ADRENAL CORTICAL CARCINOMA PRESENTING AS CENTRAL SEROUS CHORIORETINOPATHY
A. Reza Gohari, MD,* Sara P. Modjtahedi, MD,* Caleb R. Telander, BS,* Max X. Kong, MD*
Purpose: The authors describe a case of bilateral chronic central serous chorioretinop- athy secondary to adrenal cortical carcinoma.
Methods: A case report of a 70-year-old Hispanic man presenting with bilateral multifocal central serous chorioretinopathy.
Results: Clinical findings of bilateral chronic central serous chorioretinopathy along with 160 um of subretinal fluid and choroidal thickness greater than 400 um without enhanced depth optical coherence tomography were noted in a patient presenting with distortion in vision in both eyes and a weight gain of 15 pounds, weakness, and fatigue starting 8 months before. Further endocrine testing showed an elevated 24-hour urinary free cortisol level of 137 µg with no change in serum cortisol levels following the low-dose dexamethasone suppression test and undetectable serum adrenocorticotropic hormone levels consistent with adrenocorticotropic hormone-independent Cushing syndrome. Imaging of the abdomen revealed heterogeneously enhancing masses, and the biopsy of the adrenal tumor led to the diagnosis of adrenal cortical carcinoma. He died 3 weeks after starting hospice care and only completed one round of chemotherapy.
Conclusion: Adrenal tumors, including adrenal cortical carcinoma, may present as bilateral chronic central serous chorioretinopathy.
RETINAL CASES & BRIEF REPORTS 19:761-765, 2025
From the *Northern California Kaiser Permanente, Roseville, CA.
C entral serous chorioretinopathy (CSCR) is an ocular disorder characterized by accumulation of subretinal fluid in the posterior pole with a circumscribed area of serous retinal detachment.1 Central scotoma, meta- morphopsia, micropsia, or macropsia are the most common symptoms. The most common presentation of CSCR is in younger patients between 30 and 50 years of age. It is associated with an acute localized detachment of the retina with mild to moderate vision loss and one or a few focal sites of ink blot or smokestack pattern of leakage seen during fluorescein angiography. In most cases of classic CSCR, the symptoms resolve within a few months, but severe, chronic, and atypical variants
of this condition have been described with multifocal pinpoint areas of leakage on fluorescein angiography with a mottled hyperfluorescence. Widespread alteration of the retinal pigment epithelium with a tear drop pattern related to the chronic presence of shallow subretinal fluid can be present in diffuse retinal pigment epitheliopathy or chronic CSCR. Bilateral CSCR and bullous retinal detachments that are often located inferiorly with shifting fluid can also be associated with organ transplantation, systemic corticosteroid use, and in patients of Asian descent. Complications of chronic CSCR include cystoid macular edema, subretinal lipid deposition, atrophy of the choriocapillaris, and choroidal neovascular membranes.2
Subretinal fluid can be detected in a variety of conditions such as endogenous hypercortisolism (Cushing disease and adrenal tumors), choroidal metastatic disease, hypoproteinemia (dietary insuffi- ciency, liver or kidney disease, or protein-losing enteropathy), hypertension, leukemia, lymphoma, and autoimmune disease such as multiple sclerosis
None of the authors has any financial/conflicting interests to disclose.
Reprint requests: Ali Reza Gohari, MD, Northern California Kaiser Permanente, 1011 Riverside Avenue, Roseville, CA 95678; e-mail: Ali.reza.gohari@kp.org.
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and systemic lupus erythematosus.3 The association between CSCR and hypercortisolism is well estab- lished, but to our knowledge, only one previous case of CSCR secondary to adrenal cortical carcinoma has been described in the literature.4
Case Report
A 70-year-old Hispanic man presented to the retina clinic for possible neovascular age-related macular degeneration. His main subjective complaints were a decrease and distortion in vision in both eyes, starting about 8 months before the presentation. The review of systems was significant for weight gain of 15 pounds, weakness, and fatigue over the course of this time. Medical history
was significant for hypertension. His best-corrected visual acuity was 20/60 in the right eye and 20/30 in the left eye. Slit-lamp examination showed moderate nuclear sclerosis in both eyes. There was no evidence vitreous inflammation. A dilated fundus exami- nation demonstrated retinal pigment epithelium mottling in the posterior pole accompanied by shallow serous retinal detachments. Spectral-domain optical coherence tomography (OCT) showed pockets of shallow subretinal fluid bilaterally measuring 160 pm in thickness with a pigment epithelial detachment in the right eye (Figure 1) and bilateral choroidal thickening greater than 400 pm without enhanced depth imaging. Fundus photographs documented retinal pigmentary abnormalities in the macula with blunted foveal reflex and subretinal exudates without any clear evidence of metastatic yellow or cream-colored lesions (Figure 2). Autofluor- escence showed pinpoint areas of hypo and hyperautofluorescence
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corresponding to the areas of retinal pigment alteration in the posterior pole (Figure 3). Fluorescein angiography depicted multiple pinpoint areas of hyperfluorescence in the macula and peripapillary region (Figure 4). Indocyanine green testing demon- strated a speckled and mottled pattern, zones of hypoperfusion, choroidal vascular hyperpermeability, and vascular dilation in both eyes (Figure 5). Based on the clinical findings and the review of systems, an endocrinology work up was pursued that revealed an elevated 24-hour urinary free cortisol level of 137 µg (normal range 20-50 µg per 24 hours). There was no change in serum cortisol levels after the low-dose dexamethasone suppression test, and the serum adrenocorticotropic hormone levels were unremarkable. He was diagnosed with adrenocorticotropic hormone-independent Cushing syndrome. A computed tomography scan of the abdomen showed large heterogeneously enhancing masses in the right subhepatic space, the left upper pericolic gutter, and the adrenal fossa — suspicious for metastatic adrenal carcinoma (Figure 6). A needle biopsy of the abdominal mass detected high-grade oncocytic adrenal cortical carcinoma (Figure 7). The patient elected to have hospice care after one round of chemotherapy, and he canceled his scheduled follow-up visit with the retina department. He died 3 weeks after starting hospice care.
Discussion
The association between CSCR and hypercortiso- lism is well known, and CSCR has been reported in 5% of patients with endogenous Cushing syndrome.3 However, only four cases of adrenocorticotropic hormone-independent Cushing syndrome secondary to adrenal adenomas have been reported in the English literature.3,5-9 Central serous chorioretinopathy as the presenting symptoms of adrenal cortical carcinoma is rare, and to our knowledge, there is only one previous case reported. Thoelen et al4 described a case of carcinoma of the adrenal cortex, which presented as atypical CSCR in both eyes in a 54-year-old woman with history of hypertension along with increasing abdominal girth and a reduced general state of health.
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Fibrinous subretinal exudates were detected in both eyes on examination, and late slow leakage was noted on fluorescein angiogram in the right eye from four foci of retinal pigment epithelium detachment consis- tent with multifocal CSCR. Hepatic metastasis was detected on a subsequent computed tomography scan, and the patient died of sepsis 3 months later.
Some similarities were noted between our case report and the previous one described by Thoelen et al. Both cases presented as a bilateral chronic multifocal CSCR along with the presence of subretinal exudates on examination and multifocal pattern of leakage on fluorescein angiography. Hypertension and weight gain were noted in both of these presentations,
and on endocrine testing, plasma cortisol could not be suppressed by dexamethasone while the adrenocorti- cotropic hormone level was depressed. In both cases, endocrine testing led to the computed tomography scan, which detected an adrenal carcinoma with metastasis to the liver, a common site of metastasis in adrenal cortical carcinoma. The gender and age of our patient were different from the previous case of CSCR associated with carcinoma of the adrenal cortex, which was reported in the literature. Our patient was a 70-year-old man and previous case presented in a 54-year-old woman. Our case report also includes additional testing in the form of OCT, fundus autofluorescence, and indocyanine green, which were not available with the previous case report to allow for further comparison.
Bilateral choroidal thickening and subretinal fluid potentially could be caused by metastatic lesions to the choroid. Optical coherence tomography testing in this case showed distortion of the choroidal vasculature. However, color photographs did not show any evi- dence of yellow or cream-colored subretinal lesions, and there was no evidence of significant blockage from a choroidal mass on indocyanine green, as expected in cases of choroidal metastasis.9
The review of systems offered important clues that led to endocrine testing, abdominal imaging, and subsequent biopsy, which confirmed the diagnosis. Typical symptoms of Cushing syndrome include weight gain, facial rounding, plethora, muscle weakness, fatigue, and purple skin striae. Hyperten- sion, diabetes, amenorrhea, loss of libido, easy bruising, and spontaneous fractures are additional findings that may be encountered from prolonged exposure to high cortisol levels.3 Cushing syndrome should be considered in the differential diagnosis of
chronic, bilateral, and multifocal CSCR. Pathogen- esis of CSCR may be multifactorial, and it includes scleral thickness, chronic venous congestion, thick- ened choroid (pachychoroid), compression of the underlying choriocapillaris, and retinal pigment epithelium dysfunction. It is unclear whether the development of CSCR in our patient was promoted by elevated glucocorticoid levels or through another unknown mechanism secondary to metastatic adre- nal cortical carcinoma. One possible theory is alteration in the choroidal vasculature, leading to choroidal hyperpermeability or impaired retinal pigment epithelium function secondary to adrenal cortical carcinoma itself. Because the previous case report of bilateral CSCR secondary to adrenal cortical carcinoma only included fluorescein angi- ography and color photographs, this is the first case report of this condition presenting as bilateral and
chronic CSCR with additional imaging including OCT, indocyanine green, and autofluorescence.
Key words: adrenal cortical carcinoma, adrenal tumor, bilateral central serous chorioretinopathy, chronic central serous retinopathy, multifocal central serous chorioretinopathy.
Acknowledgments
The authors thank Ms. Marianna Sousa for editing the manuscript and Mr. Cheu Vang for formatting and labelling the images.
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