Intraocular coccidioidomycosis simulating a neoplasm
- 1The Tumori Foundation, San Francisco, California, USA
- 2UCSF, Department of Ophthalmology, San Francisco, California, USA
- 3Stanford, Department of Ophthalmology, Palo Alto, California, USA
- 4EMC Fresno, Fresno, California, USA
- Correspondence to Devron H Char, The Tumori Foundation, 45 Castro Street, Suite 309, San Francisco, CA 94114, USA;
Contributors All authors fulfil the criteria of authorship, and no one else fulfils the criteria who has not been included as an author on this manuscript. The principal investigator had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
- Accepted 15 April 2011
- Published Online First 5 May 2011
Coccidioidomycosis can simulate an intraocular neoplasm. We reviewed a case report of a 10-year-old girl who was referred with an intraocular tumour. This tumour consisted of a coccidioidomycosis infection in the eye. The eye was blind and painful so it was removed.
Coccidioidomycosis is an uncommon, systemic infection that rarely involves ophthalmic structures.1 The first description of ophthalmic involvement was by Rixford and Gilchrist in 1896.1 Almost all cases present as a variant of uveitis; less than 40 cases have been reported and none have simulated a large intraocular tumour.1
A 10-year-old girl from the Central Valley of California had a 1-month history of a painful left eye with sudden vision decrease. She presented at that time with tearing, pain, ptosis and left orbital swelling. In retrospect, but not told to us, it had been recommended by her paediatrician that she be treated with a systemic agent for a presumptive diagnosis of systemic coccidioidomycosis (Valley fever). She had not taken medicines. She had both IgG and IgM antibodies to coccidioidomycosis; chest x-ray demonstrated two granulomas consistent with coccidioidomycosis.
On our examination the patient had a visual acuity of 20/20 in the right eye and no light perception in the left eye. Intraocular pressures measured 16 mm Hg in the right eye and 32 mm Hg in the left eye. She had a 45 prism dioptre left exotropia. The orbital examination and the right anterior segment and fundus were unremarkable. In the left eye there was trace corneal microcystic oedema, with 1+ flare and cells with left iris neovascularisation. There was a clear left lens with a total bullous retrolental retinal detachment. Under the retina an orange-coloured mass straddled the ocular equator temporally and was 10.5×10.5×7.5 mm on clinical examination. No intra-tumour vascularity was noted. On B-scan a mass was noted (figure 1A), which on A-scan was of low reflectivity with a climbing posterior spike (figure 1B). A CT scan documented the mass lesion (figure 1C).
The differential diagnosis included an atypical melanoma, other benign intraocular neoplasms or coccidioidomycosis. We felt that a diagnosis of coccidioidomycosis was unlikely since we were not aware of any cases in which this infectious process produced a large, intraocular mass. Since the eye was blind and uncomfortable, it was removed.
The enucleated eye showed evidence of mild chronic iritis with peripheral anterior synechiae and rubeosis, a total serous retinal detachment and a diffuse non-granulomatous choroiditis. The most impressive area of inflammation was an elevated, temporal mass that involved the paramacular area. The inflammation in the mass was granulomatous with epithelioid cell nodules, giant cells, and areas of acute inflammation and necrosis (figure 2A). The periodic acid–Schiff (PAS) stain demonstrated a few spherules characteristic of Coccidoides immitis (figure 2B); the Grocott's methenamine silver (GMS) stain confirmed this (figure 2C,D).
Ocular coccidioidomycosis is rare, but the incidence of systemic disease has been increasing.1 3 Coccidioidomycosis is most common in the Central Valley of California. It is estimated that less than 0.5% of cases develop extra-pulmonary involvement.3 While any ophthalmic structure can be involved, most cases have either a chorioretinitis or a granulomatous iridocyclitis in association with disseminated disease.1 Rare cases of endophthalmitis have been reported4; a few reports of isolated ocular involvement have been documented.5 The diagnosis of an isolated ocular lesion can be difficult since serological and direct fungal studies may be negative.3 6
Our case was unique in presentation as a mass lesion. We had suspicion of a coccidioidomycosis aetiology given the patient's history, but since the eye was blind and painful it was removed. Her serological studies, chest x-ray and special stains on the ocular tissue confirmed the diagnosis. While we were confident it was not a retinoblastoma based on age, clinical pattern and ultrasonography, we could not rule out either an atypical uveal melanoma or a benign tumour with secondary detachment prior to histological evaluation.
Our patient received ketaconazole for the lung lesions. While she remains otherwise well, it is important to note that more than 50% of patients with ocular involvement have either lost the eye or died because of the systemic disease.1 Newer fungal agents are being investigated, but their efficacy for ocular involvement is uncertain.3
Funding Special thanks to The Tumori Foundation for providing financial and material support for the research project. However, The Tumori Foundation had no direct involvement in the study design, in the collection, analysis and interpretation of data, in the writing of the report, or in the decision to submit the paper for publication.
Competing interests None declared.
Patient consent Obtained.
Ethics approval This study was conducted with the approval of the California Pacific Medical Center Research Institute (CPMCRI) Institutional Review Board.
Provenance and peer review Not commissioned; externally peer reviewed.