Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
Case Reports
side effects, but with good efficacy, have been reported for the use of IVIG as a treatment for several autoimmune diseases.6,8 Unfortunately, recurrences after finishing therapy are frequent. The first patient responded well to combined immunoglobulin infusions and steroids. Our second patient highlights the role of conservative treatment where surgical intervention can have significant risks.
REFERENCES FIG. 1. Periocular eyelid swelling and thickening of the skin in the first patient.
FIG. 2. Thickening of the upper eyelid skin and lateral lower eyelid ectropion in the second patient.
gastrointestinal dysmotility, muscle weakness, joint contractures, and neurologic symptoms (such as seizures, encephalopathy, and coma), and obstructive or restrictive pulmonary disease. Ophthalmic manifestations include corneal opacities, thickened eyebrow or eyelid skin, lagophthalmos, ectropion, choroidal folds, and papilledema.4 In their case series, Davis et al.4 reported 4 patients who presented with thickened eyebrow or eyelid skin. In the same case series, 2 patients had a lower eyelid ectropion similar to our second patient, and 1 patient was found to have lagophthalmos.4 The diagnosis requires the histologic demonstration of dermal mucin with increased collagen deposition and fibroblast proliferation and the presence of a serum monoclonal gammopathy.2 Histopathological analysis of skin samples stained with hematoxylin, eosin, and colloidal iron can demonstrate dermal mucin. The epidermis is spared, and there is a proliferation of predominantly spindle and epithelioid cells with a fibromyxoid stroma containing thick bundles of collagen and increased mucin. The degree of cellularity ranges from scattered cells in the dermis to markedly hypercellular infiltrates involving the entire reticular dermis. Immunohistochemical studies can detect expression of factor XIIIa (a marker of dermal dendrocytes) and procollagen 1 (a precursor of collagen 1). The expression of factor XIIIa can be seen in up to 50% of cells in the dermis, consistent with a proliferation of dermal dendrocytes. Procollagen 1 is expressed in the basement membrane area.1 There is no established standard therapy for the systemic treatment of scleromyxedema and the prognosis is guarded.5 However, several treatment modalities including steroids, melphalan, cyclophosphamide, interferon alpha, and plasmapheresis have been suggested.2,5,6 Steroids alone are ineffective.5 The low-dose melphalan and steroids are a popular treatment regimen with mixed results and concerns about the risks of infection and secondary malignancy.7 Donato et al.2 present their observations of high-dose melphalan treatment with autologous stem cell transplantation, which has shown to be extremely successful in resolution of the cutaneous lesions and complete remission of all gastrointestinal, pulmonary, and even neurologic manifestations. Rongioletti et al.5 present their experience in treatment with IVIGs (alone or in combination with other drugs), which have induced complete remission in some of their patients with a mean treatment duration of 2 years. Only a few
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1. Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273–81. 2. Donato ML, Feasel AM, Weber DM, et al. Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation. Blood 2006;107:463–6. 3. Cokonis Georgakis CD, Falasca G, Georgakis A, et al. Scleromyxedema. Clin Dermatol 2006;24:493–7. 4. Davis ML, Bartley GB, Gibson LE, et al. Ophthalmic findings in scleromyxedema. Ophthalmology 1994;101:252–5. 5. Rongioletti F, Merlo G, Cinotti E, et al. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol 2013;69:66–72. 6. Bidier M, Zschoche C, Gholam P, et al. Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol 2012;92:408–9. 7. Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol 1995;33:37–43. 8. Enk A; European Dermatology Forum Guideline Subcommittee. Guidelines on the use of high-dose intravenous immunoglobulin in dermatology. Eur J Dermatol 2009;19:90–8.
Orbital Metastasis of Undifferentiated/Anaplastic Thyroid Carcinoma Divakar Gupta, M.D.*, Michael Chappell, M.D.*, Tina D. Tailor, M.D.†, C. Dirk Keene, M.D., Ph.D.*‡, Kris Moe, M.D.§, Arash Jian-Amadi, M.D.*, and Shu-hong Chang, M.D.* Abstract: A 52-year-old woman presented with decreased vision, diplopia, esotropia, proptosis, and right orbital pain. Clinical examination was suspicious for an orbital mass and additionally revealed a thyroid gland mass. Imaging studies showed an enhancing mass within the right lateral rectus muscle and a heterogeneous mass in the left lobe of the thyroid gland. Excisional biopsies of the thyroid and orbital lesions were consistent with metastatic undifferentiated/anaplastic thyroid carcinoma. This represents the first reported case of undifferentiated/anaplastic thyroid carcinoma metastatic to the orbit.
M
etastatic cancer infrequently involves the orbit and rarely causes dysfunction of the extraocular muscles. When orbital metastases are diagnosed, over 40% of patients *Department of Ophthalmology, University of Washington Eye Institute; †Department of Radiology, University of Washington Medical Center, ‡Department of Pathology, and §Department of Otolaryngology—Head and Neck Surgery, University of Washington Medical Center, Seattle, Washington, U.S.A. Accepted for publication January 7, 2014. This article complied with HIPAA regulations. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Divakar Gupta, M.D., University of Washington Eye Institute, Box 359608, 325 Ninth Avenue, Seattle, WA 98104. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000145
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
FIG. 1. At initial presentation, the patient was unable to abduct the proptotic OD.
do not have a known primary cancer, making metastatic disease an important consideration in the differential diagnosis of patients with orbitopathy.1 The authors present a case of lateral rectus metastasis that preceded the discovery of a primary thyroid cancer. This is the first report in the literature of an undifferentiated/anaplastic thyroid carcinoma metastasizing to the orbit. All patient information and images presented in this case report have been deidentified in accordance to the Health Portability and Accountability Act guidelines.
CASE REPORT A 52-year-old Caucasian woman presented to the emergency room with 5 months of right orbital pain and recent onset of nausea, binocular diplopia, intermittent blurring of her vision,
Case Reports
and dizziness. An outside physician had initiated treatment with oral prednisone for presumed idiopathic orbital inflammation, but her symptoms remained unchanged. Past medical history included endometriosis status post right ovary and fallopian tube resection. Family history included father with throat cancer, a sister with breast cancer, a maternal aunt with brain cancer, another maternal aunt with lymphoma, and a maternal uncle with prostate cancer. The patient reported a 20-pack-year smoking history. On examination, visual acuity was 20/50 OD and 20/20 OS. Pupil examination and color vision testing were normal. Hertel exophthalmometry was significant for 4 mm of right globe proptosis. The patient had 20 prism diopters of esotropia in primary gaze and was unable to abduct her OD past midline (Fig. 1). Slit lamp and dilated fundus examinations were normal bilaterally. During the course of the ophthalmic examination, the physician noted significant erythema and edema over the anterior aspect of the patient’s neck and palpated a thyroid mass. MRI of the brain and orbits revealed a 3.1 × 1.7 × 1.9 cm enhancing mass within the right lateral rectus muscle. CT showed no adjacent bone erosion (Fig. 2). Neck CT scan revealed a 3.8-cm heterogeneous left thyroid mass with peripheral calcifications and 1.3-cm low attenuation right thyroid nodule (Fig. 3). Laboratory workup for infectious, autoimmune, and inflammatory conditions was negative. Biopsy of the right lateral rectus muscle mass revealed a uniform population of mitotically active, markedly pleomorphic undifferentiated cells with prominent nucleoli and granular
FIG. 2. Imaging of lateral rectus lesion. Axial (A–C) and coronal slices (D–F). CT (A and D), MRI T1-weighted images (T1-WI) precontrast (B and E), and postcontrast (C and F) demonstrate an expansile mass in the right lateral rectus muscle, causing medial displacement of the right optic nerve. The mass is isointense to gray matter on the T1 sequence and enhances avidly on the postcontrast T1-WI.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e121
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Case Reports
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
FIG. 3. Imaging of thyroid. Contrast-enhanced coronal (left) and axial (right) CT demonstrates a 3.8-cm well-circumscribed hypodense mass in the left thyroid gland. Axial image demonstrates slightly rightward deviation of the trachea by the expansile mass. There is no local invasion. Also noted on the axial image is a smaller (1.3 cm) hypodense nodule in the anterior aspect of the right thyroid gland.
FIG. 4. Histopathology of orbital biopsy. Hematoxylin and eosin stain at ×400 highlighting poorly differentiated, mitotically active, epithelioid cells with high nuclear to cytoplasm ratio (A). Immunohistochemistry studies showed that this sample was pancytokeratin negative (B), uniformly vimentin positive (C), thyroglobulin negative (D), and thyroid transcription factor-1 negative (E). Gross pathology of the excised specimen (F).
FIG. 5. Histopathology of thyroid and orbital biopsy. Hematoxylin and eosin stain at ×40 (left) and ×400 (middle) of thyroid lesion. Note the similarity of cellular morphology between the thyroid and orbital biopsy (right).
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© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
cytoplasm. Fine-needle aspiration biopsy of the left thyroid mass showed similar neoplastic-appearing cells, raising the possibility of metastatic thyroid carcinoma. Unfortunately, immunohistochemistry stains failed to elucidate a definitive line of differentiation. Systemic evaluation did not reveal any additional suspicious lesions. The patient underwent surgical resection of the right lateral rectus muscle and total thyroidectomy. The orbital tumor had histologic similarities to areas of tumor from the thyroidectomy (Fig. 4 and 5). The final pathologic diagnosis was consistent with dedifferentiation from a well-differentiated thyroid carcinoma into an undifferentiated/anaplastic carcinoma with metastasis to the right orbit. Due to the high-grade nature of the neoplasm and residual carcinoma in the orbit, the patient underwent multisession Gamma knife radiosurgery to the thyroid bed (total dose, 6380 cGy) and right orbit (total dose, 2000 cGy). Her most recent MRI, 18 months after surgery, has not shown any tumor growth or new metastases. As a complication of the orbital resection surgery, the patient suffered a surgical traumatic optic neuropathy. Her postoperative vision was 20/300 OD.
DISCUSSION Orbital lesions most commonly present with ocular motility restriction, proptosis, blepharoptosis, vision loss, and/or a palpable mass.2,3 Orbital metastatic lesions are rare, comprising 10% to 12% of orbital neoplasms and only 1.5% to 3.3% of all orbital lesions.1 Forty-two percent of patients do not have a known primary cancer at the time they are diagnosed with orbital metastatic disease. Carcinomas account for 91% of orbital metastatic disease, with breast, lung, and prostate being the common primary sites, implicated in 61% to 73% of cases. Metastases to the orbit typically occur in the temporal quadrant followed by the superior quadrant.1 Only 9% to 18% of all orbital metastases localize to the rectus muscles.1,4 A review of metastases to extraocular muscles found the medial rectus followed by the lateral rectus to be the most commonly involved, with only 2% of metastases being from thyroid carcinoma.4 Between 1979 and 2012, 31 case reports of thyroid carcinoma metastasizing to the globe (n = 22) and orbital tissue (n = 9) were published.5 Papillary thyroid carcinoma is the most common subtype, and most thyroid cancers are well differentiated.6,7 The most common metastatic sites for thyroid carcinoma are lymph nodes, lung, and bone. More distant metastases are seen in older patients and those with more widespread disease, which increase cancer mortality in these patients.7 Combining the present case with the 9 previously reported orbital metastases from thyroid carcinoma, 4 of the 10 cases have involved the rectus muscles. The present case, however, represents the first report of an undifferentiated/anaplastic thyroid carcinoma metastasizing to the orbit. Typically, when a metastatic lesion is encountered with unknown primary tumor site, immunohistochemistry stains for specific cell markers help to identify the cell of origin. In this patient, immunohistochemistry stains for thyroid markers (thyroglobulin or thyroid transcription factor-1) were nondiagnostic due to the undifferentiated nature of the tumor. In addition to thyroidectomy for the primary cancer, external beam radiation, radioactive iodine ablation, and surgical excision have all been reported as treatments for thyroid carcinoma metastases to the orbit. Due to concerns of impending compressive optic neuropathy, the authors elected
Case Reports
to pursue surgical excision followed by adjuvant radiation. Anaplastic thyroid cancer has a median survival of 5 months and a 1-year survival of 20%.8 This patient remains tumor free at 18 months.
CONCLUSION Though exceedingly rare, orbital metastases can cause significant patient morbidity and confer increased mortality. Nearly half of all orbital metastases are diagnosed in the setting of unknown primary cancer, highlighting the need for a thorough review of systems on initial consultation and, occasionally, a nonophthalmologic physical examination. It is even more unusual that thyroid carcinoma involves orbital tissue, and this is the first report of undifferentiated/anaplastic thyroid carcinoma metastatic to the orbit. The synchronous presentation and diagnosis of orbital metastatic disease and primary thyroid cancer in this case reminds the authors to consider a broad differential diagnosis during the medical workup of patients with orbitopathy.
REFERENCES 1. Goldberg RA, Rootman J, Cline RA. Tumors metastatic to the orbit: a changing picture. Surv Ophthalmol 1990;35:1–24. 2. Shields JA, Shields CL, Brotman HK, et al. Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture. Ophthal Plast Reconstr Surg 2001;17:346–54. 3. Valenzuela AA, Archibald CW, Fleming B, et al. Orbital metastasis: clinical features, management and outcome. Orbit 2009;28:153–9. 4. Lacey B, Chang W, Rootman J. Nonthyroid causes of extraocular muscle disease. Surv Ophthalmol 1999;44:187–213. 5. Besic N, Luznik Z. Choroidal and orbital metastases from thyroid cancer. Thyroid 2013;23:543–51. 6. Rocha Filho FD, Lima GG, Ferreira FV, et al. Orbital metastasis as primary clinical manifestation of thyroid carcinoma–case report and literature review. Arq Bras Endocrinol Metabol 2008;52:1497–500. 7. Song HJ, Xue YL, Qiu ZL, et al. Uncommon metastases from differentiated thyroid carcinoma. Hell J Nucl Med 2012;15:233–40. 8. Smallridge RC, Copland JA. Anaplastic thyroid carcinoma: pathogenesis and emerging therapies. Clin Oncol (R Coll Radiol) 2010;22:486–97.
Spontaneous Resorption of a Penetrating Orbital Bone Fracture Fragment Ashley A. Campbell, M.D.*†, Mary Elizabeth Cunnane, M.D.‡§, Gavin P. Dunn, M.D., Ph.D.║¶, Stacy Tutt Gray, M.D.#**, and Daniel R. Lefebvre, M.D.*† Abstract: The authors describe a 20-year-old man who sustained multiple facial fractures in a high-speed motor vehicle crash, including a bone fragment from a skull base fracture that penetrated the orbital soft tissues superomedially. Serial CT scans documented spontaneous *Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary; †Department of Ophthalmology, Harvard Medical School; ‡Massachusetts Eye and Ear Infirmary; §Department of Radiology/Neuroradiology, Harvard Medical School; ║Department of Neurosurgery, Massachusetts General Hospital; ¶Department of Neurosurgery, Harvard Medical School; #Department of Otolaryngology—Head and Neck Surgery, Massachusetts Eye and Ear Infirmary; and **Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A. Accepted for publication January 8, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ashley A. Campbell, M.D., Mass Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000146
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Case Reports
side effects, but with good efficacy, have been reported for the use of IVIG as a treatment for several autoimmune diseases.6,8 Unfortunately, recurrences after finishing therapy are frequent. The first patient responded well to combined immunoglobulin infusions and steroids. Our second patient highlights the role of conservative treatment where surgical intervention can have significant risks.
REFERENCES FIG. 1. Periocular eyelid swelling and thickening of the skin in the first patient.
FIG. 2. Thickening of the upper eyelid skin and lateral lower eyelid ectropion in the second patient.
gastrointestinal dysmotility, muscle weakness, joint contractures, and neurologic symptoms (such as seizures, encephalopathy, and coma), and obstructive or restrictive pulmonary disease. Ophthalmic manifestations include corneal opacities, thickened eyebrow or eyelid skin, lagophthalmos, ectropion, choroidal folds, and papilledema.4 In their case series, Davis et al.4 reported 4 patients who presented with thickened eyebrow or eyelid skin. In the same case series, 2 patients had a lower eyelid ectropion similar to our second patient, and 1 patient was found to have lagophthalmos.4 The diagnosis requires the histologic demonstration of dermal mucin with increased collagen deposition and fibroblast proliferation and the presence of a serum monoclonal gammopathy.2 Histopathological analysis of skin samples stained with hematoxylin, eosin, and colloidal iron can demonstrate dermal mucin. The epidermis is spared, and there is a proliferation of predominantly spindle and epithelioid cells with a fibromyxoid stroma containing thick bundles of collagen and increased mucin. The degree of cellularity ranges from scattered cells in the dermis to markedly hypercellular infiltrates involving the entire reticular dermis. Immunohistochemical studies can detect expression of factor XIIIa (a marker of dermal dendrocytes) and procollagen 1 (a precursor of collagen 1). The expression of factor XIIIa can be seen in up to 50% of cells in the dermis, consistent with a proliferation of dermal dendrocytes. Procollagen 1 is expressed in the basement membrane area.1 There is no established standard therapy for the systemic treatment of scleromyxedema and the prognosis is guarded.5 However, several treatment modalities including steroids, melphalan, cyclophosphamide, interferon alpha, and plasmapheresis have been suggested.2,5,6 Steroids alone are ineffective.5 The low-dose melphalan and steroids are a popular treatment regimen with mixed results and concerns about the risks of infection and secondary malignancy.7 Donato et al.2 present their observations of high-dose melphalan treatment with autologous stem cell transplantation, which has shown to be extremely successful in resolution of the cutaneous lesions and complete remission of all gastrointestinal, pulmonary, and even neurologic manifestations. Rongioletti et al.5 present their experience in treatment with IVIGs (alone or in combination with other drugs), which have induced complete remission in some of their patients with a mean treatment duration of 2 years. Only a few
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1. Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273–81. 2. Donato ML, Feasel AM, Weber DM, et al. Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation. Blood 2006;107:463–6. 3. Cokonis Georgakis CD, Falasca G, Georgakis A, et al. Scleromyxedema. Clin Dermatol 2006;24:493–7. 4. Davis ML, Bartley GB, Gibson LE, et al. Ophthalmic findings in scleromyxedema. Ophthalmology 1994;101:252–5. 5. Rongioletti F, Merlo G, Cinotti E, et al. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol 2013;69:66–72. 6. Bidier M, Zschoche C, Gholam P, et al. Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol 2012;92:408–9. 7. Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol 1995;33:37–43. 8. Enk A; European Dermatology Forum Guideline Subcommittee. Guidelines on the use of high-dose intravenous immunoglobulin in dermatology. Eur J Dermatol 2009;19:90–8.
Orbital Metastasis of Undifferentiated/Anaplastic Thyroid Carcinoma Divakar Gupta, M.D.*, Michael Chappell, M.D.*, Tina D. Tailor, M.D.†, C. Dirk Keene, M.D., Ph.D.*‡, Kris Moe, M.D.§, Arash Jian-Amadi, M.D.*, and Shu-hong Chang, M.D.* Abstract: A 52-year-old woman presented with decreased vision, diplopia, esotropia, proptosis, and right orbital pain. Clinical examination was suspicious for an orbital mass and additionally revealed a thyroid gland mass. Imaging studies showed an enhancing mass within the right lateral rectus muscle and a heterogeneous mass in the left lobe of the thyroid gland. Excisional biopsies of the thyroid and orbital lesions were consistent with metastatic undifferentiated/anaplastic thyroid carcinoma. This represents the first reported case of undifferentiated/anaplastic thyroid carcinoma metastatic to the orbit.
M
etastatic cancer infrequently involves the orbit and rarely causes dysfunction of the extraocular muscles. When orbital metastases are diagnosed, over 40% of patients *Department of Ophthalmology, University of Washington Eye Institute; †Department of Radiology, University of Washington Medical Center, ‡Department of Pathology, and §Department of Otolaryngology—Head and Neck Surgery, University of Washington Medical Center, Seattle, Washington, U.S.A. Accepted for publication January 7, 2014. This article complied with HIPAA regulations. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Divakar Gupta, M.D., University of Washington Eye Institute, Box 359608, 325 Ninth Avenue, Seattle, WA 98104. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000145
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
FIG. 1. At initial presentation, the patient was unable to abduct the proptotic OD.
do not have a known primary cancer, making metastatic disease an important consideration in the differential diagnosis of patients with orbitopathy.1 The authors present a case of lateral rectus metastasis that preceded the discovery of a primary thyroid cancer. This is the first report in the literature of an undifferentiated/anaplastic thyroid carcinoma metastasizing to the orbit. All patient information and images presented in this case report have been deidentified in accordance to the Health Portability and Accountability Act guidelines.
CASE REPORT A 52-year-old Caucasian woman presented to the emergency room with 5 months of right orbital pain and recent onset of nausea, binocular diplopia, intermittent blurring of her vision,
Case Reports
and dizziness. An outside physician had initiated treatment with oral prednisone for presumed idiopathic orbital inflammation, but her symptoms remained unchanged. Past medical history included endometriosis status post right ovary and fallopian tube resection. Family history included father with throat cancer, a sister with breast cancer, a maternal aunt with brain cancer, another maternal aunt with lymphoma, and a maternal uncle with prostate cancer. The patient reported a 20-pack-year smoking history. On examination, visual acuity was 20/50 OD and 20/20 OS. Pupil examination and color vision testing were normal. Hertel exophthalmometry was significant for 4 mm of right globe proptosis. The patient had 20 prism diopters of esotropia in primary gaze and was unable to abduct her OD past midline (Fig. 1). Slit lamp and dilated fundus examinations were normal bilaterally. During the course of the ophthalmic examination, the physician noted significant erythema and edema over the anterior aspect of the patient’s neck and palpated a thyroid mass. MRI of the brain and orbits revealed a 3.1 × 1.7 × 1.9 cm enhancing mass within the right lateral rectus muscle. CT showed no adjacent bone erosion (Fig. 2). Neck CT scan revealed a 3.8-cm heterogeneous left thyroid mass with peripheral calcifications and 1.3-cm low attenuation right thyroid nodule (Fig. 3). Laboratory workup for infectious, autoimmune, and inflammatory conditions was negative. Biopsy of the right lateral rectus muscle mass revealed a uniform population of mitotically active, markedly pleomorphic undifferentiated cells with prominent nucleoli and granular
FIG. 2. Imaging of lateral rectus lesion. Axial (A–C) and coronal slices (D–F). CT (A and D), MRI T1-weighted images (T1-WI) precontrast (B and E), and postcontrast (C and F) demonstrate an expansile mass in the right lateral rectus muscle, causing medial displacement of the right optic nerve. The mass is isointense to gray matter on the T1 sequence and enhances avidly on the postcontrast T1-WI.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e121
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Case Reports
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
FIG. 3. Imaging of thyroid. Contrast-enhanced coronal (left) and axial (right) CT demonstrates a 3.8-cm well-circumscribed hypodense mass in the left thyroid gland. Axial image demonstrates slightly rightward deviation of the trachea by the expansile mass. There is no local invasion. Also noted on the axial image is a smaller (1.3 cm) hypodense nodule in the anterior aspect of the right thyroid gland.
FIG. 4. Histopathology of orbital biopsy. Hematoxylin and eosin stain at ×400 highlighting poorly differentiated, mitotically active, epithelioid cells with high nuclear to cytoplasm ratio (A). Immunohistochemistry studies showed that this sample was pancytokeratin negative (B), uniformly vimentin positive (C), thyroglobulin negative (D), and thyroid transcription factor-1 negative (E). Gross pathology of the excised specimen (F).
FIG. 5. Histopathology of thyroid and orbital biopsy. Hematoxylin and eosin stain at ×40 (left) and ×400 (middle) of thyroid lesion. Note the similarity of cellular morphology between the thyroid and orbital biopsy (right).
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© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015
cytoplasm. Fine-needle aspiration biopsy of the left thyroid mass showed similar neoplastic-appearing cells, raising the possibility of metastatic thyroid carcinoma. Unfortunately, immunohistochemistry stains failed to elucidate a definitive line of differentiation. Systemic evaluation did not reveal any additional suspicious lesions. The patient underwent surgical resection of the right lateral rectus muscle and total thyroidectomy. The orbital tumor had histologic similarities to areas of tumor from the thyroidectomy (Fig. 4 and 5). The final pathologic diagnosis was consistent with dedifferentiation from a well-differentiated thyroid carcinoma into an undifferentiated/anaplastic carcinoma with metastasis to the right orbit. Due to the high-grade nature of the neoplasm and residual carcinoma in the orbit, the patient underwent multisession Gamma knife radiosurgery to the thyroid bed (total dose, 6380 cGy) and right orbit (total dose, 2000 cGy). Her most recent MRI, 18 months after surgery, has not shown any tumor growth or new metastases. As a complication of the orbital resection surgery, the patient suffered a surgical traumatic optic neuropathy. Her postoperative vision was 20/300 OD.
DISCUSSION Orbital lesions most commonly present with ocular motility restriction, proptosis, blepharoptosis, vision loss, and/or a palpable mass.2,3 Orbital metastatic lesions are rare, comprising 10% to 12% of orbital neoplasms and only 1.5% to 3.3% of all orbital lesions.1 Forty-two percent of patients do not have a known primary cancer at the time they are diagnosed with orbital metastatic disease. Carcinomas account for 91% of orbital metastatic disease, with breast, lung, and prostate being the common primary sites, implicated in 61% to 73% of cases. Metastases to the orbit typically occur in the temporal quadrant followed by the superior quadrant.1 Only 9% to 18% of all orbital metastases localize to the rectus muscles.1,4 A review of metastases to extraocular muscles found the medial rectus followed by the lateral rectus to be the most commonly involved, with only 2% of metastases being from thyroid carcinoma.4 Between 1979 and 2012, 31 case reports of thyroid carcinoma metastasizing to the globe (n = 22) and orbital tissue (n = 9) were published.5 Papillary thyroid carcinoma is the most common subtype, and most thyroid cancers are well differentiated.6,7 The most common metastatic sites for thyroid carcinoma are lymph nodes, lung, and bone. More distant metastases are seen in older patients and those with more widespread disease, which increase cancer mortality in these patients.7 Combining the present case with the 9 previously reported orbital metastases from thyroid carcinoma, 4 of the 10 cases have involved the rectus muscles. The present case, however, represents the first report of an undifferentiated/anaplastic thyroid carcinoma metastasizing to the orbit. Typically, when a metastatic lesion is encountered with unknown primary tumor site, immunohistochemistry stains for specific cell markers help to identify the cell of origin. In this patient, immunohistochemistry stains for thyroid markers (thyroglobulin or thyroid transcription factor-1) were nondiagnostic due to the undifferentiated nature of the tumor. In addition to thyroidectomy for the primary cancer, external beam radiation, radioactive iodine ablation, and surgical excision have all been reported as treatments for thyroid carcinoma metastases to the orbit. Due to concerns of impending compressive optic neuropathy, the authors elected
Case Reports
to pursue surgical excision followed by adjuvant radiation. Anaplastic thyroid cancer has a median survival of 5 months and a 1-year survival of 20%.8 This patient remains tumor free at 18 months.
CONCLUSION Though exceedingly rare, orbital metastases can cause significant patient morbidity and confer increased mortality. Nearly half of all orbital metastases are diagnosed in the setting of unknown primary cancer, highlighting the need for a thorough review of systems on initial consultation and, occasionally, a nonophthalmologic physical examination. It is even more unusual that thyroid carcinoma involves orbital tissue, and this is the first report of undifferentiated/anaplastic thyroid carcinoma metastatic to the orbit. The synchronous presentation and diagnosis of orbital metastatic disease and primary thyroid cancer in this case reminds the authors to consider a broad differential diagnosis during the medical workup of patients with orbitopathy.
REFERENCES 1. Goldberg RA, Rootman J, Cline RA. Tumors metastatic to the orbit: a changing picture. Surv Ophthalmol 1990;35:1–24. 2. Shields JA, Shields CL, Brotman HK, et al. Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture. Ophthal Plast Reconstr Surg 2001;17:346–54. 3. Valenzuela AA, Archibald CW, Fleming B, et al. Orbital metastasis: clinical features, management and outcome. Orbit 2009;28:153–9. 4. Lacey B, Chang W, Rootman J. Nonthyroid causes of extraocular muscle disease. Surv Ophthalmol 1999;44:187–213. 5. Besic N, Luznik Z. Choroidal and orbital metastases from thyroid cancer. Thyroid 2013;23:543–51. 6. Rocha Filho FD, Lima GG, Ferreira FV, et al. Orbital metastasis as primary clinical manifestation of thyroid carcinoma–case report and literature review. Arq Bras Endocrinol Metabol 2008;52:1497–500. 7. Song HJ, Xue YL, Qiu ZL, et al. Uncommon metastases from differentiated thyroid carcinoma. Hell J Nucl Med 2012;15:233–40. 8. Smallridge RC, Copland JA. Anaplastic thyroid carcinoma: pathogenesis and emerging therapies. Clin Oncol (R Coll Radiol) 2010;22:486–97.
Spontaneous Resorption of a Penetrating Orbital Bone Fracture Fragment Ashley A. Campbell, M.D.*†, Mary Elizabeth Cunnane, M.D.‡§, Gavin P. Dunn, M.D., Ph.D.║¶, Stacy Tutt Gray, M.D.#**, and Daniel R. Lefebvre, M.D.*† Abstract: The authors describe a 20-year-old man who sustained multiple facial fractures in a high-speed motor vehicle crash, including a bone fragment from a skull base fracture that penetrated the orbital soft tissues superomedially. Serial CT scans documented spontaneous *Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary; †Department of Ophthalmology, Harvard Medical School; ‡Massachusetts Eye and Ear Infirmary; §Department of Radiology/Neuroradiology, Harvard Medical School; ║Department of Neurosurgery, Massachusetts General Hospital; ¶Department of Neurosurgery, Harvard Medical School; #Department of Otolaryngology—Head and Neck Surgery, Massachusetts Eye and Ear Infirmary; and **Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A. Accepted for publication January 8, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ashley A. Campbell, M.D., Mass Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000146
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