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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 243-245

Early ipsilateral orbital metastasis of choroidal melanoma without evidence of hepatic metastasis: Case report


1 Department of Ophthalmology and Visual Sciences, Ocular Oncology Service, Universidade Federal de São Paulo, São Paulo, Brazil
2 Department of Ophthalmology, University of Montreal, Hopital Maisoneuve-Rosemont, Montreal, QC H1T 2M4, Canada

Date of Submission24-Mar-2021
Date of Acceptance02-Jul-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Ever Ernesto Caso Rodriguez
Ocular Oncology Service, Department of Ophthalmology and Visual Sciences, Federal University of Sao Paulo, Rua Botucatu, 822, Sao Paulo
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_657_21

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  Abstract 


Uveal melanoma is the most common primary intraocular cancer in adults. The liver is the most common site of metastasis. Ipsilateral orbital metastasis without radiological and clinical evidence of liver metastasis is extremely rare. We present a 77-year-old patient with choroidal melanoma in his right eye who was treated with enucleation and 9 months later presented ipsilateral orbital metastasis without radiological and clinical evidence of metastatic liver lesions. After the diagnosis of orbital metastasis, he underwent exenteration and radiotherapy and the patient developed lung, liver, and subcutaneous metastases in the following months. Early detection of uveal melanoma and especially when the tumor is small, can save lives since no treatment has proven to significantly alter mortality when there is metastatic disease.

Keywords: Choroidal melanoma, hepatic micrometastases, liver imaging, metastasis


How to cite this article:
Rodriguez EE, Isenberg J, Sutil A, Morales M, Sant'ana R, Belfort R. Early ipsilateral orbital metastasis of choroidal melanoma without evidence of hepatic metastasis: Case report. Indian J Ophthalmol Case Rep 2022;2:243-5

How to cite this URL:
Rodriguez EE, Isenberg J, Sutil A, Morales M, Sant'ana R, Belfort R. Early ipsilateral orbital metastasis of choroidal melanoma without evidence of hepatic metastasis: Case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 16];2:243-5. Available from: https://www.ijoreports.in/text.asp?2022/2/1/243/334968



Choroidal melanoma is the most common primary intraocular malignant tumor in adults, with incidence of approximately 4.3 per million per year. The choroid is the most frequently involved site (90%) in uveal melanoma cases followed by the ciliary body (7%) and the iris (2%).[1] The liver is the most common site of metastasis and is involved in 90% of individuals who develop metastatic disease.[2]

About 50% of these patients with liver metastasis showed evidence of metastasis in two or more extrahepatic sites.[3] The most common extrahepatic metastasis sites are the lungs (24%), bone (16%), skin (11%), brain (5%), and orbit recurrence (1%).[3] The median survival of uveal melanoma patients with liver involvement is reported to be 4–5 months with a 1-year survival of 10%–15%.[2],[3]

The most common treatment modalities for choroidal melanoma worldwide are enucleation or brachytherapy.[4] There is no access to plaque brachytherapy for the vast majority of Brazilians under the current public health care regime. That is why enucleation then becomes the treatment of choice. We present a patient treated for choroidal melanoma with enucleation who presented orbital metastasis 9 months after the initial treatment without radiologic and clinical evidence of metastatic liver lesions.


  Case Report Top


A 77-year-old Caucasian man with medical history of arterial hypertension with a 1-year history of decreased visual acuity was referred for evaluation of a pigmented choroidal mass in his right eye. On examination, the best-corrected visual acuity was 20/70 and 20/20 in the right (RE) and left eye (LE), respectively. Fundus evaluation in the RE revealed a juxtamacular pigmented choroidal mass with lipofuscin measuring 18 mm in largest basal diameter and 5.8 mm in thickness [Figure 1]a and [Figure 1]b. Liver ultrasonography (US) and liver function tests were reported as normal. The choroidal melanoma was clinically classified using the AJCC 8th edition as T4a N0 M0 classified as stage IIIA. The patient underwent an RE enucleation. The histopathology of the surgical specimen revealed choroidal melanoma with a predominance of epithelioid cells and was classified by the AJCC 8th edition as grade G3 [Figure 1]c.
Figure 1: (a) Juxtamacular pigmented choroidal melanoma with overlying subretinal fluid and orange pigment, (b) ultrasound of tumor with thickness of 5.8 mm and 18 mm in largest basal diameter. AJCC category T4a N0 M0 melanoma. (c) Histopathology of right eye (RE) showing choroidal melanoma of mixed pattern and predominance of epithelioid cells

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At postoperative 9 months (June 2014), the patient presented to ophthalmology complaining of a rapidly growing, purple colored lesion on the medial anterior aspect of the right lower eyelid [Figure 2]. Cranial angiography revealed intense internal vascularization at the level of the lesion [Figure 3]a and [Figure 3]b, while computer tomography (CT) of the abdomen was within normal limits [Figure 4]a.
Figure 2: Nine months after treatment, the patient developed a violated lesion in the lower orbital region (near the lacrimal punctum) (a) with rapid growth in a few weeks (b and c)

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Figure 3: Magnetic resonance imaging (MRI) demonstrated that the cystine lesion in the right orbit has focal enhancement compatible with metastasis (a). Cranial angiography shows the tumor blush in the right orbit (b)

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Figure 4: (a) Computed tomography scan of the liver in October/2014 without changes. (b) May /2015 CT scan of the liver with multiple disseminated liver lesions in the parenchyma

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Incisional biopsy of the lesion was performed with microscopy confirming the tissue to be from metastasis of choroidal melanoma. The cancer was restaged as T4a N0 M1 and classified as stage IV. The patient was consented for and underwent a right orbital exenteration followed by radiation therapy of 50 Gy/25 fractions/5 weeks.

At postoperative (exenteration) month 7, the patient presented with two dark, flat skin lesions in the left frontal region of the head and scalp ipsilateral to the affected orbit, whose biopsy confirmed metastatic melanoma and submandibular lymph nodes. Over the next two months, the patient developed pulmonary, hepatic metastases [Figure 4]b and died at postoperative month 9.


  Discussion Top


Metastatic choroidal melanoma probably affects the liver in all cases; in fact, in autopsy studies, the liver was affected by metastasis in 100%, unlike the 93% of the impairment detected clinically.[3] The Collaborative Ocular Melanoma Study (COMS) determined that liver function tests are a poor surveillance tool with a sensitivity and specificity of 15% and 92%, respectively.[5] Therefore, liver imaging methods have been studied for staging and systemic screening of metastatic disease in patients with uveal melanoma. These modalities include US, contrast-enhanced CT, contrast-enhanced magnetic resonance imaging (MRI), and positron emission tomography.[6]

US in experienced hands has high sensitivity and specificity.[6] However, it is highly operator dependent.[6] CT has many advantages: universally available, well-tolerated, and fast. Disadvantages include the use of ionizing radiation and it is lower sensitivity for liver lesions smaller than 10 mm.[7] MRI is the study of choice to evaluate liver metastases because of its high specificity and similar sensitivity in the detection of liver metastasis as compared to CT.[8] Post-mortem histopathology studies note the presence of micrometastasis (lesions between 50 and 2000 μm) in the liver in all patients with metastatic choroidal melanoma elsewhere,[9] but these lesions are too small to be detected by current radiology methods (CT and MRI).[6]

The AJCC Cancer Staging Manual provides uniform guidance for cancer staging, assessing anatomic dimensions, and histopathologic factors utilizing the available evidence.[10] Compared with uveal melanoma classified as AJCC stage I, the rate of metastasis/death was 3 times greater for stage II, 9 to 10 times greater for stage III, and further greater for stage IV. Early detection of posterior uveal melanoma when the tumor is small, can be lifesaving since no treatment has proven to significantly change the mortality when metastasis is present.[11]


  Conclusion Top


To our knowledge, this is the first reported case of choroidal melanoma with ipsilateral metastasis in orbit without radiologic and clinical evidence of metastatic liver lesions when orbital metastasis was diagnosed.

The initial classification of the patient was stage IIIa and after 9 months and having multiple metastases, the reclassification was stage IV. The AJCC cancer staging system for posterior uveal melanoma has been widely validated in recent years and ocular oncologists should consider using this classification system to predict the risk of metastasis and death.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shields CL, Kaliki S, Furuta M, Mashayekhi A, Shields JA. Clinical spectrum and prognosis of uveal melanoma based on age at presentation in 8,033 cases. Retina 2012;32:1363-72.  Back to cited text no. 1
    
2.
Singh AD, Borden EC. Metastatic uveal melanoma. Ophthalmol Clin North Am 2005;18:143-50, ix.  Back to cited text no. 2
    
3.
Collaborative Ocular Melanoma Study Group. Assessment of metastatic disease status at death in 435 patients with large choroidal melanoma in the Collaborative ocular melanoma study (COMS): COMS report no. 15. Arch Ophthalmol 2001;119:670-6.  Back to cited text no. 3
    
4.
Jager MJ, Shields CL, Cebulla CM, Abdel-Rahman MH, Grossniklaus HE, Stern M-H, et al. Uveal melanoma. Nat Rev Dis Primers 2020;6:24.  Back to cited text no. 4
    
5.
Singh AD, Kivela T. The collaborative ocular melanoma study. Ophthalmol Clin North Am 2005;18:129-42, ix.  Back to cited text no. 5
    
6.
Bellerive C, Ouellet E, Kamaya A, Singh AD. Liver imaging techniques: Recognition of uveal melanoma metastases. Ocul Oncol Pathol 2018;4:254-60.  Back to cited text no. 6
    
7.
Balasubramanya R, Selvarajan SK, Cox M, Joshi G, Deshmukh S, Mitchell DG, et al. Imaging of ocular melanoma metastasis. Br J Radiol 2016;89:20160092.  Back to cited text no. 7
    
8.
Semelka RC, Martin DR, Balci C, Lance T. Focal liver lesions: Comparison of dual-phase CT and multisequence multiplanar MR imaging including dynamic gadolinium enhancement. J Magn Reson Imaging 2001;13:397-401.  Back to cited text no. 8
    
9.
Grossniklaus HE. Progression of ocular melanoma metastasis to the liver: The 2012 Zimmerman lecture. JAMA Ophthalmol 2013;131:462-9.  Back to cited text no. 9
    
10.
Kivela T, Simpson ER, Grossniklaus HE, Jager MJ, Singh AD, Caminal JM, et al. Uveal Melanoma. In: Amin MB, editor. AJCC Cancer Staging Manual. American Joint Committee On Cancer Executive Office 633 North Saint Clair Street Chicago, IL 60611-3211: Springer International Publishing; 2018. p. 805-17.   Back to cited text no. 10
    
11.
Shields CL, Kaliki S, Furuta M, Fulco E, Alarcon C, Shields JA. American joint committee on cancer classification of uveal melanoma (Anatomic stage) predicts prognosis in 7,731 patients: The 2013 Zimmerman lecture. Ophthalmology 2015;122:1180-6.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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