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Metastatic Disease to Extraocular Muscles of Undiscovered Primary Breast Cancer Maria E. Lim, BS 1 Sergul A. Erzurum, MD, FACS 1, 2 1. Department of Surgery, Northeastern Ohio Universities College of Medicine, Rootstown, OH 44272 2. Section of Ophthalmology in the Department of Surgery at St. Elizabeth Health Center, Youngstown, OH 44501 Authors acknowledge the financial support of the Polena Trust for Ocular Research at the St. Elizabeth Development Foundation,Youngstown, Ohio. Statements are the sole responsibility of the authors. Authors have no financial conflict of interest associated with products described in the report. 57 year old Caucasian female History of Present Illness (Feb 2010) Chief complaint: diplopia at distance x 3-5 weeks ◦ Resolved when left eye closed Negative for: ◦ Orbital/retroorbital pain ◦ Change in vision Physical Exam OD OS BCVA: 20/20 Tonometry: 20 SLE: NL Fundus: ◦ flame hemmorrhage off disc noted EOM movement: full in all direction Hertel: 16 mm ◦ ◦ ◦ ◦ ◦ ◦ ◦ ◦ base setting 93 PERRL Neurologic exam Cranial nerve exam, strength, movement, deep tendon reflexes, sensation, and coordination were all within normal limits. Lumbar puncture: ◦ No malignant cells BCVA: 20/20 Tonometry: 23 SLE: NL Fundus: NL EOM movement: -3 limitation of abduction -4 limitation of elevation Distance fixation: 10∆ RHT Near fixation: 1-2∆ intermittent RHT Right gaze: 8∆ RHT Left gaze: 14∆ RHT, 2∆ XT Upward gaze: approximately 25-20∆ RHT (+) retropulsion Hertel: 16 mm ◦ base setting 93 PERRL Past Medical History July 2006: Palpable left axillary lymphadenopathy Nov 2006: Left axillary lymph node dissection ◦ Pathology: poorly differentiated adenocarcinoma consistent with breast origin Oct 2007: ↑ CEA & CA 27-29 ◦ PET scan, B/L breast MRI, & CT abdomen & pelvis ◦ All findings benign May 2008: Bone metastasis, right ovarian metastasis, and retroperitoneal adenopathy ◦ ER (+), PR (-), Her-2/neu (-) in 14/15 nodes ◦ Treatment: capecitabine and pamidronate ◦ Lymphoproliferative disease markers (-) ◦ Right ureteral stent placement ◦ Patient received doxorubicin, cyclophosphamide, paclitaxel x 5 months then radiation and anastrozole. July 2009: Liver metastasis ◦ Treatment: gemcitabine and paclitaxel Jan 2010: Worsening liver lesions ◦ Treatment: 3 cycles doxorubicin MRI brain/orbits (Feb 11, 2010): Thickening of the extraocular muscles bilaterally especially on the medial inferior left side. CT scan orbits (Feb 27, 2010): Soft tissue masses of the left medial and inferior rectus muscle sheaths displacing the optic nerve superiorly and laterally. No involvement of the optic nerve or orbital bones. Mild hypertrophy of the right rectus muscles was noted. Patient Course Treatment: ◦ Radiation to the left eye and orbit ◦ Continued systemic treatment ◦ Developed thrombocytopenia secondary to therapy. March 2010: admitted to the Medical ICU for profuse rectal bleeding and hypovolemic shock ◦ Received packed red blood cells, platelets, and IV resuscitation. ◦ Mental status deterioration ◦ MRI of the brain: Multi-infarct changes. After much discussion, the patient and her family opted for Hospice care and she soon passed away. Metastasis to the Orbit Prevalence :1-13% of all orbital tumors1. Often unilateral Typically involves orbital bone and fat Most common primary origins from the breast, prostate, lung 2. 9% of all metastatic disease involves extraocular muscles ◦ even fewer involve more than two muscles 3. Breast Cancer in the EOM Literature classically describes scirrhous type breast cancer ◦ presents with enophthalmos from fibrosis & contracture of invaded tissue ◦ localize in orbital fat 4, 5. 75% of breast cancer metastases to the orbit have a primary tumor Average time between discovery of primary tumor & ophthalmic presentation: 3 years 5. Mean survival time after orbital manifestation of metastasis: 22 months 6. Differential Diagnosis Thyroid ophthalmoplegia ◦ Extraocular muscle swelling ◦ Exophthalmos with lid retraction and lid lag ◦ Systemic manifestation of hyperthyroidism Orbital pseudotumor ◦ Acute onset of pain 2 Leptomeningeal or posterior fossa metastasis ◦ Typically involves cranial nerves or long nerve tracts 8 Cranial nerve palsies Lymphoma ◦ 10-15% of orbital lesions 2, 9 ◦ Skeletal muscle metastasis commonly harbor leukemia or lymphoma In Conclusion Patient presented with left eye limitation of elevation and abduction No orbital biopsies, due to patient’s overall prognosis ◦ Images showed extraocular muscle thickening bilaterally ◦ Metastatic disease was presumed Patient had a four year course of metastatic spread to the bone, liver, retroperitoneal lymph nodes, and ovaries While the patient was initially diagnosed with breast cancer from axillary lymph node involvement in 2006, no primary tumor in the breast was found after repeated MRI, mammography, or physical exam. 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Luneau K, Falardeau J, Hardy I, Boulos PR, Boghen D. Ophthalmoplegia and lid retraction with normal initial orbit CT imaging in extraocular muscle metastases as the presenting sign of breast carcinoma. J Neuro-Ophthalmol. 2007; 27 (2): 144-6. 8. Heijden A, Twijnstra A, Lamers W, Hupperets P, Freling G. An unusual cause of diplopia in a cancer patient. Eur J Cancer. 1991; 27(1): 1315-6. 9. Weiss R, Grisold W, Jellinger K, Muhlbauer J, Scheiner W,Vesely M. Metastasis of solid tumors in extraocular muscles. Acta Neuropathol. 1984; 65: 168-71.