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Year : 2018  |  Volume : 25  |  Issue : 2  |  Page : 115-117  

Iris melanoma in a child simulating juvenile xanthogranuloma

1 Department of Ocular Oncology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA
2 Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA

Date of Web Publication18-Jul-2018

Correspondence Address:
Dr. Carol L Shields
Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/meajo.MEAJO_7_18

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An 8-year-old girl was referred for an amelanotic iris tumor believed to have enlarged slightly over 3 months, suspicious for juvenile xanthogranuloma (JXG). The affected right eye had a lightly pigmented, vascular iris mass measuring 6 mm in basal dimension and 2 mm in thickness. There were no feeder vessels, seeding, inflammatory cells, or cutaneous abnormalities. Diagnostic fine needle aspiration biopsy (FNAB) was performed, revealing spindle B melanoma cells that were immunoreactive for melanocytic markers HMB45 and Melan-A. Complete tumor resection by basal sector iridectomy was performed. Histopathology confirmed spindle B melanoma. At 14-years follow up, there has been no recurrence or metastasis and visual acuity remains 20/25. Iris melanoma can develop in children and clinically resemble nodular JXG.

Keywords: Child, eye, iris, juvenile xanthogranuloma, melanoma, pediatric, tumor

How to cite this article:
Shields JA, Shields CL, Lally SE, Milman T, Eagle RC. Iris melanoma in a child simulating juvenile xanthogranuloma. Middle East Afr J Ophthalmol 2018;25:115-7

How to cite this URL:
Shields JA, Shields CL, Lally SE, Milman T, Eagle RC. Iris melanoma in a child simulating juvenile xanthogranuloma. Middle East Afr J Ophthalmol [serial online] 2018 [cited 2021 Oct 27];25:115-7. Available from: http://www.meajo.org/text.asp?2018/25/2/115/237038

   Introduction Top

Juvenile xanthogranuloma (JXG) is a cutaneous papular eruption that generally occurs in childhood.[1],[2],[3] This condition can rarely occur in the iris as a nodular (76%) or flat diffuse (24%) vascularized mass of Caucasian (80%) children that can progress fairly rapidly over a few weeks.[3] Iris JXG carries a risk for spontaneous hyphema (19%), secondary glaucoma, and even blindness.[3] Similarly, iris melanoma can occur in children as a yellow-tan (13%) mass of mean 5.6 mm base and 2.5 mm thickness, with slower progression over months to years.[4],[5] Herein, we report a child with a yellow-tan vascular iris mass, found to show slight growth over 12 weeks and was suspicious for possible JXG or melanoma. However, fine-needle aspiration biopsy (FNAB) confirmed malignant melanoma that was removed by sector iridectomy.

   Case Report Top

A healthy 8-year-old female child was noted have a growth on her right iris. The asymptomatic lesion was followed for 3 months and demonstrated slight enlargement, leading to suspicion of JXG. She was referred for diagnosis and management.

On examination, visual acuity was 20/20 in each eye and intraocular pressures were normal. The only abnormal ocular finding was a minimally pigmented, vascular iris mass, measuring 6.0 mm in diameter and 2 mm in thickness, located in the nasal aspect of the iris [Figure 1]. Gonioscopy and ultrasound biomicroscopy revealed that the lesion was confined to the iris with no extension or seeding into the angle or ciliary body. Fluorescein angiography revealed intense vascularity confined to the mass and not involving the surrounding normal iris [Figure 1]. Our differential diagnosis included amelanotic iris melanoma versus JXG.
Figure 1: Clinical and cytopathological features of iris melanoma in a child. (a) An 8-year-old child with a vascular amelanotic iris mass. (b) Ultrasound biomicroscopy showing iris thickening (arrow), but no ciliary body involvement. Fluorescein angiogram showing (c) early features of normal iris vascularity and nasal tumor enhancement with (d) late intense hyperfluorescence. (e) Cytopathology of fine-needle aspiration biopsy specimen demonstrating a cluster of atypical spindle cells with ovoid nuclei, slightly coarse chromatin, and prominent central nucleoli (Papanicolaou stain, ×250). (f) Tumor cells in fine-needle aspiration biopsy are immunoreactive for HMB45 (IHC for HMB45; ×250)

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FNAB was performed for diagnostic purposes. Using an operating microscope, a 27-gauge needle was passed through the clear cornea 1 mm from the limbus and directed into the tumor with aspiration using standard technique.[8],[9] Cytopathology revealed atypical cells with spindle morphology, ovoid nuclei, and prominent central nucleoli, which were immunoreactive for melanocytic markers melan-A and HMB45, consistent with spindle B malignant melanoma cells [Figure 1].

Based on the cytopathological diagnosis of melanoma, basal sector iridectomy was performed. Histopathology revealed an amelanotic neoplasm, composed predominantly of spindle B melanoma cells and few cells with intermediate morphology between spindle B and epithelioid melanoma. There was one mitotic figure per 40 high-power fields [Figures 2]. There was minimal invasion into the trabecular meshwork and base of the pars plicata. The tumor appeared to be completely removed. The tumor was classified as pT2a based on the American Joint Committee on Cancer classification (8th edition).[10],[11]
Figure 2: Surgical and histopathological results. (a) Gross evaluation of the iridectomy specimen demonstrated a solid vascular amelanotic iris mass with (b) histopathology features of neoplasm composed of a syncytial arrangement of cells with cigar-shaped nuclei, finely dispersed chromatin, linear nuclear folds, and inconspicuous nucleoli (spindle A melanoma cells, white arrow) intermixed with cells with ovoid nuclei and prominent nucleoli (spindle B melanoma cells, black arrow) (H and E; ×100). Cells with morphology intermediate between spindle B and epithelioid melanoma cells are present (white arrow). (c) A mitotic figure is noted (black arrow) (H and E; ×100). (d) Appearance of iris after 14 years showing sector iridectomy and no evidence of tumor

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One week after surgery, the visual acuity was 20/40, and 6 months later, it remained 20/25. Local and systemic evaluation revealed no evidence of tumor recurrence or metastatic melanoma after 14 years and visual acuity was stable.

   Discussion Top

Although it has several clinical variations, iris melanoma usually appears as a pigmented mass that is diagnosed in adulthood and shows slow progression.[11] However, melanoma can sometimes appear clinically amelanotic and is less commonly diagnosed in children. The case reported here is unusual, in that iris melanoma appeared as a fairly rapidly enlarging amelanotic mass in an 8-year-old child. A rapidly growing amelanotic iris lesion in a child is more consistent with an inflammatory process such as JXG. We have seen several cases of JXG that had an almost identical appearance and clinical course. In our experience, however, fluorescein angiography of iris JXG shows diffuse, extensive vascular leakage, whereas melanoma is more localized.[6] In our patient, the hyperfluorescence was confined to melanoma.

The diagnosis in this atypical case was made with cytopathological study of a FNAB specimen, a reliable method for diagnosing atypical intraocular lesions.[7],[8] The technique and results of iris FNAB have been reported.[9],[10] This method should be reserved for cases where the diagnosis is not clinically established, and the results can be applied to determine the best treatment. If the lesion in this case had proved to be JXG, treatment options would have included oral, topical, or periocular corticosteroids. Since it proved to be a melanoma confined to the iris in a young child, the best treatment option was basal sector iridectomy, rather than enucleation or irradiation period. The visual result has thus far been excellent, and based on the histopathology, the chances of metastasis are very low. However, this patient will have close ocular and systemic follow-up. In summary, amelanotic iris melanoma can occur in children and must be differentiated from JXG. FNAB may be necessary in such cases to establish the diagnosis and help select a choice of treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The support was provided by a donation from the Eye Tumor Research Foundation, Philadelphia, PA (JAS, CLS), the Lucille Weidman Fund for Pediatric Cancer, Philadelphia, PA (JAS, CLS), the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Philadelphia, PA (RCE).

Conflicts of interest

There are no conflicts of interest.

   References Top

Cohen BA, Hood A. Xanthogranuloma: Report on clinical and histologic findings in 64 patients. Pediatr Dermatol 1989;6:262-6.  Back to cited text no. 1
Zimmerman LE. Ocular lesions of juvenile xanthogranuloma (nevoxanthoendothelioma). Trans Am Acad Ophthalmol Otolaryngol 1965;69:412-42.  Back to cited text no. 2
Samara WA, Khoo CT, Say EA, Saktanasate J, Eagle RC Jr., Shields JA, et al. Juvenile xanthogranuloma involving the eye and ocular adnexa: Tumor control, visual outcomes, and globe salvage in 30 patients. Ophthalmology 2015;122:2130-8.  Back to cited text no. 3
Shields CL, Kaliki S, Shah SU, Luo W, Furuta M, Shields JA, et al. Iris melanoma: Features and prognosis in 317 children and adults. J AAPOS 2012;16:10-6.  Back to cited text no. 4
Singh AD, Shields CL, Shields JA, Sato T. Uveal melanoma in young patients. Arch Ophthalmol 2000;118:918-23.  Back to cited text no. 5
Danzig CJ, Shields CL, Mashayekhi A, Ehya H, Manquez ME, Shields JA, et al. Fluorescein angiography of iris juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus 2008;45:110-2.  Back to cited text no. 6
Shields JA, Shields CL, Ehya H, Eagle RC Jr., De Potter P. Fine-needle aspiration biopsy of suspected intraocular tumors. The 1992 Urwick lecture. Ophthalmology 1993;100:1677-84.  Back to cited text no. 7
Shields CL, Manquez ME, Ehya H, Mashayekhi A, Danzig CJ, Shields JA, et al. Fine-needle aspiration biopsy of iris tumors in 100 consecutive cases: Technique and complications. Ophthalmology 2006;113:2080-6.  Back to cited text no. 8
Shields JA, Shields CL, editors. Removal of iris tumors by partial iridectomy. In: Intraocular Tumors: An Atlas and Textbook. Philadelphia: Wolters Kluwer; 2016. p. 65.  Back to cited text no. 9
Bekerman V, DiNicola M, Shields JA, Shields CL. Updated classification for primary iris melanoma key characteristics of the American Joint Committee on Cancer's revised classification system (8th edition). Retina Today 2017;40-3.  Back to cited text no. 10
Shields CL, Di Nicola M, Bekerman VP, Kaliki S, Alarcon C, Fulco E, et al. Iris melanoma outcomes based on the American joint committee on cancer classification (Eighth edition) in 432 patients. Ophthalmology 2018;125:913-23.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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