Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
procedure. This leak was successfully treated with repair of the conjunctiva and fornix defect using amniotic membrane and placement of a temporary tarsorrhaphy.
REFERENCES 1. Van Haeringen JH. Clinical biochemistry of tears. In: Milder B, Weil B, eds. The Lacrimal System. Norwalk, CT: Appleton-Century Crofts, 1983:23–48. 2. Gasset AR, Braverman LE, Fleming MC, et al. Tear glucose detection of hyperglycemia. Am J Ophthalmol 1968;65:414–20. 3. Neuhaus RW, Baylis HI. Cerebrospinal fluid leakage after dacryocystorhinostomy. Ophthalmology 1983;90:1091–5. 4. Bagheri A, Naghibozakerin J, Yazdani S. Cerebrospinal fluid leakage during dacryocystorhinostomy in a patient with meningoencephalocele. Eur J Ophthalmol 2005;15:500–3. 5. Badilla J, Dolman PJ. Cerebrospinal fluid leaks complicating orbital or oculoplastic surgery. Arch Ophthalmol 2007;125:1631–4. 6. Fayet B, Racy E, Assouline M. Cerebrospinal fluid leakage after endonasal dacryocystorhinostomy. J Fr Ophtalmol 2007;30:129–34. 7. Wulc AE, Adams JL, Dryden RM. Cerebrospinal fluid leakage complicating orbital exenteration. Arch Ophthalmol 1989;107:827–30. 8. Schick B, Weber R, Mosler P, et al. [Long-term follow-up of frontobasal dura-plasty]. HNO 1997;45:117–22. 9. Lindstrom DR, Toohill RJ, Loehrl TA, et al. Management of cerebrospinal fluid rhinorrhea: the Medical College of Wisconsin experience. Laryngoscope 2004;114:969–74. 10. Joshi KK, Crockard HA. Traumatic cerebrospinal fluid fistula simulating tears. Case report. J Neurosurg 1978;49:121–3. 11. Dryden RM, Wulc AE. Pseudoepiphora from cerebrospinal fluid leak: case report. Br J Ophthalmol 1986;70:570–4. 12. Korinek AM, Baugnon T, Golmard JL, et al. Risk factors for adult nosocomial meningitis after craniotomy: role of antibiotic prophylaxis. Neurosurgery 2006;59:126–33; discussion 126–33.
Spindle Cell Lipoma of the Conjunctiva Fariha Shafi, F.R.C.Ophth.*, Bipin Gonglore, MS, FRCS*, Soupramanien Sandramouli, FRCS, MD*, and Hardeep S. Mudhar, Ph.D., F.R.C.Path.† Abstract: A 67-year-old woman presented with progressive enlargement of a long-standing mass on the surface of her OS associated with ocular surface irritation. The mass was excised en bloc. Histopathological examination showed a well-defined encapsulated tumor composed of wiry collagen containing bland spindle cells that were strongly positive for CD34 with scattered mature adipocytes. These features confirmed a diagnosis of spindle cell lipoma.
L
ipomas are the most common mesenchymal neoplasm. They most commonly develop on the trunk and extremities. Interestingly, lipomas have only rarely been reported in ocular tissues and many of these cases involve the orbit. Clinically, conjunctival lipomas must be differentiated from herniated orbital fat, dermoids, and the lacrimal gland.1 *New Cross Hospital, Wolverhampton, West Midlands; and †National Specialist Ophthalmic Pathology Service, Department of Histopathology, Royal Hallamshire Hospital, Sheffield, England, United Kingdom Accepted for publication February 14, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Fariha Shafi, F.R.C.Ophth., New Cross Hospital, Wolverhampton Road, Wolverhampton, WV10 0QP England, United Kingdom. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000189
e28
Spindle cell lipoma is a distinct variant of lipoma in which mature fat is replaced by collagen-forming spindle cells.2,3 A patient who developed spindle cell lipoma of the nasal bulbar conjunctiva with no orbital involvement is reported. To the authors knowledge, this is the first reported case of conjunctival spindle cell lipoma.
CASE REPORT A 67-year-old Caucasian woman was referred to the oculoplastic service with a swelling on the surface of her OS, which had been present for approximately 1 year but in recent months had increased slightly in size. The mass was associated with ocular surface irritation, and there was no history of associated pain, bleeding, or discharge. Her medical history included Crohn disease and a recent diagnosis of rectal carcinoma. On examination, her visual acuity was 20/40 OD and 20/40 OS. A soft mobile subconjunctival mass was present on the nasal conjunctiva measuring 8 mm in diameter (Fig. 1). There was no associated overlying inflammation or discoloration of the conjunctiva. No proptosis or resistance to retropulsion was noted, and extraocular movements were full. Pupillary reactions were normal, and dilated examination of the fundus was unremarkable. An excision biopsy of the lesion was performed under local anesthetic. Intraoperatively, the mass was noted to be a discrete smooth surfaced white nodule, which was confined to the bulbar conjunctiva and did not involve the orbit. The mass was removed en bloc after a careful dissection from the underlying sclera and sent for histopathological examination. Gross examination revealed a well-defined, smooth surfaced white nodule measuring 6 × 4 × 3 mm, the cut surface of which displayed a solid white appearance. Microscopy revealed a benign soft tissue lesion composed mostly of wiry, ropey collagen (Fig. 2A). Scattered among the collagen were groups of mature adipocytes (Fig. 2B), bland stromal spindle cells (Fig. 2C), and occasional mast cells (Fig. 2D). The spindle cells were strongly positive for CD34 (Fig. 2E) and negative for S100, melan-A, and smooth muscle actin (not shown). There were no neurofilament-positive axons among the collagen fibers. There were no floret-type cells or lipoblasts, and no mitotic activity was identified. The features were with those of a typical spindle cell lipoma. There was no evidence of metastatic carcinoma (Fig. 2).
DISCUSSION A recent clinical survey of 1,643 conjunctival tumors by Shields et al.4 revealed that the most common diagnoses were nevus (28%), melanoma (13%), primary acquired melanosis (11%), squamous cell carcinoma (11%), and lymphoid tumor (8%). Lipomatous tumors were only found in 23 patients (1%), and of these, only 3 patients were diagnosed with lipoma (the remaining 20 patients had the diagnosis of herniated orbital fat). Spindle cell lipoma is a distinct variant of lipoma in which spindle cells replace normal fat and was first described by Enzinger and Harvey2 in 1975. It is found more frequently in men aged 50 to 70 years (mean age 56 years) and is usually located in the subcutaneous tissue of the shoulder and posterior neck. Histologically, spindle cell lipomas are wellcircumscribed, rarely encapsulated masses consisting of mature adipocytes that are separated and partly replaced by groups of small, slender spindle cells. These cells show uniformity and have a single elongated nucleus and narrow, bipolar cytoplasmic processes.2 They lack atypia and possess little or no pleomorphism and have a varying quantity of dense birefringent collagen and mast cells. Floret cells are not found, but areas of vascularization may be present within the lesion.5 There are only 3 reported cases of spindle cell lipoma in the orbit.3,5,6
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 1. A, Patient with left medial conjunctival lesion visualized on left lateral gaze. B, Detailed view of conjunctival spindle cell lipoma.
The histologic differential diagnosis of spindle cell lipoma includes both benign and atypical adipocytic lesions. Benign pleomorphic lipomas have a cardinal histologic feature of scattered multinucleate giant cells with a floret-like arrangement of multiple hyperchromatic nuclei in eosinophilic cytoplasm.7 This is important as pleomorphic lipoma is now thought to lie at the opposite end of a common histologic spectrum with spindle cell lipoma, as both spindle cell lipoma and pleomorphic lipoma share identical cytogenetic chromosomal alterations.8 While in some cases it is possible to see transitions of spindle cell lipoma with pleomorphic lipoma in the same specimen, in this study this was not the case. Another important differential is subconjunctival prolapsed orbital fat, a degenerative process, composed of mature adipocytes, floret-like cells, and fibrous septa.9 However, this process is confined to the superotemporal subconjunctival area; the lesion in the case of this study was discrete and on the medial bulbar conjunctiva. However, wiry/ropy collagen and collections of bland spindle cells are absent in prolapsed orbital fat. Atypical lipomatous tumor also enters the histologic differential diagnosis. However, this contains lobules of adipocytes varying in size with fibrous septa containing atypical hyperchromatic spindle and multinucleate cells unlike the appearance of spindle cell lipoma. An important nonlipomatous soft tissue tumor that merits consideration is neurofibroma. The collagen however is coarser (said to resemble shredded carrots), many of the spindle cells are S100 positive, and there are neurofilament-positive axons coursing through it.10 None of these features were seen in this case. This case of spindle cell lipoma that the authors present is unique because the tumor involved the medial bulbar
conjunctiva without any orbital involvement, unlike orbital fat prolapse that involves the superotemporal conjunctiva. Although spindle cell lipoma has previously been shown to occur in the orbit,3,5,6 this is the first case to show purely conjunctival involvement. The diagnosis of spindle cell lipoma should therefore be considered when assessing nodular conjunctival lesions.
REFERENCES 1. Jordan DR, Tse DT. Herniated orbital fat. Can J Ophthalmol 1987;22:173–7. 2. Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer 1975;36:1852–9. 3. Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol 1985;100:605–9. 4. Shields CL, Demirci H, Karatza E, et al. Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;111:1747–54. 5. Johnson BL, Linn JG Jr. Spindle cell lipoma of the orbit. Arch Ophthalmol 1979;97:133–4. 6. Mawn LA, Jordan DR, Olberg B. Spindle-cell lipoma of the preseptal eyelid. Ophthal Plast Reconstr Surg 1998;14:174–7. 7. 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. 8. Dal Cin P, Sciot R, Polito P, et al. Lesions of 13q may occur independently of deletion of 16q in spindle cell/pleomorphic lipomas. Histopathology 1997;31:222–5. 9. Schmack I, Patel RM, Folpe AL, et al. Subconjunctival herniated orbital fat: a benign adipocytic lesion that may mimic pleomorphic lipoma and atypical lipomatous tumor. Am J Surg Pathol 2007;31:193–8. 10. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St. Louis: Mosby, 2001:571–639.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e29
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 2. A, Hematoxylin- and eosin-stained paraffin section showing the typical wiry/ropey collagen (arrows). B, Collections of mature adipocytes between the collagen (arrows); (C) bland wavy spindle cells (arrows); (D) occasional mast cell present (arrow); and (E) immunohistochemistry showing strong brown positive staining for CD34.
Orbital Sarcoid-Like Granulomatosis After Inhibition of Tumor Necrosis Factor-α Edward J. Wladis, M.D., F.A.C.S.*, Ashley J. Tarasen, M.D.†, Zachary J. Roth, M.D.‡, Martha G. Farber, M.D.†‡, Jeffrey Ross, M.D.†, and Victoria M. Michaels, M.D.§ Abstract: Pharmacologic inhibition of tumor necrosis factor-alpha (TNF-α) has been used in the management of a variety of inflammatory conditions. Recently, reports on the development of sarcoid-like granulomatous disease *Ophthalmic Plastic Surgery, Department of Ophthalmology, Lions Eye Institute, †Department of Pathology, ‡Department of Ophthalmology, Lions Eye Institute, and §Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, New York, U.S.A. Accepted for publication March 18, 2014. The authors disclose no financial or conflicts of interest. The information presented in this manuscript has not been presented elsewhere and is not under consideration for publication elsewhere. Address correspondence and reprint requests to Edward J. Wladis, M.D., F.A.C.S., 1220 New Scotland Rd, Suite 302, Slingerlands, NY 12159. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000200
e30
at multiple systemic sites after treatment with TNFα inhibitors have emerged, although, to the authors’ knowledge, orbital manifestations of this problem have not been previously described. A 48-year-old woman who received injections of adalimumab for the treatment of psoriatic arthritis developed right-sided orbital pain and inflammation. Orbital biopsy of a focal lesion demonstrated sarcoid-like granulomatosis, and a workup for other causes of this problem was noncontributory. This report represents the first documented case of this phenomenon in the orbit, and possible mechanisms are discussed in this presentation. Given the expanding role of TNF-α inhibitors and the increased frequency of their use, clinicians should be aware of this possible side effect.
I
nhibition of tumor necrosis factor-alpha (TNF-α) has emerged as an important modality in the management of multiple systemic inflammatory conditions.1 Currently, 3 TNF-αantagonists are commercially available, including etanercept, adalimumab, and infliximab, and these agents have been previously used to treat various ophthalmic and orbital conditions, including uveitis, scleritis, myositis, idiopathic orbital inflammation, and thyroid eye disease.2–5 While these medications are generally
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Case Reports
procedure. This leak was successfully treated with repair of the conjunctiva and fornix defect using amniotic membrane and placement of a temporary tarsorrhaphy.
REFERENCES 1. Van Haeringen JH. Clinical biochemistry of tears. In: Milder B, Weil B, eds. The Lacrimal System. Norwalk, CT: Appleton-Century Crofts, 1983:23–48. 2. Gasset AR, Braverman LE, Fleming MC, et al. Tear glucose detection of hyperglycemia. Am J Ophthalmol 1968;65:414–20. 3. Neuhaus RW, Baylis HI. Cerebrospinal fluid leakage after dacryocystorhinostomy. Ophthalmology 1983;90:1091–5. 4. Bagheri A, Naghibozakerin J, Yazdani S. Cerebrospinal fluid leakage during dacryocystorhinostomy in a patient with meningoencephalocele. Eur J Ophthalmol 2005;15:500–3. 5. Badilla J, Dolman PJ. Cerebrospinal fluid leaks complicating orbital or oculoplastic surgery. Arch Ophthalmol 2007;125:1631–4. 6. Fayet B, Racy E, Assouline M. Cerebrospinal fluid leakage after endonasal dacryocystorhinostomy. J Fr Ophtalmol 2007;30:129–34. 7. Wulc AE, Adams JL, Dryden RM. Cerebrospinal fluid leakage complicating orbital exenteration. Arch Ophthalmol 1989;107:827–30. 8. Schick B, Weber R, Mosler P, et al. [Long-term follow-up of frontobasal dura-plasty]. HNO 1997;45:117–22. 9. Lindstrom DR, Toohill RJ, Loehrl TA, et al. Management of cerebrospinal fluid rhinorrhea: the Medical College of Wisconsin experience. Laryngoscope 2004;114:969–74. 10. Joshi KK, Crockard HA. Traumatic cerebrospinal fluid fistula simulating tears. Case report. J Neurosurg 1978;49:121–3. 11. Dryden RM, Wulc AE. Pseudoepiphora from cerebrospinal fluid leak: case report. Br J Ophthalmol 1986;70:570–4. 12. Korinek AM, Baugnon T, Golmard JL, et al. Risk factors for adult nosocomial meningitis after craniotomy: role of antibiotic prophylaxis. Neurosurgery 2006;59:126–33; discussion 126–33.
Spindle Cell Lipoma of the Conjunctiva Fariha Shafi, F.R.C.Ophth.*, Bipin Gonglore, MS, FRCS*, Soupramanien Sandramouli, FRCS, MD*, and Hardeep S. Mudhar, Ph.D., F.R.C.Path.† Abstract: A 67-year-old woman presented with progressive enlargement of a long-standing mass on the surface of her OS associated with ocular surface irritation. The mass was excised en bloc. Histopathological examination showed a well-defined encapsulated tumor composed of wiry collagen containing bland spindle cells that were strongly positive for CD34 with scattered mature adipocytes. These features confirmed a diagnosis of spindle cell lipoma.
L
ipomas are the most common mesenchymal neoplasm. They most commonly develop on the trunk and extremities. Interestingly, lipomas have only rarely been reported in ocular tissues and many of these cases involve the orbit. Clinically, conjunctival lipomas must be differentiated from herniated orbital fat, dermoids, and the lacrimal gland.1 *New Cross Hospital, Wolverhampton, West Midlands; and †National Specialist Ophthalmic Pathology Service, Department of Histopathology, Royal Hallamshire Hospital, Sheffield, England, United Kingdom Accepted for publication February 14, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Fariha Shafi, F.R.C.Ophth., New Cross Hospital, Wolverhampton Road, Wolverhampton, WV10 0QP England, United Kingdom. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000189
e28
Spindle cell lipoma is a distinct variant of lipoma in which mature fat is replaced by collagen-forming spindle cells.2,3 A patient who developed spindle cell lipoma of the nasal bulbar conjunctiva with no orbital involvement is reported. To the authors knowledge, this is the first reported case of conjunctival spindle cell lipoma.
CASE REPORT A 67-year-old Caucasian woman was referred to the oculoplastic service with a swelling on the surface of her OS, which had been present for approximately 1 year but in recent months had increased slightly in size. The mass was associated with ocular surface irritation, and there was no history of associated pain, bleeding, or discharge. Her medical history included Crohn disease and a recent diagnosis of rectal carcinoma. On examination, her visual acuity was 20/40 OD and 20/40 OS. A soft mobile subconjunctival mass was present on the nasal conjunctiva measuring 8 mm in diameter (Fig. 1). There was no associated overlying inflammation or discoloration of the conjunctiva. No proptosis or resistance to retropulsion was noted, and extraocular movements were full. Pupillary reactions were normal, and dilated examination of the fundus was unremarkable. An excision biopsy of the lesion was performed under local anesthetic. Intraoperatively, the mass was noted to be a discrete smooth surfaced white nodule, which was confined to the bulbar conjunctiva and did not involve the orbit. The mass was removed en bloc after a careful dissection from the underlying sclera and sent for histopathological examination. Gross examination revealed a well-defined, smooth surfaced white nodule measuring 6 × 4 × 3 mm, the cut surface of which displayed a solid white appearance. Microscopy revealed a benign soft tissue lesion composed mostly of wiry, ropey collagen (Fig. 2A). Scattered among the collagen were groups of mature adipocytes (Fig. 2B), bland stromal spindle cells (Fig. 2C), and occasional mast cells (Fig. 2D). The spindle cells were strongly positive for CD34 (Fig. 2E) and negative for S100, melan-A, and smooth muscle actin (not shown). There were no neurofilament-positive axons among the collagen fibers. There were no floret-type cells or lipoblasts, and no mitotic activity was identified. The features were with those of a typical spindle cell lipoma. There was no evidence of metastatic carcinoma (Fig. 2).
DISCUSSION A recent clinical survey of 1,643 conjunctival tumors by Shields et al.4 revealed that the most common diagnoses were nevus (28%), melanoma (13%), primary acquired melanosis (11%), squamous cell carcinoma (11%), and lymphoid tumor (8%). Lipomatous tumors were only found in 23 patients (1%), and of these, only 3 patients were diagnosed with lipoma (the remaining 20 patients had the diagnosis of herniated orbital fat). Spindle cell lipoma is a distinct variant of lipoma in which spindle cells replace normal fat and was first described by Enzinger and Harvey2 in 1975. It is found more frequently in men aged 50 to 70 years (mean age 56 years) and is usually located in the subcutaneous tissue of the shoulder and posterior neck. Histologically, spindle cell lipomas are wellcircumscribed, rarely encapsulated masses consisting of mature adipocytes that are separated and partly replaced by groups of small, slender spindle cells. These cells show uniformity and have a single elongated nucleus and narrow, bipolar cytoplasmic processes.2 They lack atypia and possess little or no pleomorphism and have a varying quantity of dense birefringent collagen and mast cells. Floret cells are not found, but areas of vascularization may be present within the lesion.5 There are only 3 reported cases of spindle cell lipoma in the orbit.3,5,6
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 1. A, Patient with left medial conjunctival lesion visualized on left lateral gaze. B, Detailed view of conjunctival spindle cell lipoma.
The histologic differential diagnosis of spindle cell lipoma includes both benign and atypical adipocytic lesions. Benign pleomorphic lipomas have a cardinal histologic feature of scattered multinucleate giant cells with a floret-like arrangement of multiple hyperchromatic nuclei in eosinophilic cytoplasm.7 This is important as pleomorphic lipoma is now thought to lie at the opposite end of a common histologic spectrum with spindle cell lipoma, as both spindle cell lipoma and pleomorphic lipoma share identical cytogenetic chromosomal alterations.8 While in some cases it is possible to see transitions of spindle cell lipoma with pleomorphic lipoma in the same specimen, in this study this was not the case. Another important differential is subconjunctival prolapsed orbital fat, a degenerative process, composed of mature adipocytes, floret-like cells, and fibrous septa.9 However, this process is confined to the superotemporal subconjunctival area; the lesion in the case of this study was discrete and on the medial bulbar conjunctiva. However, wiry/ropy collagen and collections of bland spindle cells are absent in prolapsed orbital fat. Atypical lipomatous tumor also enters the histologic differential diagnosis. However, this contains lobules of adipocytes varying in size with fibrous septa containing atypical hyperchromatic spindle and multinucleate cells unlike the appearance of spindle cell lipoma. An important nonlipomatous soft tissue tumor that merits consideration is neurofibroma. The collagen however is coarser (said to resemble shredded carrots), many of the spindle cells are S100 positive, and there are neurofilament-positive axons coursing through it.10 None of these features were seen in this case. This case of spindle cell lipoma that the authors present is unique because the tumor involved the medial bulbar
conjunctiva without any orbital involvement, unlike orbital fat prolapse that involves the superotemporal conjunctiva. Although spindle cell lipoma has previously been shown to occur in the orbit,3,5,6 this is the first case to show purely conjunctival involvement. The diagnosis of spindle cell lipoma should therefore be considered when assessing nodular conjunctival lesions.
REFERENCES 1. Jordan DR, Tse DT. Herniated orbital fat. Can J Ophthalmol 1987;22:173–7. 2. Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer 1975;36:1852–9. 3. Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol 1985;100:605–9. 4. Shields CL, Demirci H, Karatza E, et al. Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;111:1747–54. 5. Johnson BL, Linn JG Jr. Spindle cell lipoma of the orbit. Arch Ophthalmol 1979;97:133–4. 6. Mawn LA, Jordan DR, Olberg B. Spindle-cell lipoma of the preseptal eyelid. Ophthal Plast Reconstr Surg 1998;14:174–7. 7. 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. 8. Dal Cin P, Sciot R, Polito P, et al. Lesions of 13q may occur independently of deletion of 16q in spindle cell/pleomorphic lipomas. Histopathology 1997;31:222–5. 9. Schmack I, Patel RM, Folpe AL, et al. Subconjunctival herniated orbital fat: a benign adipocytic lesion that may mimic pleomorphic lipoma and atypical lipomatous tumor. Am J Surg Pathol 2007;31:193–8. 10. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St. Louis: Mosby, 2001:571–639.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e29
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 2. A, Hematoxylin- and eosin-stained paraffin section showing the typical wiry/ropey collagen (arrows). B, Collections of mature adipocytes between the collagen (arrows); (C) bland wavy spindle cells (arrows); (D) occasional mast cell present (arrow); and (E) immunohistochemistry showing strong brown positive staining for CD34.
Orbital Sarcoid-Like Granulomatosis After Inhibition of Tumor Necrosis Factor-α Edward J. Wladis, M.D., F.A.C.S.*, Ashley J. Tarasen, M.D.†, Zachary J. Roth, M.D.‡, Martha G. Farber, M.D.†‡, Jeffrey Ross, M.D.†, and Victoria M. Michaels, M.D.§ Abstract: Pharmacologic inhibition of tumor necrosis factor-alpha (TNF-α) has been used in the management of a variety of inflammatory conditions. Recently, reports on the development of sarcoid-like granulomatous disease *Ophthalmic Plastic Surgery, Department of Ophthalmology, Lions Eye Institute, †Department of Pathology, ‡Department of Ophthalmology, Lions Eye Institute, and §Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, New York, U.S.A. Accepted for publication March 18, 2014. The authors disclose no financial or conflicts of interest. The information presented in this manuscript has not been presented elsewhere and is not under consideration for publication elsewhere. Address correspondence and reprint requests to Edward J. Wladis, M.D., F.A.C.S., 1220 New Scotland Rd, Suite 302, Slingerlands, NY 12159. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000200
e30
at multiple systemic sites after treatment with TNFα inhibitors have emerged, although, to the authors’ knowledge, orbital manifestations of this problem have not been previously described. A 48-year-old woman who received injections of adalimumab for the treatment of psoriatic arthritis developed right-sided orbital pain and inflammation. Orbital biopsy of a focal lesion demonstrated sarcoid-like granulomatosis, and a workup for other causes of this problem was noncontributory. This report represents the first documented case of this phenomenon in the orbit, and possible mechanisms are discussed in this presentation. Given the expanding role of TNF-α inhibitors and the increased frequency of their use, clinicians should be aware of this possible side effect.
I
nhibition of tumor necrosis factor-alpha (TNF-α) has emerged as an important modality in the management of multiple systemic inflammatory conditions.1 Currently, 3 TNF-αantagonists are commercially available, including etanercept, adalimumab, and infliximab, and these agents have been previously used to treat various ophthalmic and orbital conditions, including uveitis, scleritis, myositis, idiopathic orbital inflammation, and thyroid eye disease.2–5 While these medications are generally
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
