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Slide 1-

Raafay Sophie Grand Rounds Orbital Mass
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CC Left eye “protruding out” that was noticed a week ago HPI 13 y F who was sent to the ED after being seen in outside eye clinic Painless clear tearing OS started 6 months ago Complaint of mild diplopia recently No redness, pain, photophobia, flashes, floaters, Patient Presentation
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Past Ocular Hx: Myopia Past Medical Hx: Unremarkable Meds: None Allergies: NKDA Social Hx: Unremarkable, Vietnamese Fam Hx: Paternal grandmother with Breast CA Maternal grandfather with Lung CA (smoker) History (Hx)
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ROS Denies any fever, weight loss, headaches, sinusitis like symptoms or respiratory problems. History (Hx)
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External Exam
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103 20.5 18
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-1 0 0 0 -1 -2
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Anterior Segment Exam
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Posterior Segment Exam No Lymphadenopathy on Physical Exam
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CBC CMP LDH Uric acid All within normal limits Labs
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CT
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CT
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CT
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CT
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MRI
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MRI
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MRI
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Differential Diagnosis benign mixed tumor lacrimal gland carcinoma lymphoproliferative disorder idiopathic inflammatory pseudotumor nodular fasciitis rhabdomyosarcoma chloroma (myeloid sarcoma) Assessment
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Proceed with lateral orbitotomy and excision of lesion Plan
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Biopsy
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Biopsy
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Pleomorphic Adenoma 3-5% of all orbital tumors 25 % of all lacrimal gland tumors 50% of epithelial lacrimal gland tumors Usually occurs in adults during 4th or 5th decade Slightly affects M>F Discussion
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Most common presentation is progressive downward displacement of the globe Symptoms are usually present for 12 months or more Firm lobular mass may be palpated near superolateral rim Imaging Usually well-circumscribed, solid lesion Enlargement of lacrimal fossa with bone remodeling, occasional calcification Discussion
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Also known as Benign Mixed Tumor Histology: Varied cellular structure with proliferation of benign Epithelial cells that form ductal structures Stroma composed of spindle shaped cells with occasional cartilaginous, mucinous or even osteoid degeneration or metaplasia Lesions usually circumscribed by pseudocapsule Discussion
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Treatment & Prognosis Complete removal of tumor with its pseudocapsule 5-year recurrence rate was 32% when capsule was incised for direct biopsy (vs 3%) In recurrences, risk of malignant degeneration is 10% per decade Discussion
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Adenoid Cystic CA Rapid onset of growth, may have pain due to perineural infiltration CT scan shows irregular border, nodularity, along with bone destruction. Malignant Mixed Recurrence after removal of benign lesion, sudden growth of previous benign lesion, or rapid denovo growth with pain and aggressive bone destruction Discussion
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12 Cases in the literature to date: 13 year M - Excision THEODORE E. SANDERS.MIXED TUMOR OF THE LACRIMAL GLAND. Arch Ophthalmol. 1939;21(2):239-260 16 M with exophthalmos since 10 years- excision Rubin IE. Adenocarcinoma of the lacrimal gland following a mixed tumor of 25 years' duration. Arch Ophthal. May 1947;37(5):686-688. 7 year old M-excision SD McPherson. Mixed tumor of the lacrimal gland: in a seven-year-old boy. Am J Ophthalmol, 61 (1966), pp. 561–563 16 year old F- excision Tsunoda S, Yabuno T, Sakaki T, Morimoto T, Moshida T, Hirabayashi M et al. Pleomorphic adenoma of the lacrimal gland manifesting as exophthalmos in adolescence. Neurol Med Chir 1994; 34: 814–816. 6 year old M- excision Faktorovich EG, Crawford JB, Char DH, Kong C. Benign mixed tumor (pleomorphic adenoma) of the lacrimal gland in a 6-year-old boy. Am J Ophthalmol. Sep 1996;122(3):446-447. 15 year old F- excision Mercado GJ, Gunduz K, Shields CL, Shields JA, Eagle JA, Eagle RC. Pleomorphic adenoma of the lacrimal gland. Arch Ophthalmol 1998 15 year old- Excision Mercado GJ, Gunduz K, Shields CL, Shields JA, Eagle RC, Jr. Pleomorphic adenoma of the lacrimal gland in a teenager. Arch Ophthalmol. Jul 1998;116(7):962-963 10 year old F- excision Cates AC, Manners RM, Rose GE. Pleomorphic adenoma of the lacrimal gland in a 10 year old girl, a case report. Br J Ophthalmol 2002; 86 (2): 249–250 14 year old- FNAC then excision Fenton S, Sie Go DM, Mourits MP. Pleomorphic adenoma of the lacrimal gland in a teenager, a case report. Eye (Lond). Jan 2004;18(1):77-79. 7 year old M- FNAC then excision Gupta A, Khandelwal A. Lacrimal gland pleomorphic adenoma: an inconceivable diagnosis in a child. BMJ Case Rep. 2013;2013. 2 more cases- have been referenced to in Book Chapter Font RL, Gamel JW. Epithelial tumours of the lacrimal gland; an analysis of 265 cases. In: Jakobiec FA (ed). Ocular and Adnexal Tumours. Aesculapius Publishing Co: Birmingham, Ala, 1978, pp 787–805 Discussion
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Pleomorphic Adenoma 3-5% of all orbital tumors 50% of epithelial lacrimal gland tumors Causes slow progressive displacement of globe without symptoms of pain CT shows well circumscribed lesion with bone remodeling but no destruction Excision for treatment (not incisional biopsy!) Usually, but not exclusively in adults Conclusions
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BCSC Orbit Eyelids and Lacrimal System- Chapter 5- Orbital Neoplasms and malformations THEODORE E. SANDERS.MIXED TUMOR OF THE LACRIMAL GLAND. Arch Ophthalmol. 1939;21(2):239-260 Rubin IE. Adenocarcinoma of the lacrimal gland following a mixed tumor of 25 years' duration. Arch Ophthal. May 1947;37(5):686-688. SD McPherson. Mixed tumor of the lacrimal gland: in a seven-year-old boy. Am J Ophthalmol, 61 (1966), pp. 561–563 Tsunoda S, Yabuno T, Sakaki T, Morimoto T, Moshida T, Hirabayashi M et al. Pleomorphic adenoma of the lacrimal gland manifesting as exophthalmos in adolescence. Neurol Med Chir 1994; 34: 814–816. Faktorovich EG, Crawford JB, Char DH, Kong C. Benign mixed tumor (pleomorphic adenoma) of the lacrimal gland in a 6-year-old boy. Am J Ophthalmol. Sep 1996;122(3):446-447. Mercado GJ, Gunduz K, Shields CL, Shields JA, Eagle JA, Eagle RC. Pleomorphic adenoma of the lacrimal gland. Arch Ophthalmol 1998 Mercado GJ, Gunduz K, Shields CL, Shields JA, Eagle RC, Jr. Pleomorphic adenoma of the lacrimal gland in a teenager. Arch Ophthalmol. Jul 1998;116(7):962-963 Cates AC, Manners RM, Rose GE. Pleomorphic adenoma of the lacrimal gland in a 10 year old girl, a case report. Br J Ophthalmol 2002; 86 (2): 249–250 Fenton S, Sie Go DM, Mourits MP. Pleomorphic adenoma of the lacrimal gland in a teenager, a case report. Eye (Lond). Jan 2004;18(1):77-79. Gupta A, Khandelwal A. Lacrimal gland pleomorphic adenoma: an inconceivable diagnosis in a child. BMJ Case Rep. 2013;2013. Font RL, Gamel JW. Epithelial tumours of the lacrimal gland; an analysis of 265 cases. In: Jakobiec FA (ed). Ocular and Adnexal Tumours. Aesculapius Publishing Co: Birmingham, Ala, 1978, pp 787–805 References