Case Reports A Case of Orbital Metastasis of Uterine Leiomyosarcoma With Intracranial Extension Presenting With Proptosis Yeon-Hee Kim, PhD*, In-Ki Park, PhD†, Gyeong Eun Min, PhD‡, Kyung-Hyun Jin, MD†, and Jae-Ho Shin, PhD† Abstract: A 57-year-old woman presented with protrusion of the OS 2 months in duration and decreased vision 1 month in duration. The patient was diagnosed with uterine leiomyosarcoma approximately 3 years before presentation. Lung and liver metastases were diagnosed by biopsy 1 year prior to this visit. CT revealed a solid mass with central necrosis in the left lateral orbit that resulted in orbital shifting, bony destruction, and intracranial extension. The diagnosis was left orbital metastatic leiomyosarcoma, and the patient died 2 months after receiving the diagnosis.

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eiomyosarcoma is a relatively rare malignancy most commonly found in the uterus, bowel, retroperitoneum, or vascular tissue1, 2 and has rarely been encountered in the orbit.3–10 In addition, very few cases of metastatic orbital leiomyosarcoma have been reported in the medical literature. To the authors’ knowledge, only one case of orbital leiomyosarcoma with intracranial extension has been described previously.11 The authors herein report the case of a middle-aged woman with metastatic orbital leiomyosarcoma showing intracranial extension.

CASE REPORT A 57-year-old woman has presented with protrusion of the OS 2 months in duration and decreased vision 1 month in duration. The patient visited this clinic 3 years previously for evaluation of vaginal bleeding and an abdominal mass that protruded into the vagina. After vaginal examination and abdominal CT, the presumed diagnosis was cervical myoma prolapse. The patient underwent subtotal abdominal hysterectomy, and the diagnosis was histologically confirmed as uterine leiomyosarcoma. Approximately 1 year before the current visit, she developed dyspnea and visited the internal medicine subdivision of this clinic. Lung and liver masses were found by chest CT. Ultrasonographyguided liver biopsy was performed, and metastatic leiomyosarcoma was confirmed by histology. The patient received 2 rounds of chemotherapy, but she could not tolerate the treatment and refused further treatment. Approximately 2 months later, she developed exophthalmos of the OS. Corrected visual acuity was 0.6 and 0.04 for the right and OS, respectively. Hertel exophthalmometry revealed 6-mm proptosis, and the left globe was displaced inferomedially. Conjunctival injection and chemosis were observed. No specific findings were observed in the posterior segment. CT scan revealed a 60 × 60 × 50 mm solid, lobulated mass with central necrosis at the lateral wall of the left orbit. The mass resulted in orbital shifting, bony destruction, and intracranial *Department of Obstetrics & Gynecology, Uijeonbu St. Mary Hospital, The Catholic University of Korea College of Medicine, Uijeonbu, Korea; and Departments of †Ophthalmology, and ‡Urology, Kyung Hee University Medical Center, Kyung Hee University, College of Medicine, Seoul, Korea Accepted for publication April 25, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Jae-Ho Shin, Department of Ophthalmology, Kyung Hee University Hospital at Gangdong, #149 Sangildong, Kangdong-gu, Seoul 134-727, Korea. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000229

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extension (Fig.). The patient refused further diagnostic work-up or treatment and died 2 months after diagnosis.

DISCUSSION Metastasis to the orbit is rare and accounts for only 3% to 7% of orbital tumors.11 Metastatic tumors are most common around the age of 60 years. The most common primary cancers leading to metastasis are breast cancer in women and lung and prostate cancer in men. Orbital metastasis may occur many years after the primary tumor has been removed and patients may present with proptosis, ocular motility disorders, or bone destruction.12 Leiomyosarcoma is a relatively rare malignancy that occurs in the smooth muscle and is commonly found in the uterus, bowel, retroperitoneum, or vascular tissue.1, 2 Leiomyosarcoma is common in middle-aged and elderly women (36–84 years of age).13 Leiomyosarcoma is rarely encountered in the orbit, and in addition, few cases of metastatic orbital leiomyosarcoma have been reported in the medical literature. There are several possible etiologies of orbital leiomyosarcoma. Primary orbital leiomyosarcoma may arise from vascular or sympathetic smooth muscle.13 Several cases of primary neoplasms have occurred in patients who received high-dose radiation for retinoblastoma.14–18 These tumors may present with symptoms resulting from direct invasion of nearby tissues, such as sinus and sphenoid bone. Nine cases of orbital metastasis of leiomyosarcoma have been reported to date. The primary tumors originated in the uterus, gastrointestinal tract, vein, spermatic cord, hip, retroperitoneum, or soft tissue of the thigh.3–10 Orbital leiomyosarcoma has no specific clinical symptoms. Because of the relatively fast growth of leiomyosarcoma, patients may experience progressive exophthalmos during the early stages of the disease. Other symptoms may include vision loss, diplopia, gaze limitation, and papilledema.9 The differential diagnosis of a solid, enhancing mass includes both benign (peripheral nerve sheath tumor, cavernous hemangioma, fibrous histiocytoma, etc.) and malignant

Axial and coronal CT scans showing a 60 × 60 × 50 mm solid, lobulated mass with central necrosis at the lateral wall of the left orbit. Bony destruction and intracranial extension are also shown.

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Case Reports

(metastases, sarcoma, lymphoma, etc.) tumors. Due to limitations of imaging, the patient’s medical history is important, and pathologic confirmation is often required. In this case, patient was diagnosed with leiomyosarcoma on the basis of her medical history and imaging findings. The patient refused further diagnostic work-up and treatment. They took into consideration her poor general condition and biopsy results of original uterine tumor and liver mass. In conclusion, metastatic leiomyosarcoma, though rare, should be considered in the differential diagnosis of a rapidly growing orbital mass.

REFERENCES 1. Arnold LM 3rd, Burman SD, O-Yurvati AH. Diagnosis and management of primary pulmonary leiomyosarcoma. J Am Osteopath Assoc 2010;110:244–6. 2. Piovanello P, Viola V, Costa G, et al. Locally advanced leiomyosarcoma of the spleen. A case report and review of the literature. World J Surg Oncol 2007;5:135. 3. Bakri SJ, Krohel GB, Peters GB, et al. Spermatic cord leiomyosarcoma metastatic to the orbit. Am J Ophthalmol 2003;136:213–5. 4. Voros GM, Birchall D, Ressiniotis T, et al. Imaging of metastatic orbital leiomyosarcoma. Ophthal Plast Reconstr Surg 2005;21:453–5. 5. Su GW, Hong SH. Leiomyosarcoma of the uterus with sphenoid bone and orbital metastases. Ophthal Plast Reconstr Surg 2007;23:428–30. 6. Kaltreider SA, Destro M, Lemke BN. Leiomyosarcoma of the orbit. A case report and review of the literature. Ophthal Plast Reconstr Surg 1987;3:35–41. 7. Jakobiec FA, Mitchell JP, Chauhan PM, et al. Mesectodermal leiomyosarcoma of the antrum and orbit. Am J Ophthalmol 1978;85:51–7. 8. Logrono R, Inhorn SL, Dortzbach RK, et al. Leiomyosarcoma metastatic to the orbit: diagnosis of fine-needle aspiration. Diagn Cytopathol 1997;17:369–73. 9. Chen J, Wei R, Ma X. Orbital metastasis of retroperitoneal leiomyosarcoma. Med Oncol 2012;29:392–5. 10. Mariniello G, Vergara P, Del Basso De Caro ML, et al. Intracranial dural metastasis from uterine leiomyosarcoma with orbital extension. Neurol Sci 2012;33:1173–7. 11. Char DH, Miller T, Kroll S. Orbital metastases: diagnosis and course. Br J Ophthalmol 1997;81:386–90. 12. Choi JH, Park IK, Seo KH, Shin JH. A case of dagnosed renal cell carcinoma with orbital metastasis presenting with proptosis. J Korean Ophthalmol Soc 2012;12:1885–8. 13. Lin IC, Wu CT, Liao SL, et al. Primary orbital leiomyosarcoma. Ophthal Plast Reconstr Surg 2005;21:451–3. 14. Klippenstein KA, Wesley RE, Glick AD. Orbital leiomyosarcoma after retinoblastoma. Ophthalmic Surg Lasers 1999;30:579–83. 15. Mihara F, Gupta KL, Kartchner ZA, et al. Leiomyosarcoma after retinoblastoma radiotherapy. Radiat Med 1991;9:183–4. 16. Folberg R, Cleasby G, Flanagan JA, et al. Orbital leiomyosarcoma after radiation therapy for bilateral retinoblastoma. Arch Ophthalmol 1983;101:1562–5. 17. Font RL, Jurco S 3rd, Brechner RJ. Postradiation leiomyosarcoma of the orbit complicating bilateral retinoblastoma. Arch Ophthalmol 1983;101:1557–61. 18. Padrón-Pérez N, Mascaró-Zamora F, Gutiérrez-Miguelez C. Adjuvant pulse dose rate brachytherapy in a secondary leiomyosarcoma of the orbit. Can J Ophthalmol 2013;48:e65–7.

Intramuscular Lipoma of Superior Rectus Muscle Vanessa Hristodulopulos, M.D., and Ramón Medel, M.D. Abstract: The authors describe a case of a 27-year-old man with progressive diplopia and proptosis in whom imaging studies revealed the presence of a mass in the superior rectus muscle. Biopsy showed an infiltrative tumor of mature adipocytes with striated muscle fibres, corresponding to

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an intramuscular lipoma. Although extraocular muscle lipomas are very rare, with only two cases reported, they must be considered in the differential diagnosis of an orbital mass. They may cause significant morbidity to the patient, thus requiring surgical removal. Complete excision is usually difficult because of their infiltrative nature to the surrounding muscular fibres and their tendency to recur.

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xtraocular muscle lipomas are extremely rare. To the authors’ knowledge only two cases have been reported: one of the superior oblique1 and another of the medial rectus muscle.2A further two cases have been published in the periocular area within the orbicularis muscle..3,4 Lipomas represent the most common benign soft tissue mesenchymal tumor (16%). They are composed of mature adipose tissue4 and arise in subcutaneous locations in any part of the body, mainly in the limbs and trunk. Only 20% of lipomas occur in the head and neck region and there are also some reported in the abdominal cavity or mediastinum. In the orbit, lipomas have been reported to occur between 0% and 11% of the cases,1 but some of these may be confused with normal orbital fat and when strict criteria are applied the true rate of incidence is 0.6%.5,6 Less commonly lipomas infiltrate muscle (1.8%), and in 1946 Regan et al. (in Dutton et al)1 classified these kinds of lipomas in a different category. This kind of intramuscular lipomas can be infiltrative or well circumscribed and regarding the head and neck region, they seem to be more common in the oral cavity.1,7

MATERIALS AND METHODS The authors present here the case of a 27-year-old male patient who reported a 6-month history of progressive nonpainful intermittent diplopia. At initial evaluation he had an uncorrected visual acuity of 20/20. Eyelid examination showed vertical interpalpebral fissures of 12 mm OD and 13,5 mm OS; superior margin reflex distance (MRD1) was measured 6 mm OD and 6.5 mm OS, with flattening of eyelid contour in the OD. Levator function was 10 mm OD and 16 mm OS. Superior scleral show was 1 mm OD and 1.5 mm OS, and inferior scleral show was 1 mm OD and 2 mm OS. No lagoftalmus was observed (Fig. 1A). An inferior displacement of the ocular globe and 4 mm of right proptosis (Hertel:18–14/111 mm) was found (Fig. 1B). Resistance to retropulsion was increased on the right side. Motility examination revealed a right hypotropia in primary position of approximately 2 prism dioptres and right hypoglobus, with limited supraduction of the OD and increased diplopia in dextro-supraversion. (Fig. 1C) Biomicroscopy showed the presence of a superior bulbar subconjunctival pale pink lobulated mass starting at 8 mm from the superior limbus, with a horizontal extension of approximately 20 mm (Fig. 1D). CT showed a well-defined lesion measuring 21 (transverse) × 11 (altitudinal) × 25 mm (anteroposterior), with the same density as orbital fat, located between the levator and superior rectus muscles, causing hypoglobus and moderate exophthalmos. On MRI, the lesion had the same signal intensity as orbital fat on T1 and T2 sequences, with a signal decrease on fat suppression. No gadolinium enhancement was found (Fig. 2). A subconjunctival biopsy reported mature adipocyte proliferation crossed by fine connective-vascular tracts and peripheral striated muscle fibers. No atypias, necrosis, or lipoblasts were found. CD34 and Institut de Microcirurgia Ocular. Josep Maria Llado 3, 08035, Barcelona, Spain. Accepted for publication May 6, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Vanessa Hristodulopulos, Institut de Microcirurgia Ocular. Josep Maria Llado 3, 08035, Barcelona, Spain. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000230

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

A Case of Orbital Metastasis of Uterine Leiomyosarcoma With Intracranial Extension Presenting With Proptosis.

A 57-year-old woman presented with protrusion of the OS 2 months in duration and decreased vision 1 month in duration. The patient was diagnosed with ...
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