Ophthal Plast Reconstr Surg, Vol. 31, No. 1, 2015
Case Reports
15. Morgenstern KE, Vadysirisack DD, Zhang Z, et al. Expression of sodium iodide symporter in the lacrimal drainage system: implication for the mechanism underlying nasolacrimal duct obstruction in I (131)-treated patients. Ophthal Plast Reconstr Surg 2005;21:337–44. 16. Brockmann H, Wilhelm K, Joe A, et al. Nasolacrimal drainage obstruction after radioiodine therapy: case report and a review of the literature. Clin Nucl Med 2005;30:543–5. 17. Kloos RT, Duvuuri V, Jhiang SM, et al. Nasolacrimal drainage system obstruction from radioactive iodine therapy for thyroid carcinoma. J Clin Endocrinol Metab 2002;87:5817–20. 18. Shepler TR, Sherman SI, Faustina MM, et al. Nasolacrimal duct obstruction associated with radioactive iodine therapy for thyroid carcinoma. Ophthal Plast Reconstr Surg 2003;19:479–81. 19. Salour H, Montazerin N. Incision site tissue necrosis after dacryocystorhinostomy. Ophthal Plast Reconstr Surg 1998;14:146–8.
Inflammatory Myofibroblastic Tumor of the Orbit: A Case Report Stephanie K. Cramer, M.D.*, Alison Skalet, M.D., Ph.D.*, Atiya Mansoor, M.D.*, David J. Wilson, M.D.†, and John D. Ng, M.D.* Abstract: Inflammatory myofibroblastic tumor (IMT) is a neoplasm most commonly found in the abdominal-pelvic region, lung, and retroperitoneum. The tumor tends to affect soft tissues of children and young adults and can locally recur but rarely metastasizes.1 Histologically, the appearance is one of bland spindle cell proliferation with a prominent, chronic inflammatory infiltrate. This article describes 1 case of IMT found in the orbit that is presented with rapidly progressive painless proptosis. In the authors’ review of the literature, they have only found 2 other case reports involving the orbit.
CASE REPORT The study protocol was exempted by the Oregon Health and Science University Institutional Review Board. The study was conducted in accordance with the provisions of the Declaration of Helsinki and was performed in compliance with the Health Insurance Portability and Accountability Act. A 21-year-old man was referred to the oculoplastics service for evaluation and treatment of a rapidly enlarging right orbit mass. The patient reported noticing a small red bump on his lower eyelid 2 months before presentation. There was no pain associated with the mass, but the patient felt pressure behind the OD. He noted that the mass caused local irritation and diplopia on wakening or with high levels of activity. He had no history of trauma. The patient had been feeling weak for the last year but had no unintentional weight loss. On examination, his best corrected visual acuity (VA) was 20/20 in the OD and 20/20 − 1 in the OS. A fleshy, soft, tan, movable mass was found in the inferior orbit underneath the conjunctiva. Right hyperglobus of 3 mm was noted. Extraocular movements were intact, but he reported diplopia in down and Accepted for publication September 7, 2013. *American Board of Ophthalmology and †Pathology, Oregon Health & Science University, Portland, Oregan, U.S.A. Financial support was received in preparation of this manuscript. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Stephanie K. Cramer, M.D., Ophthalmology, Oregon Health & Science University, 3375 SW Terwilliger Blvd., Portland, OR 97239. E-mail: [email protected]
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left gaze. Hertel measurements were 18/16 (OD/OS) with a base of 95. There was mild resistance to retropulsion in the OD. The rest of his ocular examination was normal. The patient underwent an MRI scan at an outside hospital that showed a well-circumscribed mass in the anterior inferonasal right orbit with globe deformation and induced displacement of the OD. See Figure, A,B. The patient was taken to the operating room for right anterior orbitotomy and mass removal. The mass was found to be solid and dense. A portion of the mass was sent for flow cytometry, and no monoclonal B cell population was seen. Pathologic study showed a low-grade neoplasm, composed of short compact bundles of plump spindled cells. The nuclei show open chromation with small nucleoli. There was an accompanying mild infiltrate of lymphocytes and plasma cells. Multinucleated histiocytic cells were also seen. See Figure, C,D. Immunohistochemically, the tumor was diffusely positive for cytoplasmic ALK (anaplastic lymphoma kinase). CD68 and Factor 13a highlight the histiocytes. It was negative for S100, HMB45, Melan A, pancytokeratin, CD45, bcl 2, CD34, CD99, CD30, EMA, PR, TLE1, Desmin, SMA, myogenin, CD21, CD35, Cytokeratin 7, cytokeratin 20, and pancytokeratin. Ki-67 stained 10% positive. In addition, FISH analysis was positive for an ALK rearrangement. These features supported a diagnosis of low-grade inflammatory myofibroblastic tumor of the orbit. This case report was prepared in accordance with Helsinki and Health Insurance Portability and Accountability Act regulations.
DISCUSSION Inflammatory myofibroblastic tumors (IMT) are most commonly found in children and adolescents. In general, the most common locations for them to occur are the abdomen, pelvis, lung, and retroperitoneum.2 Patients usually present with a progressively enlarging mass, and only 15% to 30% of patients will have constitutional symptoms.3 There have been case reports of the tumor at many other sites including the central nervous system and 1 other case report of primary IMT in the orbit. The prior case report of primary orbital IMT describes a subconjuctival mass found in a 10-year-old boy who presented
A, Clinical photograph of right orbital mass. B, MRI image showing prominent mass inferior nasally. C and D, Hematoxylineosin Stain: tumor comprised short compact bundles of plump spindled cells with small nucleoli. There is a mild lymphocytic infiltrate; multinucleated histiocytes are also seen.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 31, No. 1, 2015
with diplopia and limited ocular motility.4 Another case report describes IMT arising simultaneously in the lung and orbit in a 78-year-old man who presented clinically with limited extraocular movements.5 Histologically, IMT appears as spindle cells in a background of mixed inflammation. The spindle cells in IMT are bland and uniform. Rarely, necrosis and calcification can be found. The spindle cells are ultimately myofibroblasts that are positive for smooth muscle actin in 80% to 90 % of cases.6,7 They can also express desmin and calponin in 60% to 70% of cases. The mixed inflammation is predominantly lymphocytic and plasma cell dominated but polyclonal.6,8 Histologically, tumor size, cellularity, mitotic activity, and necrosis do not correlate with the patient’s clinical outcome.9,10 Molecularly, the ALK gene is expressed in 50% of IMTs; its expression in IMT can predict the existence of an IMT gene rearrangement.10 This patient showed positive expressivity of ALK; his FISH testing for the ALK rearrangement was positive. Although not conclusive, it is thought that IMT tumors harboring the ALK gene rearrangement could be of more malignant potential.11 IMT was considered to be a benign tumor until the early 1990s when a large case series was published by Meis et al.3 showed 38 cases—primarily intra-abdominal and retroperitoneal—in which 37% of cases had local recurrences, 11% developed metastasis, and 19% of patients died from the disease. Today, the World Health Organization characterizes IMT as a tumor of intermediate biological potential.1,4,12,13 The recurrence rate for intrapulmonary lesions
Case Reports
15. Morgenstern KE, Vadysirisack DD, Zhang Z, et al. Expression of sodium iodide symporter in the lacrimal drainage system: implication for the mechanism underlying nasolacrimal duct obstruction in I (131)-treated patients. Ophthal Plast Reconstr Surg 2005;21:337–44. 16. Brockmann H, Wilhelm K, Joe A, et al. Nasolacrimal drainage obstruction after radioiodine therapy: case report and a review of the literature. Clin Nucl Med 2005;30:543–5. 17. Kloos RT, Duvuuri V, Jhiang SM, et al. Nasolacrimal drainage system obstruction from radioactive iodine therapy for thyroid carcinoma. J Clin Endocrinol Metab 2002;87:5817–20. 18. Shepler TR, Sherman SI, Faustina MM, et al. Nasolacrimal duct obstruction associated with radioactive iodine therapy for thyroid carcinoma. Ophthal Plast Reconstr Surg 2003;19:479–81. 19. Salour H, Montazerin N. Incision site tissue necrosis after dacryocystorhinostomy. Ophthal Plast Reconstr Surg 1998;14:146–8.
Inflammatory Myofibroblastic Tumor of the Orbit: A Case Report Stephanie K. Cramer, M.D.*, Alison Skalet, M.D., Ph.D.*, Atiya Mansoor, M.D.*, David J. Wilson, M.D.†, and John D. Ng, M.D.* Abstract: Inflammatory myofibroblastic tumor (IMT) is a neoplasm most commonly found in the abdominal-pelvic region, lung, and retroperitoneum. The tumor tends to affect soft tissues of children and young adults and can locally recur but rarely metastasizes.1 Histologically, the appearance is one of bland spindle cell proliferation with a prominent, chronic inflammatory infiltrate. This article describes 1 case of IMT found in the orbit that is presented with rapidly progressive painless proptosis. In the authors’ review of the literature, they have only found 2 other case reports involving the orbit.
CASE REPORT The study protocol was exempted by the Oregon Health and Science University Institutional Review Board. The study was conducted in accordance with the provisions of the Declaration of Helsinki and was performed in compliance with the Health Insurance Portability and Accountability Act. A 21-year-old man was referred to the oculoplastics service for evaluation and treatment of a rapidly enlarging right orbit mass. The patient reported noticing a small red bump on his lower eyelid 2 months before presentation. There was no pain associated with the mass, but the patient felt pressure behind the OD. He noted that the mass caused local irritation and diplopia on wakening or with high levels of activity. He had no history of trauma. The patient had been feeling weak for the last year but had no unintentional weight loss. On examination, his best corrected visual acuity (VA) was 20/20 in the OD and 20/20 − 1 in the OS. A fleshy, soft, tan, movable mass was found in the inferior orbit underneath the conjunctiva. Right hyperglobus of 3 mm was noted. Extraocular movements were intact, but he reported diplopia in down and Accepted for publication September 7, 2013. *American Board of Ophthalmology and †Pathology, Oregon Health & Science University, Portland, Oregan, U.S.A. Financial support was received in preparation of this manuscript. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Stephanie K. Cramer, M.D., Ophthalmology, Oregon Health & Science University, 3375 SW Terwilliger Blvd., Portland, OR 97239. E-mail: [email protected]
e22
left gaze. Hertel measurements were 18/16 (OD/OS) with a base of 95. There was mild resistance to retropulsion in the OD. The rest of his ocular examination was normal. The patient underwent an MRI scan at an outside hospital that showed a well-circumscribed mass in the anterior inferonasal right orbit with globe deformation and induced displacement of the OD. See Figure, A,B. The patient was taken to the operating room for right anterior orbitotomy and mass removal. The mass was found to be solid and dense. A portion of the mass was sent for flow cytometry, and no monoclonal B cell population was seen. Pathologic study showed a low-grade neoplasm, composed of short compact bundles of plump spindled cells. The nuclei show open chromation with small nucleoli. There was an accompanying mild infiltrate of lymphocytes and plasma cells. Multinucleated histiocytic cells were also seen. See Figure, C,D. Immunohistochemically, the tumor was diffusely positive for cytoplasmic ALK (anaplastic lymphoma kinase). CD68 and Factor 13a highlight the histiocytes. It was negative for S100, HMB45, Melan A, pancytokeratin, CD45, bcl 2, CD34, CD99, CD30, EMA, PR, TLE1, Desmin, SMA, myogenin, CD21, CD35, Cytokeratin 7, cytokeratin 20, and pancytokeratin. Ki-67 stained 10% positive. In addition, FISH analysis was positive for an ALK rearrangement. These features supported a diagnosis of low-grade inflammatory myofibroblastic tumor of the orbit. This case report was prepared in accordance with Helsinki and Health Insurance Portability and Accountability Act regulations.
DISCUSSION Inflammatory myofibroblastic tumors (IMT) are most commonly found in children and adolescents. In general, the most common locations for them to occur are the abdomen, pelvis, lung, and retroperitoneum.2 Patients usually present with a progressively enlarging mass, and only 15% to 30% of patients will have constitutional symptoms.3 There have been case reports of the tumor at many other sites including the central nervous system and 1 other case report of primary IMT in the orbit. The prior case report of primary orbital IMT describes a subconjuctival mass found in a 10-year-old boy who presented
A, Clinical photograph of right orbital mass. B, MRI image showing prominent mass inferior nasally. C and D, Hematoxylineosin Stain: tumor comprised short compact bundles of plump spindled cells with small nucleoli. There is a mild lymphocytic infiltrate; multinucleated histiocytes are also seen.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 31, No. 1, 2015
with diplopia and limited ocular motility.4 Another case report describes IMT arising simultaneously in the lung and orbit in a 78-year-old man who presented clinically with limited extraocular movements.5 Histologically, IMT appears as spindle cells in a background of mixed inflammation. The spindle cells in IMT are bland and uniform. Rarely, necrosis and calcification can be found. The spindle cells are ultimately myofibroblasts that are positive for smooth muscle actin in 80% to 90 % of cases.6,7 They can also express desmin and calponin in 60% to 70% of cases. The mixed inflammation is predominantly lymphocytic and plasma cell dominated but polyclonal.6,8 Histologically, tumor size, cellularity, mitotic activity, and necrosis do not correlate with the patient’s clinical outcome.9,10 Molecularly, the ALK gene is expressed in 50% of IMTs; its expression in IMT can predict the existence of an IMT gene rearrangement.10 This patient showed positive expressivity of ALK; his FISH testing for the ALK rearrangement was positive. Although not conclusive, it is thought that IMT tumors harboring the ALK gene rearrangement could be of more malignant potential.11 IMT was considered to be a benign tumor until the early 1990s when a large case series was published by Meis et al.3 showed 38 cases—primarily intra-abdominal and retroperitoneal—in which 37% of cases had local recurrences, 11% developed metastasis, and 19% of patients died from the disease. Today, the World Health Organization characterizes IMT as a tumor of intermediate biological potential.1,4,12,13 The recurrence rate for intrapulmonary lesions
