Clinical Review & Education

JAMA Ophthalmology Clinical Challenge

Numbness of the Forehead Sarah W. DeParis, MD; F. Lawson Grumbine, MD; M. Reza Vagefi, MD

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Figure 1. Magnetic resonance imaging of the orbits. An 86-year-old man presents with an infiltrating mass of the left superomedial orbit demonstrated on T1-weighted axial (A) and coronal (B) images.

An 86-year-old man presented with left forehead numbness and intermittent left eyelid edema for the past several months. His medical history was significant for hypertension, gastroesophageal reflux, and prostate cancer. On examination, uncorrected visual acuity was 20/25 OU, pupils were equal and reactive to light Quiz at without afferent defect, confrontational jamaophthalmology.com fields were normal, and ocular motility was full. External examination was significant for decreased sensation in the distribution of the ophthalmic branch of the left trigeminal nerve. A palpable mass was appreciated at the left supratrochlear notch. There was 4 mm of proptosis and 2 mm of inferior globe dystopia, with increased resistance to retropulsion in the left eye. Magnetic resonance imaging of the orbits demonstrated an infiltrating superomedial mass involving the extraconal and intraconal space on the left side (Figure 1).

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WHAT WOULD YOU DO NEXT?

A. Observe B. Administer empirical corticosteroid treatment C. Perform external beam radiotherapy D. Perform orbitotomy with biopsy

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Clinical Review & Education JAMA Ophthalmology Clinical Challenge

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Figure 2. Histopathology of the orbital tumor. A lobular configuration of neoplastic cells (A, hematoxylin and eosin, original magnification ×40) stain positive for prostatic-specific acid phosphatase (B, original magnification ×20)

and P501S (C, original magnification ×20), consistent with a diagnosis of metastatic prostate carcinoma.

Diagnosis

D. Perform orbitotomy with biopsy Given the patient’s history of systemic cancer and presentation with an orbital mass, metastatic prostate carcinoma is high on the differential diagnosis. However, lymphoma, primary tumors of the orbit, and metastasis from a different malignancy must also be considered. As such, biopsy and histopathologic examination of tissue is indicated for diagnostic confirmation, and observation is not a reasonable consideration. Orbitotomy is preferred for biopsy when the tumor is easily accessible in an anterior position. In tumors that are more posterior, fine-needle aspiration may be considered. Less commonly, in some cases where disseminated metastatic cancer is already an established diagnosis and the risks of surgery outweigh the benefits, it may be reasonable to forgo biopsy and proceed to treatment.1 The patient’s presentation was without pain and slowly progressive. Thus, it is not typical for an orbital inflammatory syndrome, which most commonly involves the lacrimal gland and/or extraocular muscles. Therefore, empirical steroids would not be a prudent option. After the diagnosis is substantiated, radiotherapy is the preferred treatment method, with a primary aim of palliation of symptoms because it does not prolong survival.2 Complete surgical resection is not indicated in suspected cases of metastasis to the orbit. The patient underwent anterior orbitotomy with biopsy through an upper eyelid crease incision, and pathology was consistent with metastatic prostate carcinoma (Figure 2).

or third most prevalent primary malignancy, with breast and lung cancers also commonly seen.1 The most frequent presenting symptoms are proptosis, diplopia, pain, eyelid edema, ptosis, and decreased visual acuity.2,3 This case was unusual in that the patient presented with hypesthesia in the distribution of the ophthalmic division of the trigeminal nerve as the primary symptom, which resulted from tumor impingement of the sensory nerve. Trigeminal neuropathy is an uncommon finding in metastatic prostate carcinoma but has been reported in one case with metastases to the skull base4 and in another with hypesthesia in the distribution of the mandibular branch of the trigeminal nerve.5 In addition, the presentation is atypical in that it involves metastasis to the orbital soft tissues without bony involvement. In general, metastatic prostate cancer of the orbit primarily involves the orbital bones, with osteoblastic lesions seen most commonly and osteolytic less frequently.2 Metastasis of prostate carcinoma confined to the orbital soft tissues has seldom been reported,2 with metastasis to the optic canal resulting in compressive optic neuropathy even more infrequent.6 In summary, manifestations of orbital metastases are protean and should be considered whenever a patient presents with an unusual cranial neuropathy. Orbital imaging is essential followed by biopsy with histopathologic examination of tissue for diagnostic confirmation, especially in cases of clinical uncertainty. Treatment of orbital metastases in prostate carcinoma is palliative and prognosis is poor, with a median survival after diagnosis of 2.5 years.7

Discussion

Patient Outcome

Metastatic tumors of the orbit represent 1% to 13% of all orbital lesions.3 Among orbital metastases, prostate cancer is the second

The patient received palliative radiotherapy and died of metastatic disease 3 months after diagnosis.

Metastatic prostate carcinoma of the orbit

What To Do Next

ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, University of California, San Francisco. Corresponding Author: M. Reza Vagefi, MD, Department of Ophthalmology, University of California, San Francisco, 10 Koret Way, San Francisco, CA 94143 ([email protected]). Conflict of Interest Disclosures: None reported. REFERENCES 1. Shields JA, Shields CL, Brotman HK, Carvalho C, Perez N, Eagle RC Jr. Cancer metastatic to the

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orbit. Ophthal Plast Reconstr Surg. 2001;17(5):346354. 2. Boldt HC, Nerad JA. Orbital metastases from prostate carcinoma. Arch Ophthalmol. 1988;106 (10):1403-1408. 3. Ahmad SM, Esmaeli B. Metastatic tumors of the orbit and ocular adnexa. Curr Opin Ophthalmol. 2007;18(5):405-413. 4. Long MA, Husband JE. Features of unusual metastases from prostate cancer. Br J Radiol. 1999; 72(862):933-941. neuropathy: presentation of 7 cases. Med Oral Patol Oral Cir Bucal. 2006;11(2):.16505784

6. Galloway G, McMullan T, Shenoy R, Jones C. Rapid bilateral sequential visual loss secondary to optic canal metastases in prostatic carcinomatosis. Eye (Lond). 2003;17(4):539-540. 7. Aus G, Robinson D, Rosell J, Sandblom G, Varenhorst E; South-East Region Prostate Cancer Group. Survival in prostate carcinoma: outcomes from a prospective, population-based cohort of 8887 men with up to 15 years of follow-up: results from three countries in the population-based National Prostate Cancer Registry of Sweden. Cancer. 2005;103(5):943-951.

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