Ciliary Body Mass Research Articles

A Case of Bilateral Diffuse Uveal Melanocytic Proliferation Followed by Massive Unilateral Uveal Proliferation

ABSTRACT Objective To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) followed by massive unilateral uveal proliferation. Methods Retrospective case report. Results A 47-year-old female with history of metastatic ovarian carcinoma initially presented with bilateral vision loss and multifocal red patches on posterior poles consistent with BDUMP. Five years later, she presented with bilateral neovascular glaucoma and unilateral iris and ciliary body mass concerning for malignancy. Enucleation revealed diffuse uveal growth involving almost the entirety of the uveal tract. Conclusions BDUMP can rarely be associated with uveal proliferation. Routine examinations are recommended to monitor for any changes concerning malignancy.

Intraocular medulloepithelioma clinical features and management of 11 cases

AimsTo describe the clinical features, imaging characteristics, histopathology, treatment and outcomes of intraocular medulloepithelioma.MethodsMedical records of 11 patients with clinically or histopathologically confirmed medulloepithelioma were retrieved and reviewed. Clinical features,...

Analysis of clinical and pathological features of ciliary body medulloepithelioma.

To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma. The clinical and pathological data of 11 patients (11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital, Capital Medical University, from 2007 to 2021 were retrospectively analyzed. The initial symptoms of 11 patients included vision loss (6 eyes), atrophia bulbi (1 eye), proptosis (2 eyes), and leukocoria (2 eyes). Most patients suffered with corneal opacity, anterior chamber flare and hyphema. Iris neovascularization and synechia, complicated cataract, and secondary glaucoma occurred in several cases. Three patients even had lens subluxation and retinal detachment. B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball. Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces. All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence. In this study, 6 patients had enucleation (2 patients had systemic chemotherapy after surgery), and the other 5 patients had local tumor resection (1 patient had plaque radiotherapy after surgery). Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma. Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma, and can make an accurate diagnosis and better treatment.

Ciliary Body Nevus with Episcleral Extension in a 91-Year-old Patient

A 91-year-old White woman was referred to the Wills Eye Hospital Ocular Oncology Service for evaluation of a pigmented scleral lesion right eye (OD). Per patient history, the lesion was stable for 25 years. Slit-lamp examination showed well-circumscribed episcleral pigmentation OD measuring 3.0 mm in diameter (A). Anterior-segment OCT revealed an underlying ciliary body mass with intrascleral invasion (B, white arrow), measuring 5.6 mm in diameter on ultrasound biomicroscopy and with minor extrascleral component (C, yellow arrow). A diagnosis of transcleral ciliary body nevus with episcleral extension was made. Observation was recommended. Extrascleral extension is a serious feature of ciliary body melanoma, but the remarkable chronicity of this lesion over 25 years was more suggestive of a benign entity (Magnified version of Figure A-C is available online at www.aaojournal.org).

Uveal Melanoma and Paraneoplastic Perivascular Dermal Melanocytic Proliferation in the Setting of Bilateral Diffuse Uveal Melanocytic Proliferation: The Potential Role of the Hepatocyte Growth Factor/c-Met Axis in Their Pathogenesis

Two patients, with non-small cell lung carcinoma treated with pembrolizumab, developed bilateral diffuse uveal melanocytic proliferation (BDUMP) with interesting histopathological features. The first patient developed a right ciliary body mass concurrently with BDUMP. The globe was enucleated. The ciliary body mass was a mitotically active epithelioid uveal melanoma, invading the trabecular meshwork and peripheral corneal stroma, with over 90% of the cells expressing Cyclin D1 protein. The melanoma showed no chromosome 3 or 8 changes. The background uvea showed diffuse, bland spindle cell melanocytic proliferation with much lower Cyclin D1 expression (around 10%). In the choroid, this population was punctuated by islands of pigmented epithelioid cells, some of which were necrotic. All these islands expressed a high level of Cyclin D1, and some islands expressed nuclear preferentially expressed antigen in melanoma (PRAME). The ciliary body mass, epithelioid cell islands, and the BDUMP all expressed c-Met (the receptor for hepatocyte growth factor [HGF]). The features were those of ciliary body melanoma and choroidal melanoma “tumorlets,” developing on a background of BDUMP. The second patient developed bilateral periocular skin pigmentation following a diagnosis of BDUMP, which when biopsied, showed dermal islands of paraneoplastic perivascular melanocytic cell proliferation. These cells also expressed c-Met protein. These observations implicate the HGF/c-Met axis in the pathogenesis of BDUMP, the uveal melanomas in the ciliary body and choroid in the first patient and the paraneoplastic dermal melanocytic proliferation in the second patient.

External Beam Radiation Therapy for Metastatic Tumor to the Iris: A Case Report and Brief Literature Review

Tumor metastases involving the iris are rare and represent about 3% of metastases to the uveal tract. The typical presentation is a patient with a known history of carcinoma who develops blurry vision, ocular pain, and/or eye redness, which may be erroneously diagnosed as uveitis. In this case, we report the workup, diagnosis, and treatment of a 76 year old man with a history of node negative multi-focal adenocarcinoma of the lung who was found to have a left iris metastasis. He presented with blurry vision and left eye pain. Exam revealed a 7 x 7 mm amelanotic ciliary body mass, which was ultimately biopsied and found to be consistent with his lung primary. He was treated with external beam radiation therapy, 3000cGy in 10 fractions, to the left globe. His three and nine-month follow up MRIs showed good treatment response and he is clinically without disease progression. EBRT is a safe and effective modality for the treatment of iris metastases.

Just another metastatic carcinoid tumour to the uveal tract

We describe the clinic, image, and histopathologic features of a well differentiated neuroendocrine carcinoma (carcinoid tumour) metastatic to choroid and ciliary body in a 52-year-old Mexican Mestizo man. The ophthalmologic examination showed an inferior choroidal mass accompanied by exudative retinal detachment. Ultrasound B-Scan study revealed a diffuse thickened choroid with overlying serous retinal detachment, ultrasound A-Scan revealed a high internal reflectivity solid lesion. Ultrasound biomicroscopy (UBM) evidenced a dome shaped ciliary body mass, presumptive diagnosis was uveal tract metastatic disease. Scleral flap choroidal incisional biopsy was performed. Microscopic evaluation demonstrated a hypercellular lesion replacing choroid, composed by cohesive oval-round cells with finely granular chromatin arranged in organoid pattern. Immunohistochemical reactions were Pankeratin AE1/AE3 (+), Cytokeratin CK5/6 (+), Chromogranin A (+), Ki67 (20%), typical well differentiated neuroendocrine carcinoma (carcinoid tumour) was diagnosed. Patient had a mediastinal carcinoid diagnosed 3 years earlier. Metastatic cancer to the eye is perhaps the leading cause of intraocular tumour, despite this fact metastases are rarely seen by the ophthalmologist while the patient is alive. Intraocular metastasis should be considered in the presence of ciliary body or/and choroidal amelanotic or pigmented mass and serous retinal detachment in a patient with history of carcinoid tumor, althought its low frequency (2.2%).

Case report of ring ciliary body and choroidal lymphoma

PurposeThe aim of the study was to analyze case of ring ciliary body and choroidal lymphoma.MethodsRetrospective analysis.Results54 years old man was sent in October 2014 to Department of Ophthalmology of the Jagiellonian University Medical College in Cracow with diagnosis of choroidal melanoma in left eye. Tumor was detected accidently. BCVA in both eyes was 1.0. Intraocular pressure in both eye was correct. In patients history stomach lymphoma treated in 1996 with radiotherapy and chemiotherapy. In fundus examination we found mass located temporally in choroid and cilary body. Ultrasound examination (USG and UBM) showed ciliary body and choroidal mass located between 2 and 7 clock hour (almost 180 degrees), thickness 8.2 mm, base 14.30 mm. The patient was qualified to biopsy of ciliary body and choroidal mass and I‐125 brachytherapy. During 2 weeks tumor enlarged, was located in 270–300 degrees and looks like ciliary body ring melanoma (thickness 9.0 mm, base 23.3 mm) with iris elevation and conjunctival edema. Enucleation of left eyeball was performed. Histopathological examination reveled neoplasma malignum probabiliter non epitheliale. Lypmoma malignum – marginal zone lymphoma. Immunohistochemical profile of tumor showed experesion CD20+, CD5‐, CD23‐, CD3‐, lack of expression lambda gains.ConclusionsLypmoma malignum – marginal zone lymphoma may occur like ciliary body and choroidal ring which should be considered in the differential diagnosis of intraocular tumors.

Pseudotumor of ciliary body.

Orbital pseudotumor is a benign disease involving the orbital structures. Pseudotumor of the ciliary body is rare. We present a case of a 27-year-old male who presented with gradual visual loss, pain, and redness in his left eye. On examination he was found to have a yellowish white mass at the periphery of anterior chamber in his left eye and ultrasound biomicroscopy (UBM) revealed a ciliary body mass in the same eye. He was treated with systemic steroids, which was tapered over a period of 8 weeks. His symptoms improved and the ciliary body mass disappeared with no recurrence over the next 6 months. UBM is an important diagnostic tool for diagnosing ciliary body mass. Early diagnosis and prompt treatment with systemic steroids may help resolve pseudotumor of the ciliary body.

Multicavitary ciliary body melanoma presenting as a cyst

Cyst-like cavities in uveal melanoma occur rarely and can simulate a benign intraocular cystic lesion resulting in delayed diagnosis and inappropriate management. Herein, we describe a 66-year-old Caucasian female who presented with a “cystic” ciliary body mass in the right eye oculus dexter (OD). Slit lamp examination OD showed anterior bulging of the iris temporally from an underlying pigmented ciliary body mass and transillumination disclosed slight shadow from the tumor. Ultrasound biomicroscopy (UBM) revealed multiple cyst-like cavities within a tumor, lined by “thick walls” of at least 200 μm and occupying 80% of the tumor volume. A clinical diagnosis of multi-cavitary ciliary body melanoma was suspected and partial lamellar sclero iridocyclectomy was performed. Histopathology confirmed the diagnosis of low-grade spindle melanoma of the ciliary body with multiple empty and fluid filled cyst-like cavities without epithelial lining. UBM is an important diagnostic tool in the differentiation of “thick walled” cavitary melanoma from “thin walled” benign pigment epithelial cyst.

EMBYRONAL CARCINOMA OF TESTIS METASTATIC TO CILIARY BODY PRESENTING AS SPONTANEOUS HYPHEMA AND PAINFUL SECONDARY GLAUCOMA

To report a rare presentation of metastatic testicular cancer to the uvea. Observational case report. A 24-year-old man without known systemic illness presented with acute total loss of vision and severe pain in his left eye and associated nausea, anorexia, and dehydration. He was found to have a total hyphema, high intraocular pressure, and a ciliary body mass (by computed tomography scan and ultrasound biomicroscopy). The affected eye was enucleated, and pathologic study showed a hemorrhagic poorly differentiated neoplasm of the ciliary body. Subsequent patient evaluation revealed a primary testicular embryonal carcinoma with widespread metastasis. This case represents the first report of a metastasis from a testicular carcinoma to the ciliary body and the first tumor of this type to be associated with spontaneous hyphema.

Unusual multifocal pigmented lesions of the uvea in a patient with ocular melanocytosis.

To report findings of unusual multifocal pigmented lesions in a single patient with sector uveal melanocytosis. Retrospective case report of an otherwise healthy 64-year-old man with unusual uveal pigmentation. Complete ocular examination, including slit-lamp biomicroscopy, ophthalmoscopy, optical coherence tomography, and ultrasound biomicroscopy, was performed. Slit-lamp examination disclosed inferior sector pigmentation of the iris. Ophthalmoscopy showed multiple discrete, deeply pigmented, inferior choroidal lesions, and ultrasound biomicroscopy confirmed a small inferior ciliary body mass. The findings were consistent with sector uveal melanocytosis, believed to be the same as a diffuse melanocytoma. Melanocytoma is typically a unilateral, unifocal lesion that occurs on the optic disc but can be located anywhere in the uveal tract. This is a case of unusual pigmented uveal lesions, likely representing a variant of sector uveal melanocytosis or multiple diffuse uveal melanocytomas.

The Masquerades of a Childhood Ciliary Body Medulloepithelioma: A Case of Chronic Uveitis, Cataract, and Secondary Glaucoma

Ciliary body medulloepitheliomas in childhood often masquerade other intraocular conditions due to its insidious nature as well as its secondary effects on proximal intraocular tissues in the anterior chamber. We report a case where a ciliary body medulloepithelioma in a two-year-old boy presents with chronic uveitis, cataract, and an uncontrolled secondary glaucoma after an innocuous blunt ocular trauma. The diagnosis was only made after the occurrence of a ciliary body mass. We discuss the clinical features of ciliary body medulloepitheliomas, the implications of a delayed diagnosis and treatment as well as the concern of periorbital tumor seeding with the use of an aqueous shunt implant in this case.

Renal cell carcinoma metastasis to the ciliary body responds to proton beam radiotherapy: a case report

IntroductionWe report an unexpected presentation of metastatic renal cell carcinoma (RCC) to the ciliary body and an interesting response to proton beam radiotherapy.Case presentationWe encountered a case of angle-closure glaucoma as the initial presentation of ocular metastasis to the ciliary body in a 65-year-old Caucasian man who had undergone right radical nephrectomy for RCC 15 years earlier. He underwent YAG (yttrium aluminium garnet) laser peripheral iridotomy while further metastatic workup took place. His condition was eventually diagnosed as stage IV metastatic RCC of the clear cell type and involved multiple sites, including the ciliary body, brain, lungs, liver, and pancreas. The progression of RCC metastasis to the ciliary body was studied for 16 months. The ciliary body mass continued to grow despite systemic treatment with temsirolimus and interleukin-2 and intravitreal injections of bevacizumab. The tumor size peaked at 6.11 × 6.06 mm before the start of proton therapy, which reduced the tumor size to 5.07 × 4.39 mm.ConclusionsRCC can produce metastases involving unusual sites many years after resection of the primary tumor. Proton therapy was found to be effective in treating RCC metastasis to the ciliary body in settings in which other treatment modalities failed.

Adenoma of the nonpigmented ciliary epithelium presenting with recurrent iridocyclitis: unique expression of glial fibrillary acidic protein

Dear Editor, Adenoma of the nonpigmented ciliary epithelium (NPCE) is extremely rare and may mimic malignant melanoma. It has variable clinical and histopathological features. Here, we provide the first report of a case of an adenoma of the NPCE that presents with recurrent iridocyclitis, showing positive immunostaining for glial fibrillary acidic protein (GFAP) in the tumor. An 18-year-old boy was referred for a nonpigmented iris mass in his right eye. Before the detection of this mass, he had been treated for recurrent iridocyclitis for 2 years. Best-corrected visual acuity (BCVA) was 20/20, and intraocular pressure was 17 mmHg in the right eye. Slitlamp biomicroscopy revealed 2+ anterior chamber cells and a grayish-white mass with a well-demarcated margin and prominent vascularization at the 5 o’clock position (Fig. 1a). Gonioscopy showed that the mass, which arose from the ciliary body, partially covered the iris surface and the anterior chamber angle. Ultrasound biomicroscopy revealed medium internal reflectivity in the mass, measuring 3.0 × 2.3 mm (Fig. 1b). The differential diagnosis included uveal melanoma, ciliary epithelial tumor, ciliary body granuloma, and leiomyoma. Partial lamellar sclerouvectomy was performed without complications. One year after surgery, BCVA was 20/20, and there was no evidence of recurrence of the tumor or intraocular inflammation. Fig. 1 Slit-lamp photography of a vascularized ciliary body mass extending into the anterior chamber. There is mild conjunctival hyperemia with 2+ anterior chamber cells (a). Ultrasound biomicroscopy discloses the ciliary body mass (*) encroaching on the iris ... Microscopically, the tumor consisted of sheets, cords, and tubules occasionally forming glandular configurations (Fig. 1c). The tumor cells had abundant eosinophilic cytoplasm with bland-looking round nuclei and moderately prominent nucleoli. The cellular borders were scalloped due to cytoplasmic indentation and artifactural clefting. The tumor cell nests were surrounded by abundant hyalinized stroma. Neither necrosis nor mitotic figures were observed. Immunohistochemical analyses were positive for S-100 protein, cytokeratins (AE1/AE3), vimentin, and GFAP (Fig. 1d), but negative for HMB-45 and melan A. The histopathological and immunohistochemical findings confirmed the diagnosis of an adenoma of the NPCE. Adenoma of the NPCE may be congenital or acquired, and shows a wide spectrum of biological behavior [1, 2]. Despite its benign nature, adenoma of the NPCE may have local aggressiveness that results in cataract, glaucoma, vitreous hemorrhage, and intraocular inflammation; therefore, it is difficult to clinically differentiate from malignant tumors of the ciliary body [2]. Our case was diagnosed as an adenoma of the NPCE because of the histopathological features of the tumor and the immunohistochemical stains, which confirmed that the tumor originated from the NPCE [1, 3]. Positive immunostaining for GFAP in adenoma of the NPCE, to our knowledge, has never been reported. GFAP is thought to be specific for glial cells, especially astrocytes. In our case, however, astrocytic phenotype was not observed. Recent studies have demonstrated that the adult human ciliary epithelium contains progenitor cells with properties of neural stem cells which express GFAP and have the potential to proliferate and differentiate into both neurons and glia [4, 5]. Diffuse expression of GFAP may be largely attributed to the neural progenitor cells in the ciliary epithelium. Possible explanations for the intraocular inflammation observed in our case include direct infiltration of the iris by the tumor and altered immune responses of the anterior chamber. We report a case of an adenoma of the NPCE that presented with recurrent iridocyclitis. Although benign, adenoma of the NPCE may exhibit progressive growth and local invasion, causing intraocular inflammation. Positive expression of GFAP in this tumor has never been reported, but should be considered in the differential diagnosis. Partial lamellar sclerouvectomy seems appropriate to confirm the diagnosis and to preserve a good visual acuity.

Ocular perivascular epithelioid cell tumor: report of 2 cases with distinct clinical presentations

Perivascular epithelioid cell tumors comprise a rare and recently described family of neoplasms that characteristically coexpress melanocytic and myoid markers. We describe the clinicopathologic features of 2 ocular cases. Case 1 occurred in a 26-year-old woman with a recurrent left upper eyelid tumor, and case 2 was diagnosed in a 7-year-old boy with a left ciliary body mass. This is the first report of perivascular epithelioid cell tumor arising in the ciliary body or eyelid. Neither patient in our series had documented evidence of the tuberous sclerosis complex. Despite its rarity, perivascular epithelioid cell tumor should be considered in the differential diagnosis of ocular melanocytic lesions. Although most examples appear cytologically bland, experience is limited regarding their malignant potential; and therefore, complete surgical resection and close follow-up are recommended.

A Case of Ciliary Body Melanocytoma Presenting as a Painful Iris Mass

We report a case of ciliary body melanocytoma in a Korean patient, which presented as an intermittently painful pigmented iris mass and was successfully managed by iridocyclectomy. A 52-year-old healthy man presented with an irregularly-shaped and heavily-pigmented mass at the iris root of his right eye. Visual acuity of the right eye was 20/20 with normal intraocular pressure. Ultrasound biomicroscopy showed a 1.5x1.3-mm ciliary-body mass with extension into the iris root. Iridocyclectomy with scleral resection under a lamellar scleral flap was performed, and the histopathologic features of the resected tissue were consistent with melanocytoma of the ciliary body. The patient's visual acuity remained 20/20 with good postoperative cosmesis. During one year of follow-up, no signs of tumor recurrence were seen, and the patient reported resolution of the intermittent ocular pain in the involved eye.

MR Imaging of Adenomas of the Nonpigmented Ciliary Epithelium of the Eye

ANPCEs are rare benign tumors of the eye arising from the NPCE in adults, which may be clinically mistaken for melanoma. This study was undertaken to delineate clinical and MR imaging features of these tumors. Clinical presentation and MR imaging findings of 8 patients (6 women and 2 men; median age, 51 years) with pathologically confirmed ANPCEs were retrospectively reviewed. Location, size, shape, margin, signal intensity, and gadolinium-enhancement characteristics of all tumors were evaluated. Signal intensity and degree of enhancement were graded in comparison with the ipsilateral lacrimal gland. MR imaging revealed a circumscribed enhancing mass within the ciliary body of the eye in all 8 patients. The mass was ovoid in 6 patients and spheric in 2. Gadolinium enhancement was marked in 4 lesions and moderate in the other 4. Both T1 and T2 relaxation times were qualitatively identical to those in the lacrimal gland in 2 tumors. In the remaining 6 tumors, the T1 was identical to and the T2 longer than that in the lacrimal gland. ANPCE should be included in the differential diagnosis of a spheric or ovoid enhancing ciliary body mass with T1 similar to that in the lacrimal gland and T2 equal to or longer than that in the lacrimal gland.

Ciliary Body Tumour Occurring in a Nigerian - A case report

Objective: To report the rare case of a 33-year-old female Nigerian who presented to our retina clinic with a chronic total retinal detachment and visual acuity of no light perception in her left eye. Re-attachment surgery in the eye was not attempted as prognosis for return of vision was poor. She was later noted to harbor a progressively enlarging ciliary body mass in the inferotemporal quadrant. Method: An observational case report was performed, with documentation of findings as patient was seen in the clinic. Conclusion: This is the first report of a ciliary body mass lesion in a Nigerian. The ciliary body mass could be a ciliary melanoma (ciliary body lesion being rarer than a choroidal mass) but other benign differential diagnoses must be borne in mind. Enucleation and histological assessment of the specimen will give the definitive diagnosis. Difficulties with patient acceptance of enucleating a non seeing eye though harboring a potentially harmful disease still abound in our environment.

Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study

The purpose of this study was to describe the growth pattern of congenital malignant teratoid medulloepithelioma of the ciliary body by reporting clinical and imaging findings with pathological correlation. An 11-month-old little girl presented with a whitish-pink iris mass in the right eye resulting from a small ciliary body mass consistent with medulloepithelioma at both clinical and computed tomography (CT) findings. At CT, the lesion showed heterogeneous attenuation, without intraocular calcifications. Eleven months later, clinical and ultrabiomicroscopy showed a clear enlargement of the mass, which invaded the pupil. At magnetic resonance imaging (MRI), the lesion showed T1-weighted hyperintensity and T2-weighted slight hypointensity when compared to the vitreous and a notch in the anterolateral aspect of the ipsilateral lens. After intravenous gadolinium administration, the lesion showed intense homogeneous enhancement, and there was leakage of gadolinium in the anterior chamber, resulting from impairment of blood-aqueous barrier. Biopsy revealed a malignant teratoid medulloepithelioma. The eye was then enucleated, and histology confirmed the diagnosis. Systemic chemotherapy and radiotherapy were not performed, since there was no extraocular extension. The 57-month clinical and MRI follow-up did not show disease relapse. This uncommon case displays the natural history of congenital malignant teratoid medulloepithelioma of the ciliary body. While the tumour might have been successfully treated by local excision at diagnosis, the delay in surgical treatment led to tumour overgrowth with consequent need for enucleation. The most important prognostic feature is extraocular extension, which carries a risk of local recurrence, eventually resulting in intracranial extension and/or lymphatic spread.