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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.
E
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.
Ophthal Plast Reconstr Surg, Vol. 32, No. 3, 2016
Case Reports
FIG. 1. Preoperative and postoperative pictures. Preoperative pictures of the patient showing right hypoglobus, mild right ptosis compared to OS left eyelid retraction (A) 4 mm of right proptosis (B) limited right supraversion (C) and a yellowish pink mass in the superior bulbar conjunctiva (D). Postoperative pictures showing improvement of eyelid height, with right 2-mm ptosis and left eyelid retraction (E) that improved after a phenylephrine test in the OD (G). Right proptosis improved to only 0.5 mm relative to the OS (F).
FIG. 2. Orbital magnetic resonance: Orbit T1 weighted axial (A) and coronal (B, D) images showing an intramuscular high signal intensity mass infiltrating the superior rectus muscle, with the same intensity as orbital fat. Loss of signal intensity on fat suppression and absence of contrast (gadolinium) enhancement are shown (C). Findings are consistent with an intramuscular lipoma. S100 stains were negative. The diagnosis of intramuscular lipoma was made (Fig. 3). Intralesional corticosteroids were injected in an attempt to diminish the size of the tumor, but no improvement of the patient´s symptoms or clinical lesion size was achieved. A decision was made to remove the tumor for functional and aesthetic reasons, despite the known difficulties to achieve complete
excision because of the infiltrative nature of the tumor and the risk of disturbances in ocular motility in a small muscle such as the superior rectus. Using a transconjunctival approach, 10 mm from the superior limbus, a marginal/subtotal and wide but incomplete dissection was performed on the tumor, above the superior rectus muscle, from it’s insertion, as far as possible posteriorly up to the common sheath between the
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Case Reports
FIG. 3. Histopathology of the lesion: Striated muscle fibres of superior rectus muscle (A, arrows) in between mature adipose tissue (A, B star). S100 stain was negative, showing only staining of a nerve (C) and CD 34 stain was negative, showing only staining around vessels (D). Courtesy: Dr Francesc Tressera. Hospital Universitari Quiron Dexeus. Barcelona. Spain.
levator and rectus muscles. The anatomo-pathologic report reconfirmed the diagnosis. Postoperative exophthalmometry was 16,5 mm OD - 16 mm OS base 110 (Fig. 1F). Interpalpebral vertical fissures were 10 mm OD and 12 mm OS, MRD1 was 4 mm and 6 mm respectively, with 2 mm of ptosis in the OD and normal levator function in OU. Phenylephrine test was positive, which showed a good height in OD and improvement of left-eyelid compensatory retraction. Posterior conjunctivomullerectomy of the OD was performed to successfully correct the ptosis (Fig. 1E,G). There has been no recurrence of patient’s symptoms or radiological growth of the tumor after 8 months of bimonthly follow-up.
DISCUSSION Typical lipomas are composed of mature fat cells enclosed by a thin fibrous capsule, whereas intramuscular lipomas do not have a capsule and infiltrate the skeletal muscle, sometimes causing muscle fibre atrophy. Intramuscular lipomas are slow-growing, nonpainful benign tumours. Owing to their deep location and poorly defined borders intramuscular lipomas sometimes may be confused with liposarcomas.4,8 On CT and on T1 and T2 MRI scans lipomas have the same density or intensity as fat and behave similarly. Signal intensity decreases on fat suppression, and lipomas do not enhance with gadolinium. They are visualized as a well-defined mass within the muscle belly that is usually uninodular, although sometimes may be multilobulated. They tend to be homogeneous but can also have low-signal-intensity linear structures corresponding to muscle fibers within it.2,8 On echography they are found to be more echogenic than muscle. Cytogenetic studies of all lipomatous tumors reveal simple translocations, paracentric or pericentric inversions, or loss of chromosomal material involving the q14–15 region of
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chromosome 12, aberrations involving 6p21–22, or loss of material from the q12–14 or q22 region of chromosome 13.9 Dong et al.10 reported 88% (7 of 8) of chromosome 12 abnormalities in patients with intramuscular lipomas.4 Immunohistochemical analysis is infrequently required for diagnosis. Normal adipocytes usually show negativity for CD34 and positivity for S100 protein, but in some cases tumoral adipocytes may lose their antigen expression and stains may be negative or weekly positive, making it unsuitable for determining lineage.11 In other variants of lipomas, however, like spindle or pleomorphic lipomas, spindle and floret cells stain positively for CD34.1,12 The differential diagnosis of intramuscular lipomas has to be made with a well-differentiated liposarcoma. Both lesions are infiltrative and tend to recur. The presence of infiltrative margins and intermingled muscle fibers indicates a benign rather than a malignant lesion, and although lipomas tend to be uninodular masses, liposarcomas are more often multinodular with thick, irregular septa. Furthermore, undifferentiated liposarcomas are composed of fatty tissue and another type of tumor tissue. They show high signal intensity on T2 and unevenly enhance with Gadolinium.8 The histological hallmark of liposarcomas is immature fat cells (lipoblasts). Other features are myxoid differentiation, cellular pleomorphism, plexiform vascularity, atypia, and mitotic activity4. Liposarcomas also may show a giant chromosomic ring in the q12–15 region of chromosome 12.1 Other differential diagnoses to consider are angiolipomas1 or leiomyolipomas.13 Intramuscular lipomas do not metastize unless they transform to liposarcomas, which is very rare.9 Matsumoto reported 1 case of possible malignant transformation.14 The main complications of intramuscular lipomas are due to infiltration of the muscle and symptoms of compression.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 3, 2016
In classical lipomas, measuring less than 2.5 cm, intralesional steroid injections have been suggested to diminish the size of the tumor through lipolysis or local fat atrophy.15,16 Injections of phosphatidylcholine solubilized with deoxycholate, a bile salt, have also been used to treat subdermal lipomas,17 but further studies are necessary to prove their effectiveness. The current recommended management of intramuscular lipomas is surgical removal. Complete excision of the lesion is difficult because of its infiltrative nature, and when excision is incomplete recurrence may occur. In studies involving limbs, head and neck recurrence ranges from 19% to 62.5%.1,9 Well-differentiated liposarcomas tend to recur more often than intramuscular lipomas, and large tumours more so than smaller ones.9 In extra-ocular muscles, resection could lead to significant functional morbidity. In this case the tumor was causing aesthetical and functional impairment. It was decided to reduce tumor size to improve the signs and symptoms. Subtotal excision was performed. Exophthalmos and orbital symmetry improved and diplopia disappeared in primary position, however a non incapacitating diplopia in extreme supraversion persists. The postoperative course remains favourable to date after 8 months. However the risk of recurrence requires long-term follow up.
REFERENCES 1. Dutton JJ, Wright JD Jr. Intramuscular lipoma of the superior oblique muscle. Orbit 2006;25:227–33. 2. Shiraki K, Kamo M, Sai T, et al. Rare site for an intramuscular lipoma. Lancet 2002;359:2077. 3. Charles NC, Palu RN. Intramuscular lipoma of the eyelid. Ophthalmic Surg Lasers 2000;31:340–1. 4. Buller A, O’Donnell A, Bonshek RE, et al. Intramuscular lipoma of the eyelid: a case report. Eye (Lond) 2004;18:743–5. 5. Shah NB, Chang WY, White VA, et al. Orbital lipoma: 2 cases and review of literature. Ophthal Plast Reconstr Surg 2007;23:202–5. 6. Toledano Fernández N, Stoica BT, Genol Saavedra I, et al. Diplopia from pleomorphic lipoma of the orbit with lateral rectus muscle involvement. Ophthal Plast Reconstr Surg 2013;29:e53–5. 7. Furlong MA, Fanburg-Smith JC, Childers EL. Lipoma of the oral and maxillofacial region: Site and subclassification of 125 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:441–50. 8. Matsumoto K, Hukuda S, Ishizawa M, et al. MRI findings in intramuscular lipomas. Skeletal Radiol 1999;28:145–52. 9. Bassett MD, Schuetze SM, Disteche C, et al. Deep-seated, well differentiated lipomatous tumors of the chest wall and extremities: the role of cytogenetics in classification and prognostication. Cancer 2005;103:409–16. 10. Dong SY, Morgan R, Stone J, et al. Translocation (12;14) in lipoma: a case report and review of the literature. Cancer Genet Cytogenet 1998;103:59–61. 1 1. Heim-Hall J, Yohe SL. Application of immunohistochemistry to soft tissue neoplasms. Arch Pathol Lab Med 2008;132: 476–89. 12. Daniel CS, Beaconsfield M, Rose GE, et al. Pleomorphic lipoma of the orbit: a case series and review of literature. Ophthalmology 2003;110:101–5. 13. Borrelli M, Buhlbuck D, Strehl A, et al. Leiomyolipoma of the orbit. Ophthal Plast Reconstr Surg 2012;28:e21–3. 14. Matsumoto H, Hukuda S. Liposarcoma associated with multiple intramuscular lipomas. Clin Orthop Relat Res 2000;373:202–7. 15. Salam GA. Lipoma excision. Am Fam Physician 2002;65:901–4. 16. Lamagna B, Greco A, Guardascione A, et al. Canine lipomas treated with steroid injections: clinical findings. PLoS One 2012;7:e50234. 17. Rotunda AM, Ablon G, Kolodney MS. Lipomas treated with subcutaneous deoxycholate injections. J Am Acad Dermatol 2005;53:973–8.
Case Reports
Tick Infestation of the Eyelid With Histopathologic Characterization Ruju Rai, M.D.*†, Michael K. Yoon, M.D.†‡, and Rebecca C. Stacy, M.D., Ph.D.*† Abstract: Ocular tick infestation is a rare occurrence. The authors report a case that is unique for being the first published example from New England, for its chronic presentation, and for the inclusion of histopathologic analysis in its diagnostic workup. A 75-year-old man was evaluated for a persistent eyelid growth secondary to an incompletely removed tick that had attached 6 months earlier. The lesion was completely excised, and a partially destroyed arthropod was observed embedded within the tissue. Light microscopy demonstrated a mixed granulomatous reaction. Given the disruption of the tick’s anatomy, speciation could not be performed. The patient had an uneventful recovery. A corresponding review of tick bites to the eye is provided.
T
ick attachment to the eye and adnexa is infrequently reported; only 21 instances to the eyelid and 11 instances to the conjunctiva exist in the literature to date.1–20 Among these, just 5 cases occurred in the United States, and none were in the Northeast.5,8,10,19,20 Furthermore, most published reports have focused on clinical manifestations and treatment. To the best of the authors’ knowledge, only 1 former article has incorporated histopathologic representation.20 To add to this scant body of data, the authors delineate a case of tick infestation in the eyelid of a patient residing in New England. They herein emphasize the chronic cutaneous response to the tick and the role of pathologic diagnosis in its management. This study was conducted under the auspices of the Massachusetts Eye and Ear Infirmary Institutional Review Board, in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act, and in adherence to the Declaration of Helsinki and all other relevant federal and state laws.
CASE REPORT A 75-year-old man from Massachusetts with no past ocular history was referred to the ophthalmic plastic surgery service at this institution for evaluation of a medial right upper eyelid nodule. Six months earlier, after walking through a wooded area in Cape Cod, the patient had noticed 3 to 4 ticks attached to his legs, which he removed immediately. A short time later, he discovered a tick on his right upper eyelid and attempted to remove it with his fingers. Though some of the parasite was removed, a portion was retained subcutaneously. After telephone consultation with his primary care physician, the patient was prescribed a single 200-mg dose of doxycycline for Lyme disease prophylaxis. Over the next few weeks, a small, pink, elevated lesion formed at the site of the injury *Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary; †Department of Ophthalmology, Harvard Medical School; and ‡ Department of Ophthalmology, Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A. Accepted for publication May 6, 2014. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Rebecca C. Stacy, M.D., Ph.D., David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, 243 Charles Street–Suite 328, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000231
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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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.
E
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.
Ophthal Plast Reconstr Surg, Vol. 32, No. 3, 2016
Case Reports
FIG. 1. Preoperative and postoperative pictures. Preoperative pictures of the patient showing right hypoglobus, mild right ptosis compared to OS left eyelid retraction (A) 4 mm of right proptosis (B) limited right supraversion (C) and a yellowish pink mass in the superior bulbar conjunctiva (D). Postoperative pictures showing improvement of eyelid height, with right 2-mm ptosis and left eyelid retraction (E) that improved after a phenylephrine test in the OD (G). Right proptosis improved to only 0.5 mm relative to the OS (F).
FIG. 2. Orbital magnetic resonance: Orbit T1 weighted axial (A) and coronal (B, D) images showing an intramuscular high signal intensity mass infiltrating the superior rectus muscle, with the same intensity as orbital fat. Loss of signal intensity on fat suppression and absence of contrast (gadolinium) enhancement are shown (C). Findings are consistent with an intramuscular lipoma. S100 stains were negative. The diagnosis of intramuscular lipoma was made (Fig. 3). Intralesional corticosteroids were injected in an attempt to diminish the size of the tumor, but no improvement of the patient´s symptoms or clinical lesion size was achieved. A decision was made to remove the tumor for functional and aesthetic reasons, despite the known difficulties to achieve complete
excision because of the infiltrative nature of the tumor and the risk of disturbances in ocular motility in a small muscle such as the superior rectus. Using a transconjunctival approach, 10 mm from the superior limbus, a marginal/subtotal and wide but incomplete dissection was performed on the tumor, above the superior rectus muscle, from it’s insertion, as far as possible posteriorly up to the common sheath between the
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Case Reports
FIG. 3. Histopathology of the lesion: Striated muscle fibres of superior rectus muscle (A, arrows) in between mature adipose tissue (A, B star). S100 stain was negative, showing only staining of a nerve (C) and CD 34 stain was negative, showing only staining around vessels (D). Courtesy: Dr Francesc Tressera. Hospital Universitari Quiron Dexeus. Barcelona. Spain.
levator and rectus muscles. The anatomo-pathologic report reconfirmed the diagnosis. Postoperative exophthalmometry was 16,5 mm OD - 16 mm OS base 110 (Fig. 1F). Interpalpebral vertical fissures were 10 mm OD and 12 mm OS, MRD1 was 4 mm and 6 mm respectively, with 2 mm of ptosis in the OD and normal levator function in OU. Phenylephrine test was positive, which showed a good height in OD and improvement of left-eyelid compensatory retraction. Posterior conjunctivomullerectomy of the OD was performed to successfully correct the ptosis (Fig. 1E,G). There has been no recurrence of patient’s symptoms or radiological growth of the tumor after 8 months of bimonthly follow-up.
DISCUSSION Typical lipomas are composed of mature fat cells enclosed by a thin fibrous capsule, whereas intramuscular lipomas do not have a capsule and infiltrate the skeletal muscle, sometimes causing muscle fibre atrophy. Intramuscular lipomas are slow-growing, nonpainful benign tumours. Owing to their deep location and poorly defined borders intramuscular lipomas sometimes may be confused with liposarcomas.4,8 On CT and on T1 and T2 MRI scans lipomas have the same density or intensity as fat and behave similarly. Signal intensity decreases on fat suppression, and lipomas do not enhance with gadolinium. They are visualized as a well-defined mass within the muscle belly that is usually uninodular, although sometimes may be multilobulated. They tend to be homogeneous but can also have low-signal-intensity linear structures corresponding to muscle fibers within it.2,8 On echography they are found to be more echogenic than muscle. Cytogenetic studies of all lipomatous tumors reveal simple translocations, paracentric or pericentric inversions, or loss of chromosomal material involving the q14–15 region of
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chromosome 12, aberrations involving 6p21–22, or loss of material from the q12–14 or q22 region of chromosome 13.9 Dong et al.10 reported 88% (7 of 8) of chromosome 12 abnormalities in patients with intramuscular lipomas.4 Immunohistochemical analysis is infrequently required for diagnosis. Normal adipocytes usually show negativity for CD34 and positivity for S100 protein, but in some cases tumoral adipocytes may lose their antigen expression and stains may be negative or weekly positive, making it unsuitable for determining lineage.11 In other variants of lipomas, however, like spindle or pleomorphic lipomas, spindle and floret cells stain positively for CD34.1,12 The differential diagnosis of intramuscular lipomas has to be made with a well-differentiated liposarcoma. Both lesions are infiltrative and tend to recur. The presence of infiltrative margins and intermingled muscle fibers indicates a benign rather than a malignant lesion, and although lipomas tend to be uninodular masses, liposarcomas are more often multinodular with thick, irregular septa. Furthermore, undifferentiated liposarcomas are composed of fatty tissue and another type of tumor tissue. They show high signal intensity on T2 and unevenly enhance with Gadolinium.8 The histological hallmark of liposarcomas is immature fat cells (lipoblasts). Other features are myxoid differentiation, cellular pleomorphism, plexiform vascularity, atypia, and mitotic activity4. Liposarcomas also may show a giant chromosomic ring in the q12–15 region of chromosome 12.1 Other differential diagnoses to consider are angiolipomas1 or leiomyolipomas.13 Intramuscular lipomas do not metastize unless they transform to liposarcomas, which is very rare.9 Matsumoto reported 1 case of possible malignant transformation.14 The main complications of intramuscular lipomas are due to infiltration of the muscle and symptoms of compression.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 3, 2016
In classical lipomas, measuring less than 2.5 cm, intralesional steroid injections have been suggested to diminish the size of the tumor through lipolysis or local fat atrophy.15,16 Injections of phosphatidylcholine solubilized with deoxycholate, a bile salt, have also been used to treat subdermal lipomas,17 but further studies are necessary to prove their effectiveness. The current recommended management of intramuscular lipomas is surgical removal. Complete excision of the lesion is difficult because of its infiltrative nature, and when excision is incomplete recurrence may occur. In studies involving limbs, head and neck recurrence ranges from 19% to 62.5%.1,9 Well-differentiated liposarcomas tend to recur more often than intramuscular lipomas, and large tumours more so than smaller ones.9 In extra-ocular muscles, resection could lead to significant functional morbidity. In this case the tumor was causing aesthetical and functional impairment. It was decided to reduce tumor size to improve the signs and symptoms. Subtotal excision was performed. Exophthalmos and orbital symmetry improved and diplopia disappeared in primary position, however a non incapacitating diplopia in extreme supraversion persists. The postoperative course remains favourable to date after 8 months. However the risk of recurrence requires long-term follow up.
REFERENCES 1. Dutton JJ, Wright JD Jr. Intramuscular lipoma of the superior oblique muscle. Orbit 2006;25:227–33. 2. Shiraki K, Kamo M, Sai T, et al. Rare site for an intramuscular lipoma. Lancet 2002;359:2077. 3. Charles NC, Palu RN. Intramuscular lipoma of the eyelid. Ophthalmic Surg Lasers 2000;31:340–1. 4. Buller A, O’Donnell A, Bonshek RE, et al. Intramuscular lipoma of the eyelid: a case report. Eye (Lond) 2004;18:743–5. 5. Shah NB, Chang WY, White VA, et al. Orbital lipoma: 2 cases and review of literature. Ophthal Plast Reconstr Surg 2007;23:202–5. 6. Toledano Fernández N, Stoica BT, Genol Saavedra I, et al. Diplopia from pleomorphic lipoma of the orbit with lateral rectus muscle involvement. Ophthal Plast Reconstr Surg 2013;29:e53–5. 7. Furlong MA, Fanburg-Smith JC, Childers EL. Lipoma of the oral and maxillofacial region: Site and subclassification of 125 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:441–50. 8. Matsumoto K, Hukuda S, Ishizawa M, et al. MRI findings in intramuscular lipomas. Skeletal Radiol 1999;28:145–52. 9. Bassett MD, Schuetze SM, Disteche C, et al. Deep-seated, well differentiated lipomatous tumors of the chest wall and extremities: the role of cytogenetics in classification and prognostication. Cancer 2005;103:409–16. 10. Dong SY, Morgan R, Stone J, et al. Translocation (12;14) in lipoma: a case report and review of the literature. Cancer Genet Cytogenet 1998;103:59–61. 1 1. Heim-Hall J, Yohe SL. Application of immunohistochemistry to soft tissue neoplasms. Arch Pathol Lab Med 2008;132: 476–89. 12. Daniel CS, Beaconsfield M, Rose GE, et al. Pleomorphic lipoma of the orbit: a case series and review of literature. Ophthalmology 2003;110:101–5. 13. Borrelli M, Buhlbuck D, Strehl A, et al. Leiomyolipoma of the orbit. Ophthal Plast Reconstr Surg 2012;28:e21–3. 14. Matsumoto H, Hukuda S. Liposarcoma associated with multiple intramuscular lipomas. Clin Orthop Relat Res 2000;373:202–7. 15. Salam GA. Lipoma excision. Am Fam Physician 2002;65:901–4. 16. Lamagna B, Greco A, Guardascione A, et al. Canine lipomas treated with steroid injections: clinical findings. PLoS One 2012;7:e50234. 17. Rotunda AM, Ablon G, Kolodney MS. Lipomas treated with subcutaneous deoxycholate injections. J Am Acad Dermatol 2005;53:973–8.
Case Reports
Tick Infestation of the Eyelid With Histopathologic Characterization Ruju Rai, M.D.*†, Michael K. Yoon, M.D.†‡, and Rebecca C. Stacy, M.D., Ph.D.*† Abstract: Ocular tick infestation is a rare occurrence. The authors report a case that is unique for being the first published example from New England, for its chronic presentation, and for the inclusion of histopathologic analysis in its diagnostic workup. A 75-year-old man was evaluated for a persistent eyelid growth secondary to an incompletely removed tick that had attached 6 months earlier. The lesion was completely excised, and a partially destroyed arthropod was observed embedded within the tissue. Light microscopy demonstrated a mixed granulomatous reaction. Given the disruption of the tick’s anatomy, speciation could not be performed. The patient had an uneventful recovery. A corresponding review of tick bites to the eye is provided.
T
ick attachment to the eye and adnexa is infrequently reported; only 21 instances to the eyelid and 11 instances to the conjunctiva exist in the literature to date.1–20 Among these, just 5 cases occurred in the United States, and none were in the Northeast.5,8,10,19,20 Furthermore, most published reports have focused on clinical manifestations and treatment. To the best of the authors’ knowledge, only 1 former article has incorporated histopathologic representation.20 To add to this scant body of data, the authors delineate a case of tick infestation in the eyelid of a patient residing in New England. They herein emphasize the chronic cutaneous response to the tick and the role of pathologic diagnosis in its management. This study was conducted under the auspices of the Massachusetts Eye and Ear Infirmary Institutional Review Board, in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act, and in adherence to the Declaration of Helsinki and all other relevant federal and state laws.
CASE REPORT A 75-year-old man from Massachusetts with no past ocular history was referred to the ophthalmic plastic surgery service at this institution for evaluation of a medial right upper eyelid nodule. Six months earlier, after walking through a wooded area in Cape Cod, the patient had noticed 3 to 4 ticks attached to his legs, which he removed immediately. A short time later, he discovered a tick on his right upper eyelid and attempted to remove it with his fingers. Though some of the parasite was removed, a portion was retained subcutaneously. After telephone consultation with his primary care physician, the patient was prescribed a single 200-mg dose of doxycycline for Lyme disease prophylaxis. Over the next few weeks, a small, pink, elevated lesion formed at the site of the injury *Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary; †Department of Ophthalmology, Harvard Medical School; and ‡ Department of Ophthalmology, Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A. Accepted for publication May 6, 2014. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Rebecca C. Stacy, M.D., Ph.D., David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, 243 Charles Street–Suite 328, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000231
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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