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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 170-172

Enophthalmos as a sole clinical manifestation in advanced breast carcinoma


1 Department of Ophthalmology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Medical Oncology, Sum Hospital, Bhubaneswar, Odisha, India
3 Chief Consultant Pathologist, Genx Diagnostics, Bhubaneswar, Odisha, India

Date of Submission04-May-2022
Date of Acceptance26-Aug-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Bijnya B Panda
B-3, 202, Shreekhetra Residency, Aiginia, Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1093_22

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  Abstract 


Orbital metastases from breast carcinoma presenting as enophthalmos are a rare occurrence and must be carefully investigated to stage the disease and initiate management, which can be lifesaving in such patients. We report our experience in diagnosing and managing a case of enophthalmos that, on histopathology, revealed orbital metastases from lobular breast carcinoma.

Keywords: Breast carcinoma, cancer, enophthalmos, eye, orbital metastases


How to cite this article:
Panda BB, Mishra P, Panda S, Senapati S. Enophthalmos as a sole clinical manifestation in advanced breast carcinoma. Indian J Ophthalmol Case Rep 2023;3:170-2

How to cite this URL:
Panda BB, Mishra P, Panda S, Senapati S. Enophthalmos as a sole clinical manifestation in advanced breast carcinoma. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:170-2. Available from: https://www.ijoreports.in/text.asp?2023/3/1/170/368123



Breast cancer is the most common cancer among Indian females with an age-adjusted rate as high as 25.8 per 100,000 women and a mortality of 12.7 per 100,000 women.[1] The most common sites for metastases are bones, liver, lungs, skin, and brain. However, in 12%–31% of cases, the initial presentation may be the metastatic deposits.[2] Orbital metastases may clinically manifest as enophthalmos in metastatic breast carcinomas.[3],[4] It may present clinically as ptosis mimicking involutional ptosis,[5] progressive ophthalmoplegia,[6] infiltration to optic nerve and meninges as leptomeningeal carcinomatosis,[7] and orbital metastatic deposits.[8]


  Case Report Top


A 57-year-old postmenopausal female presented with complaints of insidious onset of drooping of the right upper eyelid and diminution of vision for the past six months. She was diabetic and hypertensive. There was no family history of breast and uterine cancer. Her best-corrected visual acuity was 20/200 in the right eye (OD) and 20/80 in the left eye (OS). The intraocular pressures were 16 mmHg in both eyes. The margin reflex distance 1 (MRD 1) was −1 mm OD and +4 mm OS. Levator function was 1 mm OD and 10 mm OS. The right eye showed hypotropia of 1 mm with restriction of extraocular movements in all directions, suggestive of a frozen globe [Figure 1]a. The Hertel exophthalmometer readings were 16 mm in the right eye and 20 mm in the left eye (base at 98), suggestive of enophthalmos. An ill-defined and firm mass could be felt in the inferolateral part of the lower eyelid. The left eye examination was grossly normal except for an immature cataract. The presence of poor levator function with gross restriction in ocular movements were suggestive of restrictive pathology rather than an involutional ptosis. Contrast enhanced CT scan showed enhancing lesion in extraconal and intraconal spaces extending to orbital apex [Figure 1]b and [Figure 1]c. A right anterior orbitotomy (transcutaneous approach) incisional biopsy of the mass from the lower eyelid was performed. Histopathological examination of the specimen suggested metastatic deposits of epithelial origin with tumor cells showing strong cytoplasmic membranous positivity for pan-cytokeratin (pan-CK) [Figure 1]d and negative for CD-45 along with a few signet ring–type cells [Figure 1]e. She was referred to an oncologist for further systemic evaluation. Fluorodeoxyglucose (FDG)-positron emission tomography (PET) computed tomography (CT) scans were performed to know the metabolically active malignancy which showed mildly FDG avid soft tissue thickening in the intraconal and extraconal region of the right orbit (0.6 × 0.5 cm) and bilateral breast lesions each measuring (3 × 1 cm). Mild FDG avid sclerotic lesions were also found in all axial and appendicular skeletal systems. Further biopsy from the breast lesions was performed, which was suggestive of invasive lobular carcinoma positive for estrogen receptor (ER) and progesterone receptor (PR) and negative for human epidermal growth factor receptor 2 (HER2). Compounding the above positive findings, she was diagnosed with metastatic bilateral invasive lobular breast carcinoma (breast imaging-reporting and data system category 5 [BIRADS 5]). The clinical diagnosis was established as cT2 cN1 pM1 stage Ⅳ disease and management was planned accordingly.
Figure 1: (a) Clinical photo showing right eye moderate ptosis and enophthalmos with a frozen globe. (b) Axial CT scan orbits showing tumor infiltration extending to the orbital apex in the right eye and lateral aspect of the globe in the left eye. (c) Coronal view showing infiltration of periorbital soft tissue in both eyes. (d) IHC showing tumor cells with strong cytoplasmic membranous positivity for pan-CK. (e) Histopathology examination (10×, H and E stain) showing fibro-collagenous tissue infiltrated by tumor cells arranged in small nests and cords showing few signet ring cells (pink arrow)

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Considering the advanced stage of the disease, she was started on palliative chemotherapy with systemic targeted drug therapy (abemaciclib 200 mg twice daily), hormonal therapy (anastrazole 1 mg once daily), and external beam radiation therapy (EBRT) (60 grays in 30 fractions) to the right orbit. After one year of treatment, the periorbital lesions were nonpalpable; however, the enophthalmos persisted.


  Discussion Top


Clinical presentation as metastatic deposits from breast carcinoma are not very common and has been reported mostly in the anterior orbit than the posterior orbit.[8] A poor levator function associated with enophthalmos and a frozen globe in our patient was suspicious of some malignant infiltration into the superior orbital tissues rather than a simple involutional ptosis. Luckily, our patient had an ill-defined mass in the inferolateral orbital rim which was accessible for biopsy and had been missed by the referring ophthalmologist. Tissue biopsy becomes essential when no primary site is discoverable at first presentation and the lesion is at an accessible location. Histologically, it has been known that the majority of orbital breast metastases were found to be lobular carcinomas similar to that in our patient.[9] The other subtype of breast carcinoma which can manifest as orbital metastases is the infiltrating ductal carcinoma which is more destructive to surrounding structures as compared to the lobular variant. In two different case series of various orbital metastases, 8 out of 70 patients and 3 out of 9 patients manifested as enophthalmos, which is mostly attributed to the scirrhous infiltration into the extraocular muscles and orbital fat causing retraction of the globe.[8],[9]

Treatment of metastatic breast carcinoma is mostly palliative, owing to their poor prognosis. Treatment options for orbital metastases are palliative external beam radiation therapy, hormonal therapy, and systemic chemotherapy with or without targeted therapy. Breast carcinoma metastases into the choroid have also been described by many authors for which intravitreal bevacizumab has been a recommended line of management.[10] Surgical mastectomy is not recommended at present as it may further increase morbidity rather than cure the disease. Abemaciclib is a small-molecule inhibitor of cyclin-dependent kinases (CDK) 4 and 6 specifically indicated for HR+/HER2−, locally advanced or metastatic breast cancer in combination with an aromatase inhibitor or fulvestrant.[11] Our patient has received abemaciclib 200 mg twice daily for the last one year and has tolerated it well with few adverse effects like diarrhea, nausea, fatigue, and anemia. EBRT has been recommended for the orbital and ocular adnexal metastases with common adverse effects of cataracts, radiation retinopathy, etc., The median survival in such patients varies from 22 to 31 months, though survival up to 116 months has been reported.[12] Our patient has no palpable lesions at the end of one year of therapy and needs a longer follow-up to know the exact course of the disease.


  Conclusion Top


Interesting findings of enophthalmos and frozen globe in one eye in elderly age with absence of a history of trauma should arouse suspicion of metastases. Early biopsy and histopathological confirmation clinched the diagnosis and ultimately saved a life. Multidisciplinary management of both orbital metastases and primary cancer improves overall patient survival.

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.
Malvia S, Bagadi SA, Dubey US, Saxena S. Epidemiology of breast cancer in Indian women. Asia Pac J Clin Oncol 2017;13:289-95.  Back to cited text no. 1
    
2.
Muhd H, Zuhaimy H, Ismail MF, Arshad F, Azmi S, Sahak NH. Orbital metastasis as the initial presentation of breast cancer. Malays Fam Physician 2020;15:74-8.  Back to cited text no. 2
    
3.
El-Khazen Dupuis J, Marchand M, Javidi S, Nguyen TQT. Enophthalmos as the initial systemic finding of undiagnosed metastatic breast carcinoma. Int Med Case Rep J 2021;14:25-31.  Back to cited text no. 3
    
4.
Yılmaz E, Güldoğan N, Arslan A, Civan C. Pleomorphic invasive lobular cancer of the breast presenting with orbital metastasis: A case report. Curr Med Imaging 2022;18:432-5. doi: 10.2174/1573405617666210916115321.  Back to cited text no. 4
    
5.
Safi M, Fethat K, Silkiss RZ. A 'never miss' diagnosis: Ptosis secondary to metastatic breast cancer diagnosed as involutional ptosis and a review of the literature. SAGE Open Med Case Rep 2021;9:2050313X211040680. doi: 10.1177/2050313X211040680.  Back to cited text no. 5
    
6.
Karimaghaei S, Raviskanthan S, Mortensen PW, Malik AI, Lee AG. Metastatic breast cancer presenting as progressive ophthalmoplegia 30 years after initial cancer diagnosis. J Neuroophthalmol 2022;42:e446-7. doi: 10.1097/WNO.0000000000001385.  Back to cited text no. 6
    
7.
Chew C, Wan Hitam WH, Ahmad Tajudin LS. Leptomeningeal carcinomatosis with optic nerve metastasis secondary to breast cancer. Cureus 2021;13:e14200. doi: 10.7759/cureus. 14200.  Back to cited text no. 7
    
8.
Shields JA, Shields CL, Brotman HK, Carvalho C, Perez N, Eagle RC Jr. Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture. Ophthalmic Plast Reconstr Surg 2001;17:346-54.  Back to cited text no. 8
    
9.
Jakobiec FA, Stagner AM, Homer N, Yoon MK. Periocular breast carcinoma metastases: Predominant origin from the lobular variant. Ophthalmic Plast Reconstr Surg 2017;33:361-6.  Back to cited text no. 9
    
10.
Lin IH, Kuo BI, Liu FY. Adjuvant intravitreal bevacizumab injection for choroidal and orbital metastases of refractory invasive ductal carcinoma of the breast. Medicina (Kaunas) 2021;57:404.  Back to cited text no. 10
    
11.
Abemaciclib for breast cancer. Aust Prescr 2020;43:94-5.  Back to cited text no. 11
    
12.
Ahmad SM, Esmaeli B. Metastatic tumors of the orbit and ocular adnexa. Curr Opin Ophthalmol 2007;18:405-13.  Back to cited text no. 12
    


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