癌32/2例（20.4%）,黑色素瘤29例（17.4%）,神经鞘瘤14例（8.4%）,平滑肌瘤12例（7.2%）,胶质神经瘤11例（6.6%）,髓上皮瘤9例（5.4%）,色素上皮腺瘤7例（4.2%）,血管瘤2例（1.2%）,炎症2例（1.2%）,色素痣、淋巴瘤和颗粒细胞病变各1例（0.6%）。其中,恶性病变占24.6%（41/167）,良性病变占75.4%（126/167）。睫状体上皮层起源的肿物占36.5%,基质起源的肿物占63.5%;上皮层起源的肿物前三位分别为无色素上皮腺瘤、髓上皮瘤、色素上皮腺瘤;基质起源的肿物前三位分别为黑色素细胞瘤、黑色素瘤、神经鞘瘤。有色素肿物和无色素肿物数量相当（49.7%和50.3%）;有色素肿物的前三位分别为黑色素细胞瘤、黑色素瘤和色素上皮腺瘤;无色素肿瘤的前三位分别为无色素上皮腺瘤、神经鞘瘤和平滑肌瘤。结论 本研究较大样本的睫状体肿物中占前三位的病理类型是黑色素细胞瘤、无色素上皮腺瘤、黑色素瘤。恶性肿瘤仅占四分之一,提示在选择治疗方案时要优先考虑肿瘤局部切除术。（眼科, 2020, 29： 391-395）     

Seborrheic keratosis of conjunctiva: a case report

CASE REPORT: Pigmented conjunctival lesions are a diagnostic challenge for the clinician. A 37-year-old man presented with a pigmented mass involving the conjunctiva of his right eye. Clinically, a diagnosis of malignant melanoma was made and a wide excision of the tumor was performed. The histopathologic diagnosis was seborrheic keratosis. DISCUSSION: Seborrheic keratosis is a benign lesion which occurs on the eyelids and face of middle-aged and elderly individuals. The occurrence of this lesion on the conjunctiva is rare, however there are 2 cases reported in the world literature. Seborrheic keratosis should be considered in the differential diagnosis of conjunctival pigmented lesions.     

Clinical and pathological status of mesectodermal leiomyoma of the ciliary body

The case of a 28-year-old woman with a well-vascularized, yellowish neoplasm in her right eye is reported. The findings upon slit-lamp examination and fluorescein iridography strongly suggested a malignant lesion. The tumor and the adjacent portion of the iris, angle structures, ciliary body, cornea, and sclera were removed en bloc under the half-thickness corneoscleral flap. On the basis of light-microscopic studies, the tumor was suspected to be a neurogenic or glial tumor but further studies confirmed it to be a mesectodermal leiomyoma of the ciliary body. Electron-microscopic studies revealed the smooth muscle nature of the tumor. Because of the high percentage of benign lesions in this site, we believe that even though examinations may suggest malignancy, excision of the tumor should be preferred to enucleation of the globe in cases of ciliary tumor.     

Borderline spindle cell nevus of the ciliary body as a second primary melanoma--a clinicopathologic case

We present the case of a 22-year-old woman who underwent surgical excision of a malignant melanoma of the skin three years previously and whom we saw for a pigmented tumor of the iridocorneal angle of her right eye associated with end-stage secondary glaucoma. After the clinical diagnosis of ring melanoma, she underwent enucleation of the eye. The pathologic examination showed a lesion predominantly appearing as a nevus with areas of borderline melanoma formation. The main features of this case were the young age of the patient, the predominant nevoid appearance of this diffuse ciliary body tumor with ring configuration, and the fact that it is a second primary melanoma in such a young patient.     

Lacrimal Gland Choristoma of the Ciliary Body

Background

Lacrimal gland choristoma manifesting in the ciliary body is quite rare. We report a case of lacrimal gland choristoma manifesting in the ciliary body, coupled with orbital cellulites, in an infant.

Case

A 10-month-old female infant with swelling and tenderness of the right upper eyelid.

Observations

The results of ophthalmic examinations of the patient were consistent with orbital cellulitis. During the funduscopic examination, a fleshy mass was incidentally detected at the superotemporal portion of the ciliary body in the right eye. A local resection of the lesion was carried out. The results of a pathologic examination showed lacrimal gland choristoma of the ciliary body that was accompanied by an epithelia-lined cyst. The patient has had no hypotonia or enlargement of the lesion for 1 year after surgery.

Conclusion

Lacrimal gland choristoma should be included in the differential diagnosis of a ciliary body mass in pediatric patients.?Jpn J Ophthalmol 2006;50:100–102 © Japanese Ophthalmological Society 2006     

Ciliary body schwannoma.

PURPOSE: Intraocular schwannomas are very rare, benign, peripheral nerve neoplasms. The authors report a case of ciliary body schwannoma. METHOD: A 39-year-old Korean woman presented with slowly decreasing visual acuity and proptosis of the right eye for 4 years. At the time of her visit, the eyeball protruded and deviated laterally. She could not sense light with her right eye. We enucleated the eyeball. The enucleated eyeball with tumor was examined histopathologically, immunohistochemically, and under electron microscope. RESULTS: Microscopic examination revealed spindle cells and characteristic Antoni type A and B areas with Verocay bodies. Immunohistochemical study showed tumor cell expressions of S-100 and vimentin, but other immunohistochemical studies were negative. Electron microscopic examination demonstrated Luse body. CONCLUSIONS: We diagnosed the tumor as a schwannoma arising from the ciliary body on the basis of the above mentioned results. Schwannomas are very rare intraocular neoplasms, but they are benign so we should differentiate from other intraocular neoplasms.     

Lacrimal gland choristoma of the ciliary body

A 22-month-old girl was found to have a pigmented mass of the ciliary body of the right eye. Five months later the globe was enucleated because of enlargement of the tumor and the appearance of brown pigment on the adjacent epibulbar surface. Histopathologic examination disclosed a lacrimal gland choristoma of the ciliary body with involvement of the limbal conjunctival surface and superficial sclera. Choristomas of the lacrimal gland are rare and only eight cases have been reported previously to occur intraocularly. Most of these cases showed extrascleral involvement which suggests that the anlage for these choristomas may be entrapped intraocularly during the development of the globe. We recommend conservative management, either by observation or local resection if possible.     

Cystic adenoma of the pigmented ciliary epithelium. Clinical, pathologic, and immunohistopathologic findings.

A 51-year-old white man was found to have a deeply pigmented mass in the ciliary body and peripheral choroid of his right eye with an associated vitreous hemorrhage. Although the tumor appeared to be a ciliochoroidal melanoma, a melanocytoma and adenoma of the pigment epithelium also were considered in the differential diagnosis. The tumor was removed by a large partial lamellar sclerocyclochoroidectomy. Results of histopathologic evaluation showed a cystic adenoma of the pigmented ciliary epithelium. To the authors' knowledge, this was the first immunohistopathologic study of this tumor. Results of the study showed marked immunoreactivity for low molecular weight cytokeratins, vimentin, and S-100 protein. These immunohistochemical studies are consistent with the origin of this tumor from pigment epithelial cells.     

睫状体肿瘤局部切除联合玻璃体视网膜手术的疗效分析

目的：探讨睫状体肿瘤局部切除联合玻璃体视网膜手术的疗效、安全性及适应证。方法：12例睫状体肿瘤患者,均经超声生物显微镜、B超等辅助诊断。其中男性5例,女性7例;年龄13－48岁,平均32岁;肿瘤直径5－20mm,厚4-12mm;2例合并视网膜脱离。采用全身低血压麻醉或局麻法,行局部层巩膜睫状体或局部板层巩膜睫状体前脉络膜切除联合玻璃体视网膜手术及异体巩膜移植术,包括晶状体和玻璃体切除、过氟化碳液体应用、眼内光凝、气体或硅油充填联合巩膜环扎术。切除的肿瘤送病理检查。 结果：术后随访7－38个月,平均14．2个月,未发现肿瘤复发与转移。末次随访时,视力＜0．05者1例,0．05－0．2者3例,0．3－1．0者8例。病理诊断：睫状体恶性黑色素瘤5例,黑色素细胞瘤2例,无色素上皮瘤3 例,神经纤维瘤1例,胶质瘤1例。结论：睫状体肿瘤局部切除不仅能保存患眼、挽救视力,而且可对切除组织进行病理检查,以明确诊断;联合玻璃体视网膜手术可减少术后并发症,提高手术成功率;局部板层巩膜睫状体切除联合玻璃体视网膜手术是治疗睫状体良、恶性肿瘤的安全、有效方法之一。     

Ciliary body adenoma after application of mitomycin

PURPOSE: To describe a ciliary body tumor that was detected in a rabbit eye. METHODS: For a specific experiment, the rabbit underwent trabeculectomy with mitomycin. The eye was evaluated by electron microscopy 5 weeks following surgery. RESULTS: A ciliary body tumor was discovered in close relation to pigmented epithelial cells. The tumor cells were large with a small amount of pigment and contained prominent vesicles. CONCLUSIONS: The development of a ciliary body adenoma may be caused by the application of mitomycin. Whether the demonstrated tumor was benign or malignant could not be determined. While it could not be proven that the tumor developed related to the use of mitomycin, this possibility may be important for surgeons using this substance.     

Occult foreign body simulating a choroidal melanoma with extrascleral extension

A 62-year-old man was noted on routine examination to have a dark lesion in the peripheral fundus of the right eye and a corresponding dark scleral mass. The lesion was initially suspected to be a choroidal melanoma with extrascleral extension. The patient denied having ocular trauma. Orbital x-rays and ultrasonography, however, demonstrated the lesion to be an occult transcleral metallic foreign body. An occult foreign body should be considered in the differential diagnosis of a small choroidal or ciliary body melanoma with extrascleral extension.     

Diffuse uveal melanoma. A clinical case

CASE REPORT: We report the case of a 58-year-old man who presented at our emergency department with ocular pain and progressive loss of vision in his left eye. Ophthalmic examination detected a pigmented mass in the iridocorneal angle, involving the ciliary body. Glaucoma had also developed secondary to the melanotic lesion. Ophthalmoscopy showed a large pigmented choroidal tumor. After enucleation, pathologic examination confirmed the diagnosis of a diffuse ocular melanoma. DISCUSSION: Diffuse uveal melanoma is a malignant tumor that metastasises early. Enucleation is the treatment of choice.     

Uveal prolapse following cataract extraction simulating melanoma

Prolapsed uveal tissue through a cataract incision can simulate any pigmented epibulbar mass, including conjunctival melanoma, extraocular extension ofa ciliary body or choroidal melanoma, and pigmented squamous cell carcinoma of the conjunctiva. The authors describe an 88-year-old woman who presented with an enlarging pigmented epibulbar mass on the left eye. Although the lesion closely simulated a conjunctival melanoma or extraocular extension of a uveal melanoma, closer evaluation revealed thin uveal tissue extending through partial wound dehiscence from cataract surgery that was performed 3 years earlier. The lesion was consistent with prolapsed uveal tissue through a cataract wound masquerading as a melanoma. Therefore, uveal prolapse should be considered in the differential diagnosis of conjunctival melanoma or extraocular extension of uveal melanoma.     

Progressive enlargement of cavity within melanoma masquerading as iris cyst

PURPOSE: To describe progressive enlargement of intralesional cavities within a mass that masqueraded clinically as an iris pigment epithelial cyst for 2 years but later proved to be a melanoma. METHODS: An 81-year-old woman developed asymptomatic focal elevation of the iris, and ultrasound biomicroscopy showed a fluid-filled mass that was interpreted as an iris pigment epithelial cyst. Over a 2-year period of observation, the cavity enlarged from 0.7 to 10 mm, and the solid component enlarged from approximately 6 to 16 mm basal dimension. RESULTS: On referral, visual acuity was 20/400 in the right eye and 20/25 in the left eye. The affected right eye displayed a pigmented mushroom-shaped ciliary body mass with anterior chamber invasion, distorting the pupil and occupying 40% of the anterior chamber. Ultrasound biomicroscopy and B-scan ultrasonography revealed many cavities within the 14-mm-thick mass, suggestive of ciliary body melanoma. After enucleation, histopathology disclosed multiple cavities, some with granular eosinophilic material, within a mushroom-shaped mixed-cell melanoma. CONCLUSION: Cavitation can occur in ciliary body melanoma and can lead to misinterpretation as a cyst. Cavitary melanoma should be considered in the differential diagnosis of cystic lesions of the anterior segment.     

Ciliary body melanocytoma with anterior segment pigment dispersion and elevated intraocular pressure

PURPOSE: To discuss a case in which melanocytoma of the ciliary body presented with widespread pigment dispersion and elevated intraocular pressure (IOP). METHODS: A 64-year old woman presented with increased IOP and persistent anterior segment inflammation in her right eye. She had undergone a trabeculectomy for uncontrolled IOP before presentation. Slit-lamp examination, gonioscopy, and ultrasound biomicroscopy revealed a ciliary body mass with extension into the subconjunctiva in addition to widespread pigmentary dispersion in the anterior segment. Enucleation of the right eye was performed for histopathologic evaluation, as a diagnosis of ciliary body melanoma was made on clinical examination. RESULTS: Histopathologic findings were diagnostic of a melanocytoma of the ciliary body with necrosis and focal malignant transformation with extension of melanocytoma cells and melanophages into the subconjunctival space, trabecular meshwork, and anterior chamber angle. CONCLUSION: Melanocytoma of the ciliary body is a rare intraocular tumor that may present with pigment dispersion and secondary elevated IOP. Careful examination of the anterior segment is imperative in such cases.     

Transscleral resection of a ciliary body leiomyoma in a child: case report and review of the literature

Purpose To present the case of a patient with leiomyoma of the ciliary body and discuss the histological features and treatment of this rare intraocular tumor.Methods/case report A 13-year-old boy presented with an asymptomatic tumor of the right eye. Visual acuity was 20/20 in both eyes. Ophthalmoscopy revealed an amelanotic, vascularized ciliary body tumor with exudative retinal detachment and partial transillumination. On ultrasound examination the tumor height was 8 mm and a low internal reflectivity was found. T2-weighted MRI scans showed a hypointense and T1-weighted scans a hyperintense intraocular mass with significant Gd-TPA enhancement. On the assumption that the diagnosis was consistent with an amelanotic ciliary body melanoma, a transscleral resection with adjuvant ruthenium-106 brachytherapy was performed. Visual acuity was 20/40 at 6 months after the operation.Results Routine stains revealed a pleomorphic tumor composed mainly of spindle cells with palisading in some areas and a prominent intercellular fibrillary background. Immunohistochemistry showed positivity for desmin, vimentin and actin. No reactivity with S-100 and HMB-45 was seen. Intracytoplasmatic filaments and micropinocytotic vesicles were detected by transmission electron microscopy. These findings were consistent with the diagnosis of a ciliary body leiomyoma.Conclusion Typical clinical features of leiomyoma include a dome-shaped configuration and translucency, but the final diagnosis can only be confirmed by histology with the aid of immunohistochemistry and electron microscopy. Though rare, leiomyoma should be considered in the differential diagnosis of amelanotic uveal tumors. Transscleral resection is the treatment of choice of anterior uveal leiomyomas, with a fairly good visual prognosis.     

Graphite foreign body of the conjunctiva simulating melanoma

A case of a growing vascular pigmented mass of the conjunctiva resembling a melanoma in a patient with a history of a pencil injury to the eye is presented. The lesion was surgically removed and histopathologically found to be a graphite foreign body granuloma. In the differential diagnosis of enlarging pigmented conjunctival lesions, graphite foreign body granuloma should be considered.     

Adenoma of the nonpigmented epithelium of the ciliary body

A 42-year-old woman developed a ciliary body mass which indented the lens equator and produced a dense focal cataract in the right eye. The clinical diagnosis was malignant melanoma of the ciliary body, and the lesion was removed by a large iridocyclectomy. Histopathologic examination revealed an acquired adenoma of the non-pigmented ciliary epithelium. The clinical and histopathologic features of this rare intraocular tumor are discussed with emphasis upon its differentiation from malignant melanoma.