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| | | Year : 2010 | Volume : 53 | Issue : 4 | Page : 850-852 | | | Eosinophilic angiocentric fibrosis of orbit | | | Muhammad Azam1, Yousuf A Husen2, Sheema H Hasan1
1 Department of Pathology & Microbiology, Aga Khan University, Karachi, Pakistan
2 Department of Radiology, Aga Khan University, Karachi, Pakistan
Click here for correspondence address and email | Date of Web Publication | 27-Oct-2010 | | |      | | How to cite this article:
Azam M, Husen YA, Hasan SH. Eosinophilic angiocentric fibrosis of orbit. Indian J Pathol Microbiol 2010;53:850-2 |
Eosinophilic angiocentric fibrosis (EAF) is a rare recently described benign but slowly progressing disease. Very few cases of this entity have been described in the literature. First case was reported by Holmes and Panje [1] as "intranasal granuloma faciale" and renamed by Roberts and McCann [2] as "Eosinophilic angiocentric fibrosis." Most of the cases involve sinonasal tract and only two cases had been reported involving orbit. We report a case of 35-year-old woman with EAF of the orbit.
A 35-year-old woman presented with a history of pain and right lower eyelid swelling of 4 years duration. Initially, it subsided spontaneously but later she developed persistent swelling which increased in size gradually. The patient had history of trauma to the right maxillary area and lower eyelid with bruise formation, 6 months prior to the appearance of swelling. There was no history of visual disturbance, skin lesions, nasal symptoms, asthma, or atopy. No immunological abnormality or other clinical diseases had been reported. There was no history of self-medication. In 2005, computed tomography scans of head and brain revealed soft tissue swelling beneath the right lower eyelid outside the orbit. Eyeballs, optic nerves, extraocular muscles, and retrobulbar spaces were normal. Adjacent maxillary bone and paranasal sinuses appeared unremarkable [Figure 1]a. Patient did not seek any treatment. There was gradual increase in the size of lesion and later on, she developed pain and epiphora. In August 2008, unenhanced magnetic resonance imaging of paranasal sinuses showed abnormal signal intensity mass involving retrobulbar area of right orbit encasing the eyeball. It also involved the lower eyelid, subcutaneous fat. The right optic nerve was not involved [Figure 1]b.  | Figure 1a: Contrast-enhanced coronal scan shows thick rind of mildly enhancing soft tissue around right eye ball
b: Soft tissue thickening seen isointense to the muscle, around right eye ball
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Excision biopsy of the lesion was done. On gross examination, the specimen consisted of multiple fragments of tan gray soft tissue measuring 4.5×3.0×1.5 cm in aggregate. Cut surface of the fragments was tan gray and fibrotic. Histological examination revealed a lesion composed of fibrocollagenous tissue with variable areas of fibrosis and dense inflammation comprising of lymphocytes, plasma cells, and numerous eosinophils. Lymphoid aggregates were also noted. Scattered blood vessels were seen showing characteristic onionskin whirling perivascular fibrosis [Figure 2]a,b. This perivascular whirling pattern of fibrosis was highlighted on Masson trichrome special stain [Figure 2]c. Eosinophils were seen clustering around the vessels and migrating through the walls. No fibrinoid necrosis was seen [Figure 2]d. Few areas showed dense stromal fibrosis with scanty inflammatory cells. Foci of calcification were seen. No granulomata, geographic necrosis, or nuclear dust was noted. No organisms identified on special stains . Laboratory investigations revealed normal erythrocyte sedimentation rate, complete blood count, serum anti-IgE levels, and negative serum ANCA. Based on these characteristic findings, the case was diagnosed as EAF.  | Figure 2a: Fibrous areas with scattered blood vessels showing perivascular fibrosis (H and E, ×40)
b: Blood vessels with perivascular onion skin whirling fibrosis. Mixed inflammation rich in eosinophils is also present in the background H and E, ×100)
c: Masson trichrome stain demonstrate perivascular onion skin whirling fibrosis (Masson trichrome stain, ×100)
d: A vessel showing eosinophilic vasculitis without fibrinoid necrosis H and E, ×100)
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On follow-up, the patient was asymptomatic 8 months after surgery.
EAF is a very rare condition of unknown etiology. In 1983, Holmes and Punje reported a 49-year-old man with recurrent intranasal mass accompanied by erythematous plaques on the forehead. [1] Later, Roberts and McCann [2] renamed this peculiar benign condition in a case report of three cases as EAF in 1985.They also reported two additional cases in in1997. [3] To best of our knowledge, only 23 cases of this entity have reported so far. Initial reports indicated a female preponderance; however, there seems to be equal gender distribution. The age of onset ranged from third to eighth decades. [4] Sinonasal tract was involved in most of the previous cases. Four cases had extension into the orbit. In two cases, the lesion was primarily located in the orbit. [4],[5] The symptoms and radiological findings are predominantly nonspecific as described in the literature. In our patient, there is long duration of symptoms suggesting a slowly progressing disease process. Etiology is unknown. However, history of typical eosinophil-rich inflammation in some patients suggests an allergic origin. In our patient, there is no history of allergies rather she had trauma with bruise formation. The history of trauma might have some relationship to the development of this disease process.
The characteristic histological findings are the basis for establishing the diagnosis. According to Roberts and McCann, the lesions can be separated into early and late stages. [2] Both patterns can be seen in a same biopsy suggesting a continually evolving process. Eosinophilic vasculitis without fibrinoid necrosis is common in early lesions occurring in a patchy fashion and involving groups of capillaries and venules. The late lesions are characterized by dense perivascular onionskin whirling fibrosis. Our case also showed both early and late patterns.
The differential diagnosis includes Wegener granulomatosis, Churg-strauss syndrome, granuloma faciale, and infections. [6] Previously a case with associated Wegener granulomatosis had also been described. [7] In our case, there was no granuloma formation, fibrinoid necrosis, geographic necrosis, nuclear dust, or history of facial nodules. Serum IgE levels were normal and serum ANCA was negative.
EAF is an indolent and progressive benign disorder without malignant transformation. No previous case of EAF-related mortality is recorded. [4] Recurrences usually occur at the same anatomic site. Surgery and corticosteroids may provide some symptomatic relief and local control. However, most authors think a wide local excision with replacement of the defect by noninvolved tissue may be more effective than immunosuppressive treatment. [8]
 References | |  |
| 1. | Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol 1983;4:184-6. 
[PUBMED] | | 2. | Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? A report of three cases. Histopathology 1985;9:1217-25.
[PUBMED] | | 3. | Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: A postscript. Histopathology 1997;31:385-86.
[PUBMED] | | 4. | Leibovitch I, James CL, Wormald PJ, Selva D. Orbital eosinophilic angiocentric fibrosis, A case report and review of literature. Ophthalmology 2006;113:148-52.
[PUBMED] [FULLTEXT] | | 5. | Kiratli H, Onder S, Yildiz S, Ozseka h. Eosinophilic angiocentric fibrosis of orbit. Clin Expeiment Ophthalmol 2008;36:274-76.
| | 6. | Thompson LD, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis: A report of three cases. Am J Clin Pathol 2001;115:243-48.
[PUBMED] [FULLTEXT] | | 7. | Loane J, Jaramillo M, Young HA, Kerr KM. Eosinophilic angiocentric fibrosis and Wegener's granulomatosis: A case report and literature review. J Clin Pathol 2001;54:640-41.
[PUBMED] [FULLTEXT] | | 8. | Valenzuela AA, Whitehead KJ, Sullivan TJ. Eosinophilic angiocentric fibrosis: An unusual entity producing complete lacrimal duct obstruction. Orbit 2006;25:159-60.
[PUBMED] [FULLTEXT] |
Correspondence Address:
Muhammad Azam
Department of Pathology & Microbiology, Aga Khan University, Karachi
Pakistan
DOI: 10.4103/0377-4929.72086 PMID: 21045443
[Figure 1], [Figure 2] | |
| This article has been cited by | | 1 | Primary orbital eosinophilic angiocentric fibrosis with intranasal extension | | | Apostolos Karligkiotis,Luca Volpi,Fabio Ferreli,Michele Cerati,Effrosyni Kagkelari,Francesco Meloni,Paolo Castelnuovo | | Head & Neck. 2013; : n/a | | [Pubmed] | |
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