CASE REPORT
Nodular Fasciitis Involving the Cornea: A Case Report and Review of the Literature Jedediah McClintic, MD,* Thomas Plesec, MD,† Steven Billings, MD,† and Arun D. Singh, MD*
Purpose: To report a case of nodular fasciitis involving the cornea and to review the literature of all published cases of epibulbar nodular fasciitis.
Methods: A 38-year-old man presented with a progressively enlarging flesh-colored nodule adherent to the central cornea of his right eye. Results: The nodule was surgically excised. Pathologic examination and immunohistochemistry were consistent with nodular fasciitis, a pseudoneoplastic spindle cell tumor. Conclusions: A review of the literature confirms this is the first reported case of nodular fasciitis involving the cornea since the original series by Zimmerman et al describing nodular fasciitis of the eye. Surgical excision alone is likely the most appropriate treatment option. Key Words: nodular fasciitis, dermoid, keloid (Cornea 2015;34:1168–1169)
odular fasciitis is a benign fibroblastic process that rarely involves ocular and adnexal structures. Clinically, these tumors may raise concern for malignancy due to their rapid growth. Since the initial report by Font and Zimmerman in 1966, only 6 additional cases of epibulbar nodular fasciitis have been published.1–5 In this study, we report the case of a 38-year-old man with nodular fasciitis of the limbus and cornea.
N
CASE REPORT A 38-year-old white man presented with an enlarging epibulbar mass in his right eye of 3 weeks duration. He was morbidly obese with obstructive sleep apnea, a longstanding history of floppy eyelids, and hypertension. Both upper and lower eyelids were lax. A flesh-colored nonpedunculated nodule (10 · 8 ·5 mm) adherent and covering most of the cornea with inferior feeder vessels was evident. The surface of the lesion was smooth without ulceration or vessels (Fig. 1). Ultrasound biomicroscopy revealed a corneal thinning of 0.3 mm. Differential diagnosis included corneal keloid, atypical conjuntival intraepithelial neoplasia, and fibrous Received for publication February 28, 2015; revision received April 5, 2015; accepted April 7, 2015. Published online ahead of print June 9, 2015. From the *Cole Eye Institute, Cleveland Clinic, Cleveland, OH; and †Anatomic Pathology, Cleveland Clinic, Cleveland, OH. The authors have no funding or conflicts of interest to disclose. Reprints: Arun D. Singh, MD, Cole Eye Institute, i32 Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195 (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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histiocytoma. Intraoperatively, the mass seemed to arise from the inferior limbus and conjunctiva, lacked intrinsic vascularity, and was firmly adherent to the corneal stroma. Approximately 90% of the lesion was excised in 3 successive lamellar dissections. Further excision was deferred due to the risk of corneal perforation.
PATHOLOGY The nodular lesion was composed of plump, stellate spindle cells with pale-staining oval nuclei and prominent nucleoli arranged in short and irregular fascicles and embedded within a pale myxoid stroma. Scattered extravasated red blood cells were present. Nuclear hyperchromasia and atypical mitotic figures were absent. Two mitotic figures per 10 high-power field (field diameter 0.55 mm) were present. Smooth muscle actin immunostain demonstrated patchy positivity in the spindle cells. S-100 protein immunostain was negative. The tissue proliferation was limited to stromal layers, with sparing of the overlying epithelium (Fig. 1).
DISCUSSION Although the exact etiology is still debatable, nodular fasciitis is postulated to be a reactive process in connective tissue exposed to repeated trauma or inflammation. Similar to another recent case report, our patient had a history of floppy eyelid syndrome, which may have predisposed him to chronic irritation required to incite this condition.4 Although most reported cases have occurred in the orbit, eyelid, or periorbital regions, there have been only 6 cases of epibulbar nodular fasciitis published in the literature (Table 1). The only case of corneal involvement was described by Font and Zimmerman in a 3-year-old girl, who developed a nodule arising from the limbus with extension through the cornea and into the iris and ciliary body. Of the now 7 reported epibulbar lesions, 4 invaded the sclera and 2 showed invasion of the cornea. In our case, ultrasound biomicroscopy was useful in determining the extent of corneal depth and formulating a proper surgical plan. Owing to its clinical behavior of rapid growth, this condition often masquerades as a soft tissue malignancy, and histopathologic distinction from other spindle cell tumors such as sarcomas may prove challenging. In general, nodular fasciitis is a morphology-based diagnosis—the characteristic “tissue culture” appearance of the smooth muscle actin–positive myofibroblastic proliferation, the myxoid to fibrous background, and the RBC extravasation all contribute to the diagnosis. In our case, Cornea Volume 34, Number 9, September 2015
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Cornea Volume 34, Number 9, September 2015
Nodular Fasciitis Involving the Cornea
FIGURE 1. Preoperative (A) and postoperative (B) appearance on slitlamp examination. Spindle cell proliferation composed of regular delicate cells with pale chromatin and small nucleoli arranged in short irregular fascicles (C, hematoxylin and eosin, ·200). Smooth muscle actin stain of spindle cells with variable positivity, consistent with myofibroblastic differentiation (D).
S-100 protein negativity helped to exclude spindle cell peripheral nerve sheath tumors or unusual melanocytic lesions. The lesion lacked the biphasic growth pattern of a myofibroma and did not reveal a hemangiopericytoma-type vascular pattern.6,7 Other benign spindle cell proliferations that were considered include benign fibrous histiocytoma and fibromatosis. Benign fibrous histiocytoma was excluded given the absence of peripheral collagen trapping, storiform growth pattern, and reactive epithelial changes overlying the lesion.8 Last, fibromatosis was also excluded due to the lack of broad, sweeping fascicles of the spindle cells.9 Treatment of ocular nodular fasciitis is surgical excision. Orbital and periorbital nodules tend to be well defined
and typically allow for clear margins. Nodules approximating the globe however should raise suspicion for infiltration into the muscle, sclera, and cornea. This presents a challenge at the time of excision, demanding partial thickness sclerectomy or lamellar keratectomy. Ultimately, however, total excision may not be critical in such cases. Excision of the limbal nodule in Font’s 3-year-old patient was limited to its external portion only, and recurrence was never observed despite 13 years of follow-up. In fact, Hutter reported cases of nodular fasciitis that regressed spontaneously.10 In conclusion, we present the case of a 38-year-old man with limbal and corneal nodular fasciitis highlighting a rare entity that may mimic a malignant tumor. REFERENCES
TABLE 1. Reported Cases of Epibulbar Nodular Fasciitis Report
Eye
Location
Font and Zimmerman5 Font and Zimmerman5
R
Temporal
Tolls et al1
L
Ferry and Sherman2 Holds et al3
L
L
R
Stone and Chodosh4
R
Present case 2015
R
Involved Structures
Cornea, iris, ciliary body Temporal Lateral rectus insertion, scleral invasion Inferior Medial border inferior rectus, scleral invasion Inferonasal Tenon capsule inferior fornix Superonasal Superior rectus insertion Inferotemporal Inferior border lateral rectus, scleral invasion Inferior Inferior limbus, corneal invasion
Sex
Age
Female
3
Female
25
Male
43
Female
47
Female
3
Male
53
Male
38
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
1. Tolls RE, Mohr S, Spencer WH. Benign nodular fasciitis originating in Tenon’s capsule. Arch Ophthalmol. 1966;75:482–483. 2. Ferry AP, Sherman SE. Nodular fasciitis of the conjunctiva apparently originating in the fascia bulbi (Tenon’s capsule). Am J Ophthalmol. 1974;78:514–517. 3. Holds JB, Mamalis N, Anderson RL. Nodular fasciitis presenting as rapidly enlarging episcleral mass in a 3 year-old. J Pediatr Ophthalmol Strabismus. 1990;27:157–160. 4. Stone DU, Chodosh J. Epibulbar nodular fasciitis associated with floppy eyelids. Cornea. 2005;24:361–362. 5. Font RL, Zimmerman LE. Nodular fasciitis of the eye and adnexa. Arch Ophthalmol. 1966;75:475–481. 6. Fletcher CD, Achu P, Van Noorden S, et al. Infantile myofibromatosis: a light microscopic, histochemical, and immunohistochemical study suggesting true smooth muscle differentiation. Histopathology. 1987; 11:245–258. 7. Beham A, Badve S, Suster S, et al. Solitary myofibroma in adults: clinicopathologic analysis of a series. Histopathology. 1993;22:235–241. 8. Calonje E, Fletcher CDM. Cutaneous fibrohistiocytic tumors: an update. Adv Anat Pathol. 1994;1:2–13. 9. Goldblum JR, Fletcher JA. Fibromatosis. In: Fletcher CDM, Bridge JA, Hogendoorn P, et al, eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. WHO; 2013:70–73. 10. Hutter RVP, Stewart FW, Foote FW. Fasciitis: a report of 70 cases with follow-up proving benignity of the lesion. Cancer. 1962;15:992–1003.
www.corneajrnl.com |
1169
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Nodular Fasciitis Involving the Cornea: A Case Report and Review of the Literature Jedediah McClintic, MD,* Thomas Plesec, MD,† Steven Billings, MD,† and Arun D. Singh, MD*
Purpose: To report a case of nodular fasciitis involving the cornea and to review the literature of all published cases of epibulbar nodular fasciitis.
Methods: A 38-year-old man presented with a progressively enlarging flesh-colored nodule adherent to the central cornea of his right eye. Results: The nodule was surgically excised. Pathologic examination and immunohistochemistry were consistent with nodular fasciitis, a pseudoneoplastic spindle cell tumor. Conclusions: A review of the literature confirms this is the first reported case of nodular fasciitis involving the cornea since the original series by Zimmerman et al describing nodular fasciitis of the eye. Surgical excision alone is likely the most appropriate treatment option. Key Words: nodular fasciitis, dermoid, keloid (Cornea 2015;34:1168–1169)
odular fasciitis is a benign fibroblastic process that rarely involves ocular and adnexal structures. Clinically, these tumors may raise concern for malignancy due to their rapid growth. Since the initial report by Font and Zimmerman in 1966, only 6 additional cases of epibulbar nodular fasciitis have been published.1–5 In this study, we report the case of a 38-year-old man with nodular fasciitis of the limbus and cornea.
N
CASE REPORT A 38-year-old white man presented with an enlarging epibulbar mass in his right eye of 3 weeks duration. He was morbidly obese with obstructive sleep apnea, a longstanding history of floppy eyelids, and hypertension. Both upper and lower eyelids were lax. A flesh-colored nonpedunculated nodule (10 · 8 ·5 mm) adherent and covering most of the cornea with inferior feeder vessels was evident. The surface of the lesion was smooth without ulceration or vessels (Fig. 1). Ultrasound biomicroscopy revealed a corneal thinning of 0.3 mm. Differential diagnosis included corneal keloid, atypical conjuntival intraepithelial neoplasia, and fibrous Received for publication February 28, 2015; revision received April 5, 2015; accepted April 7, 2015. Published online ahead of print June 9, 2015. From the *Cole Eye Institute, Cleveland Clinic, Cleveland, OH; and †Anatomic Pathology, Cleveland Clinic, Cleveland, OH. The authors have no funding or conflicts of interest to disclose. Reprints: Arun D. Singh, MD, Cole Eye Institute, i32 Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195 (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
1168
| www.corneajrnl.com
histiocytoma. Intraoperatively, the mass seemed to arise from the inferior limbus and conjunctiva, lacked intrinsic vascularity, and was firmly adherent to the corneal stroma. Approximately 90% of the lesion was excised in 3 successive lamellar dissections. Further excision was deferred due to the risk of corneal perforation.
PATHOLOGY The nodular lesion was composed of plump, stellate spindle cells with pale-staining oval nuclei and prominent nucleoli arranged in short and irregular fascicles and embedded within a pale myxoid stroma. Scattered extravasated red blood cells were present. Nuclear hyperchromasia and atypical mitotic figures were absent. Two mitotic figures per 10 high-power field (field diameter 0.55 mm) were present. Smooth muscle actin immunostain demonstrated patchy positivity in the spindle cells. S-100 protein immunostain was negative. The tissue proliferation was limited to stromal layers, with sparing of the overlying epithelium (Fig. 1).
DISCUSSION Although the exact etiology is still debatable, nodular fasciitis is postulated to be a reactive process in connective tissue exposed to repeated trauma or inflammation. Similar to another recent case report, our patient had a history of floppy eyelid syndrome, which may have predisposed him to chronic irritation required to incite this condition.4 Although most reported cases have occurred in the orbit, eyelid, or periorbital regions, there have been only 6 cases of epibulbar nodular fasciitis published in the literature (Table 1). The only case of corneal involvement was described by Font and Zimmerman in a 3-year-old girl, who developed a nodule arising from the limbus with extension through the cornea and into the iris and ciliary body. Of the now 7 reported epibulbar lesions, 4 invaded the sclera and 2 showed invasion of the cornea. In our case, ultrasound biomicroscopy was useful in determining the extent of corneal depth and formulating a proper surgical plan. Owing to its clinical behavior of rapid growth, this condition often masquerades as a soft tissue malignancy, and histopathologic distinction from other spindle cell tumors such as sarcomas may prove challenging. In general, nodular fasciitis is a morphology-based diagnosis—the characteristic “tissue culture” appearance of the smooth muscle actin–positive myofibroblastic proliferation, the myxoid to fibrous background, and the RBC extravasation all contribute to the diagnosis. In our case, Cornea Volume 34, Number 9, September 2015
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Cornea Volume 34, Number 9, September 2015
Nodular Fasciitis Involving the Cornea
FIGURE 1. Preoperative (A) and postoperative (B) appearance on slitlamp examination. Spindle cell proliferation composed of regular delicate cells with pale chromatin and small nucleoli arranged in short irregular fascicles (C, hematoxylin and eosin, ·200). Smooth muscle actin stain of spindle cells with variable positivity, consistent with myofibroblastic differentiation (D).
S-100 protein negativity helped to exclude spindle cell peripheral nerve sheath tumors or unusual melanocytic lesions. The lesion lacked the biphasic growth pattern of a myofibroma and did not reveal a hemangiopericytoma-type vascular pattern.6,7 Other benign spindle cell proliferations that were considered include benign fibrous histiocytoma and fibromatosis. Benign fibrous histiocytoma was excluded given the absence of peripheral collagen trapping, storiform growth pattern, and reactive epithelial changes overlying the lesion.8 Last, fibromatosis was also excluded due to the lack of broad, sweeping fascicles of the spindle cells.9 Treatment of ocular nodular fasciitis is surgical excision. Orbital and periorbital nodules tend to be well defined
and typically allow for clear margins. Nodules approximating the globe however should raise suspicion for infiltration into the muscle, sclera, and cornea. This presents a challenge at the time of excision, demanding partial thickness sclerectomy or lamellar keratectomy. Ultimately, however, total excision may not be critical in such cases. Excision of the limbal nodule in Font’s 3-year-old patient was limited to its external portion only, and recurrence was never observed despite 13 years of follow-up. In fact, Hutter reported cases of nodular fasciitis that regressed spontaneously.10 In conclusion, we present the case of a 38-year-old man with limbal and corneal nodular fasciitis highlighting a rare entity that may mimic a malignant tumor. REFERENCES
TABLE 1. Reported Cases of Epibulbar Nodular Fasciitis Report
Eye
Location
Font and Zimmerman5 Font and Zimmerman5
R
Temporal
Tolls et al1
L
Ferry and Sherman2 Holds et al3
L
L
R
Stone and Chodosh4
R
Present case 2015
R
Involved Structures
Cornea, iris, ciliary body Temporal Lateral rectus insertion, scleral invasion Inferior Medial border inferior rectus, scleral invasion Inferonasal Tenon capsule inferior fornix Superonasal Superior rectus insertion Inferotemporal Inferior border lateral rectus, scleral invasion Inferior Inferior limbus, corneal invasion
Sex
Age
Female
3
Female
25
Male
43
Female
47
Female
3
Male
53
Male
38
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
1. Tolls RE, Mohr S, Spencer WH. Benign nodular fasciitis originating in Tenon’s capsule. Arch Ophthalmol. 1966;75:482–483. 2. Ferry AP, Sherman SE. Nodular fasciitis of the conjunctiva apparently originating in the fascia bulbi (Tenon’s capsule). Am J Ophthalmol. 1974;78:514–517. 3. Holds JB, Mamalis N, Anderson RL. Nodular fasciitis presenting as rapidly enlarging episcleral mass in a 3 year-old. J Pediatr Ophthalmol Strabismus. 1990;27:157–160. 4. Stone DU, Chodosh J. Epibulbar nodular fasciitis associated with floppy eyelids. Cornea. 2005;24:361–362. 5. Font RL, Zimmerman LE. Nodular fasciitis of the eye and adnexa. Arch Ophthalmol. 1966;75:475–481. 6. Fletcher CD, Achu P, Van Noorden S, et al. Infantile myofibromatosis: a light microscopic, histochemical, and immunohistochemical study suggesting true smooth muscle differentiation. Histopathology. 1987; 11:245–258. 7. Beham A, Badve S, Suster S, et al. Solitary myofibroma in adults: clinicopathologic analysis of a series. Histopathology. 1993;22:235–241. 8. Calonje E, Fletcher CDM. Cutaneous fibrohistiocytic tumors: an update. Adv Anat Pathol. 1994;1:2–13. 9. Goldblum JR, Fletcher JA. Fibromatosis. In: Fletcher CDM, Bridge JA, Hogendoorn P, et al, eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. WHO; 2013:70–73. 10. Hutter RVP, Stewart FW, Foote FW. Fasciitis: a report of 70 cases with follow-up proving benignity of the lesion. Cancer. 1962;15:992–1003.
www.corneajrnl.com |
1169
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
