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. 2022 Feb 15;46(5):343–346. doi: 10.1080/01658107.2022.2034889

Superior Orbital Fissure Syndrome in a Case of Breast Cancer

Divya Deepthi Syamala a, Pavan Raghava Reddy Kalluri b, Hemant Kumar Singh c, Pratyusha Ganne a,
PMCID: PMC9635536  PMID: 36337231

ABSTRACT

Metastasis from breast cancer to the head and neck region is a rare occurrence. However, of all the cancers that metastasise to the orbit, breast cancer is the leading cause in women. Usually, breast cancer metastasises to the fat and muscles in the orbit, and involvement of the bony orbital wall is rare. We report a case of a 52-year-old woman who presented with metastasis to the left greater wing of the sphenoid and adjacent squamous temporal bone. Ophthalmoplegia and proptosis were the initial manifestations in this patient, which lead to the diagnosis of the underlying breast cancer.

KEYWORDS: Breast cancer, metastasis, sphenoid, superior orbital fissure

Case report

A 52-year-old woman presented to the ophthalmology clinic with left orbital pain and drooping of the left upper eyelid for one month. There was no history of fever, weight loss, visual disturbance, vomiting, seizures, or antecedent trauma. She denied noticing any lumps in her body. She had hypothyroidism and was on thyroxine replacement therapy. There were no other medical problems.

Ophthalmological examination revealed a best-corrected visual acuity (BCVA) of 20/30 in her left eye. She had early cataracts in both eyes. Extra-ocular movements of the left eye were restricted in all directions and there was mild proptosis. There was mild ptosis of the left upper eyelid (Figure 1 A-I). Pupillary reflexes and colour vision were normal in both eyes. Corneal sensation was intact and fundus examination was normal bilaterally (Figure 1 J,K). Two weeks following the initial presentation, the drooping and restriction in ocular movements progressed to complete ptosis and total external ophthalmoplegia of the left eye.

Figure 1.

Figure 1.

A-I: Clinical photograph showing mild ptosis, proptosis and external ophthalmoplegia of the left eye. J,K: Fundus photographs of the right and left eyes were normal. L: Clinical photograph of the left breast showing retraction of the nipple and peau d’orange appearance of the skin overlying the tumour.

A detailed systemic examination was undertaken, which revealed a hard lump (7 x 5 cm sized) in the left breast (retro-areolar) with retraction of the nipple, peau d’orange appearance of the overlying skin, and a matted left axillary lymph node (clinical stage: T4bN2) (Figure 1L). A core needle biopsy of the breast lump revealed an infiltrating ductal carcinoma (Figure 2A), which was oestrogen receptor positive (Figure 2B), and progesterone receptor and human epidermal growth factor receptor 2 negative.

Figure 2.

Figure 2.

A: Histopathology of the core needle biopsy specimen from the left breast lump showing breast tissue with infiltrative tumour comprised of ductal epithelial cells arranged as sheets and nests. The nuclei are hyper-chromatic with pleomorphism. Stromal desmoplastic response is also seen. The features are consistent with infiltrating ductal carcinoma B: Positive reaction of oestrogen receptors in the tumour cells.

The results of the blood investigations were as follows: a complete haemogram was normal except for a neutrophilic leukocytosis (14.3 x 103/μl); erythrocyte sedimentation rate was 10 mm/hr; thyroid profile, blood sugars and renal function tests were normal. Contrast-enhanced magnetic resonance imaging (MRI) of the brain and orbits showed a lytic lesion in the left greater wing of the sphenoid and adjacent squamous temporal bone with enhancing soft tissue at the orbital apex causing mass effect on the orbital apical structures (Figure 3A).

Figure 3.

Figure 3.

A: Contrast-enhanced magnetic resonance imaging of the brain and orbits showing a lesion in the left greater wing of the sphenoid and adjacent squamous temporal bone with enhancing soft tissue at the orbital apex causing mass effect on the orbital apical structures (asterisk). B: Whole body fluoro-deoxyglucose (FDG)-positron emission tomography computed tomography (PET-CT) scan showing intensely increased FDG concentration in the lytic lesion with a soft tissue component involving the greater wing of the left sphenoid and squamous part of the left temporal bone. C,D: Whole body FDG PET-CT scan showing increased FDG concentration in the left axillary, sub-pectoral, and internal mammary lymph nodes; and in multiple osteolytic lesions involving both the appendicular and axillary skeleton (arrowed).

A whole-body fluoro-deoxyglucose (FDG)-positron emission tomography computed tomography scan showed intensely increased FDG concentration in the lytic lesion with a soft tissue component involving the greater wing of the left sphenoid and squamous part of the left temporal bone (maximum standardised uptake value [SUV] of 20.1), in the left axillary, sub-pectoral, and internal mammary lymph nodes (maximum SUV of 13.6), and in multiple osteolytic lesions involving both the appendicular and axillary skeleton (maximum SUV of 17.1) (Figure 3B-D).

She was diagnosed with metastatic breast cancer and was started on palliative external beam radiotherapy (30 Greys in ten divided fractions) to the left orbit and chemotherapy (cyclophosphamide with adriamycin).

Discussion

Orbital metastases have been reported in 2–5% of the patients with systemic cancer.1 Orbital metastases are more common with carcinomas than with sarcomas or melanomas. In a study by Valenzuela et al., it was shown that most of the orbital metastases presented in patients with a known primary disease (85%) and orbit was the first presentation in only 15% of the cases.2 Breast cancer commonly metastasises to the bones (ribs, spine, pelvis, and long bones of arms and legs), lung, liver, and brain.3 The head and neck region is an infrequent location for breast cancer metastasis. However, among the cases of orbital metastasis, the most common site of the primary cancer is the breast in women, accounting for 28% – 53% of cases.2,4 Breast cancer mostly metastasises to the fat and muscles in the orbit and involvement of the bony orbital wall is rare.2,5

Breast cancer metastasis to the sphenoid has been rarely reported in the literature. Details of such cases is presented in Table 1. In all these cases, the treatment given was generally palliative to relieve symptoms. This points towards the fact that the prognosis is poor in patients with sinus metastasis from the breast.

Table 1.

Summary of reported cases

Study Age Histological variety Presentation Treatment Outcome
Hiromura et al.6 54 years N/A Presented 7 months after mastectomy and adjuvant chemotherapy with left oculomotor nerve palsy Chemotherapy Patient died 2 months later due to liver metastasis
Xiong et al.7 67 years ER, PR and HER2 negative intra-ductal breast cancer Presented 5 years after the initial diagnosis of breast cancer with right-sided pain and numbness of the face and impaired vision Chemotherapy and radiotherapy Liver and vertebral metastases 32 months later
Imre et al.8 43 years ER and PR positive and HER2 negative breast cancer Presented with orbital apex syndrome Palliative radiotherapy Died 4 months later
Johnston et al.9 75 years ER and PR positive adenocarcinoma of breast Presented 20 years after initial diagnosis with right-sided nasal blockage and pain Chemotherapy N/A
Walker et al.10 62 years ER and PR positive and HER2 negative Presented 4 years after the initial diagnosis of breast cancer with left lateral rectus palsy, ptosis and right-sided nystagmus Radiotherapy N/A
Davey et al.11 75 years ER and PR positive and HER2 negative Presented two years after the initial diagnosis of breast cancer with diplopia and incomplete sixth cranial nerve palsy Radiotherapy Died 1 month later

ER = Oestrogen receptor; PR = Progesterone receptor; HER = Human epidermal growth factor receptor 2; N/A = data not available

Our case is one of the rarer manifestations of metastatic breast cancer wherein the metastasis involved the orbit, the orbital metastasis was the initial presentation (even before the diagnosis of breast cancer was made), and the metastasis involved the orbital wall rather than the fat and muscles of the orbit.

In the current case, the presence of progressive, painful ophthalmoplegia with proptosis, and the presence of lytic lesions on MRI pointed towards a possible underlying malignancy. A thorough systemic examination should be undertaken in such cases to look for the primary tumour. Ophthalmologists can play a crucial role in the diagnosis and prompt referral of patients with metastatic orbital cancer.

Funding Statement

The authors reported there is no funding associated with the work featured in this article.

Disclosure statement

No potential conflict of interest was reported by the authors.

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