Surgery for orbital metastasis from breast carcinoma initially presenting with progressive proptosis

Abstract

We present a 45-year-old woman with metastatic breast disease who initially presented with progressive proptosis of her right eye causing limited motility, diplopia and eye pain. MRI done showed an avidly enhancing right sphenoorbital mass causing displacement in the lateral and superior recti muscles with lysis and infiltration of the greater sphenoid wing and lateral orbital wall. The patient underwent surgery resulting in immediate relief of proptosis and resolution of symptoms. Although surgery is not recommended for orbital metastasis as it is not curative, it should be considered as a treatment option as it can provide relief to patients and improve their quality of life.

Background

Orbital metastasis is an infrequent cause of proptosis and is usually seen in patients harbouring advanced metastatic disease.^{1 2} For breast cancer, the time from diagnosing the primary disease to diagnosing the orbital metastasis varies from 4.5 to 6.5 years.^{1 3} In 25% of cases, orbital metastasis can be the initial finding for a previously unknown primary cancer and this diagnosis if often unexpected.^{2 4–6} Here, we report a case of metastatic breast carcinoma initially presenting with eye proptosis from a sphenoorbital mass treated with surgery.

Case presentation

A 45-year-old woman who was previously well with good functional capacity and no known comorbidities sought consultation for a 2-month history of progressive proptosis of her right eye which caused significant personal distress. She also presented with diplopia and eye pain. On neurologic examination, vision was 20/30 on both eyes but she had limited abduction and elevation of her right eye. On physical examination, she had a firm, non-movable 7×4×3 cm right breast mass with palpable axillary and supraclavicular lymph nodes. MRI done revealed a 4×2.5×3 cm T1-hypo, T2-hyper, avidly enhancing right sphenoorbital mass causing displacement of the optic nerve as well as the lateral and superior recti muscles with lysis and infiltration of the greater sphenoid wing and lateral orbital wall (figure 1). Further workup revealed multiple pulmonary nodules on bilateral lungs as well as lytic lesions at the right scapula and proximal humerus. A core needle biopsy was done for the breast mass which revealed invasive carcinoma with mucinous features with immune. The patient underwent right pterional craniotomy and excision of the sphenoorbital mass. Intraoperatively, the tumour was grey-white, moderately vascular and fairly suctionable. The part of the tumour infiltrating into the sphenoid and lateral orbital wall was drilled away. The underlying dura was indurated and grossly abnormal. This was excised along with the small intradural component underneath. After excision, the orbit was decompressed with immediate resolution of the proptosis (figure 2). Histopathologic analysis of the tumour revealed mucinous carcinoma (positive for mammaglobin and GATA3) which was consistent with the core needle biopsy results of the breast mass.

Figure 1.

Plain T1-weighted axial (A), contrast (B), coronal (C), sagittal (D) and T2-weighted axial (E) images show the avidly enhancing mainly orbital mass with extension to the sphenoid.

Figure 2.

Preoperative (above) and postoperative (below) comparison between the degree of proptosis and its resolution after the surgery.

Outcome and follow-up

Postoperatively the patient did not have any new neurologic deficits with improvement of her diplopia. The plan for the patient is to do whole-brain radiotherapy as well as to start the patient on anthracycline-based followed by plantinum-based systemic chemotherapy. Concomitant administration of bisphosphonate treatment with zoledronic acid 4 mg/month was also intended to be given for the bone metastasis and hormonal therapy was to be initiated after chemotherapy. However, the patient refused any further treatment. Currently, she has no new symptoms at 6 months follow-up.

Discussion

Breast cancer accounts for 28.5%–58.8% of all orbital metastases.^{1 4 7} The clinical presentation of orbital metastasis can be varied but it is usually not prolonged. The presentation can be as short as 2 months, which was similar to our patient.⁸ Diplopia is usually the most common symptom due to the preference of breast cancer in infiltrating the extraocular muscles and periorbital fat.^{4 5 8} Other symptoms such as proptosis, pain and strabismus can also present early on in the disease.⁸ Metastatic tumours are usually seen unilaterally and are predominantly found at the lateral and superior quadrants of the orbital complex, which was also seen in our case.^{1 3 4}

Treatment for breast carcinoma is multidisciplinary and involves a multifaceted approach which includes surgery, chemotherapy, radiotherapy, hormonal and targeted therapy.⁸ However, palliation is usually the main goal of treatment for patients with metastatic orbital disease as this usually means that there is systemic disease and that other sites have been invariably affected as seen in our patient.^{1 5 9} Treatment aims to relieve symptoms as well as improve and preserve the quality of life of patients.⁹ According to the literature, radiotherapy remains to be the mainstay of treatment which is given at 20–40 Gy divided into 1–2 weeks.^{1 5} Radiotherapy can improve symptoms in up to 80% of cases but patients should be aware of potential side effects such as cataract formation and retinopathy which could severely impact the quality of life of patients.^{2 9} Systemic chemotherapy is another mainstay of treatment due to the fact that most patients also have concomitant progressive systemic disease.^{5 9} In addition, bisphosphonate treatment should also be included in the presence of bone metastasis.⁹ This can further be followed by hormonal therapy as the hormonal status of the disease permits thereby providing a more robust palliation than when used in isolation.^{5 10} Case reports and case series reporting breast cancer metastasising to the orbit were reviewed after an exhaustive search in the literature and only 37 patients had their treatment regimen specified. According to the review, 33 patients underwent palliation either through chemotherapy, hormonal therapy, radiotherapy or combinations thereof.1 3–5 9–35 Out of the 33 patients, 27% had complete resolution of symptoms, 48% had partial resolution of symptoms, 3% had worsening of symptoms and 9% died. The remaining 12% had no outcomes specified. This highlights the fact that majority do not achieve symptom freedom.

Surgery is not currently recommended by most authors as it may be associated with severe ocular morbidity.^{1 5 9} However, data are sparse with regard to this as there are only four other cases reported who underwent surgery for orbital metastasis from breast cancer. Based on the review, one case had complete recovery, one case had no change in symptoms and two cases had no outcomes specified.^36–39 Therefore, surgery as a treatment option should be considered especially for patients presenting with proptosis, intractable ocular pain or diplopia.¹ Although the treatment is not curative, it can provide a drastic improvement in the quality of life of patients. As seen in our case, surgical treatment provided complete and immediate resolution of symptoms thereby improving the quality of life of our patient. Despite all the available treatment options, orbital metastatic disease carries a poor prognosis with a mean survival of 22–31 months.^{1 9} Therefore, every effort should be made to always take in consideration the impact of our palliative treatment to the quality of life of the patient.

Learning points.

• Ocular manifestations can be the initial presentation of metastatic disease.

• Surgery should be considered for patients presenting with proptosis, intractable ocular pain or diplopia.

• This is the second case documenting complete and immediate resolution of symptoms of orbital metastasis due to surgical treatment.

• Every effort should be made to always take in consideration the impact of our palliative treatment to the quality of life of the patient.