- Email: [email protected]
Eosinophilic angiocentric fibrosis of the sinonasal tract
Available online at www.sciencedirect.com
Annals of Diagnostic Pathology 12 (2008) 267 – 270
Eosinophilic angiocentric fibrosis of the sinonasal tract Ognjen Kosarac, MDa, Mario A. Luna, MDb, Jae Y. Ro, MD, PhDa, Alberto G. Ayala, MDa,4 a
b
Department of Pathology, The Methodist Hospital, Houston, TX 77030, USA Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA
Abstract
Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion of the upper respiratory tract and orbit that occurs mainly in young to middle-aged women. The etiology of EAF is unknown. To our knowledge, approximately 28 cases have been previously reported in the English literature. We report here 3 additional cases of EAF of the sinonasal tract; 2 in women aged 19 and 31 years, and 1 in a man aged 49 years. The 19-year-old woman is the youngest patient with EAF ever described. The patients presented with a nasal cavity mass, face pain, or nasal obstructive symptoms of long duration. D 2008 Elsevier Inc. All rights reserved.
Keywords:
Eosinophilic angiocentric fibrosis; Nasal cavity; Onion-skin pattern
1. Introduction Eosinophilic angiocentric fibrosis (EAF) is a rare, benign, obstructive lesion affecting the respiratory mucosa of the nasal cavity, larynx, and orbit. Since its first description in 1985 by Roberts and McCann [1], approximately 28 cases of EAF have been described in the English literature. The specific histologic features include a perivascular inflammatory cell infiltration (mainly eosinophils), which is gradually replaced with progressive fibrosis (bonion-skinQ pattern) around small blood vessels [1-17]. The etiology of EAF is unknown, and diagnosis is based on specific histologic findings. We report 3 cases of EAF, including one of a 19-year-old woman, the youngest patient described so far with this disease. 2. Case reports The pertinent clinical history, therapy, and follow-up of the 3 patients with EAF of the sinonasal tract are summarized in Table 1. 2.1. Case 1 A 19-year-old woman presented with recurrent right nasal congestion and face pain. Computed tomography 4 Corresponding author. Tel.: +1 713 441 1339; fax: +1 713 793 1603. E-mail address: [email protected] (A.G. Ayala). 1092-9134/$ – see front matter D 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.anndiagpath.2007.02.002
showed a complete opacification of the right maxillary sinus, extending to the adjacent middle meatus. Right endoscopic sinus surgery was performed, and a large fibrotic mass measuring 4 3 cm was found within the right maxillary sinus. 2.2. Case 2 A 31-year-old woman presented with nasal obstruction and a mass involving the right side of the nasal cavity at 5 years evolution. A physical examination showed a flat tumor forming a plaque measuring 3 2 cm. 2.3. Case 3 A 49-year-old man presented with nasal obstruction and a mass in the nasal septum at 3 years evolution. A physical examination showed a plaque in the nasal septum measuring 2 1.6 cm. 3. Pathologic findings The histologic appearance of the EAF was almost identical for all 3 cases. The normal architecture of the maxillary sinus (case 1), the right side of the nasal cavity (case 2), and the nasal septum (case 3) were completely distorted by fibrous-collagenous proliferation with a variable dense inflammatory infiltrate. The fibrous component displayed a concentrically layered onion-skin–type perivascular arrangement (Fig. 1A, B, and C), which is character-
268
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
Table 1 Summary of 3 new cases of EAF Age (y)
Sex
Clinical history
Site of lesion
Management
Follow up
19 31 49
Female Female Male
Nasal congestion and face pain Nasal obstruction Nasal obstruction
Right maxillary sinus Right nasal cavity Nasal septum
Surgical excision Surgical excision Surgical excision
Free of disease—1 year Free of disease—7 years Free of disease—12 years
istic of EAF. The inflammatory infiltrate consisted of lymphocytes, plasma cells, scattered neutrophils, and numerous eosinophils (Fig. 2A, B, and C). Other areas of the lesion demonstrated dense fibrosis with scanty inflammatory infiltrate. There were no granulomas, foreign-body giant cells, or fibrinoid necroses. 4. Treatment and follow-up The lesions were completely curettaged, and the patients were free of disease at 1, 7, and 12 years, respectively (see Table 1). 5. Discussion Although most of the 28 reported cases of EAF occurred in the nasal cavity (25 cases), including 3 cases with extension into the orbit, 2 cases occurred in the larynx, and 1 case occurred in the orbit (Table 2). Patient ages ranged from 25 to 79 years (median, 50.5 years), with a slight female preponderance (female-male ratio, 1.5/1). Eosinophilic angiocentric fibrosis is a slowly progressing disease that can exist for more than 4 years before it is diagnosed [1-3,6]. The etiology of EAF remains unknown. Some researchers have suggested that the association of EAF with allergic and atopic disorders and the predominance of an eosinophilic infiltrate represent an abnormal inflammatory response to a nonspecific stimulus in predisposed individuals [7,10]. Trauma may play a role in this condition because most patients had a history of nasal surgery or trauma to the nose before the onset of EAF [1,2,4,5,14,15]. Eosinophilic angiocentric fibrosis is also considered the mucosal variant of granuloma faciale because its histology is similar to that of this condition and because it has a rare association with granuloma faciale [1,12,15,16]. Symptoms are nonspecific and include nasal obstruction, nasal discharge, epistaxis, sinusitis, pain, and a mass [1-6,11]. A recent patient with orbital involvement presented with periorbital edema and painless proptosis [17]. The imaging findings are usually nonspecific and include clouding and opacification of the sinuses, bony sclerosis, and focal destruction of the surrounding sinusoidal or nasal cavity bony tissue [6,17]. Histologically, EAF covers the spectrum of lesions from an early inflammatory lesion to a late fibrotic lesion. The early lesion consists of an eosinophilic vasculitis without fibrinoid necrosis involving small blood vessels (capillaries and/or venules). The eosinophilic infiltrate predominates but
is invariably accompanied by plasma cells and lymphocytes. Furthermore, a granulomatous-type picture may be present but without true granulomas consisting of epithelioid cells or multinucleated giant cells. In contrast, the late lesion shows a dense fibrous thickening of the subepithelial stroma with obliterative perivascular onion-skin whorling of collagen and reticulin fibers. The inflammatory infiltrate is scanty, but eosinophils remain in the lesion [1,13]. The differential diagnosis of EAF includes infectiousinflammatory processes, including granulomatous diseases, Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, angiolymphoid hyperplasia with eosinophilia, erythema elevatum diutitum, inflammatory pseudotumor, and granuloma faciale. It may rarely simulate neoplastic (neurogenic, vascular, mesenchymal tumors) conditions. An initial approach to the differential diagnosis is to exclude some of these conditions by usual laboratory diagnostics tests, such as antineutrophil cytoplasmic antibodies, erythrocyte sedimentation rate, immunoglobulin E serum titers, and central and peripheral eosinophilic counts [5]. Nasal disease affects the mucosa of the lateral wall and septum, and this may help to clinically differentiate EAF from midline granulomatous diseases [13,15]. In addition, the absence of granulomas, negative staining with special stains (Gomori methenamine silver, acid-fast bacilli), and the characteristic whorling and onion-skin pattern of perivascular fibrosis strongly favor EAF. The limited form of Wegener granulomatosis, which is restricted to the nose and upper respiratory tract, should be considered in the differential diagnosis [8]. However, Wegener granulomatosis contains substantial necrobiosis, vasculitis, and scattered foreign-body–type giant cells. Churg-Strauss syndrome has fibrinoid necrosis and usually contains granulomas [6]. It is not difficult to rule out Kimura disease, a rare lesion in the Western hemisphere, which shows a mixed inflammatory infiltrate with a prominent fibrosis but without onion-skin perivascular fibrosis [5,6]. Angiolymphoid hyperplasia with eosinophilia is a lesion of the skin of the head and neck that characteristically displays eosinophilic infiltrate and should be differentiated from EAF. Furthermore, in the hyperplastic stage of this disease, there is a conspicuous vascular proliferation with prominent epithelioid endothelial cells that are not seen in typical cases of EAF [3,5,6]. Granuloma faciale is a rare inflammatory condition that affects the skin and may coexist with EAF [9,12,15]. Although some researchers consider EAF to be an extracutaneous lesion of granuloma faciale [11,12,15], others believe it is a separate clinical and histologic entity [7].
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
269
ically (steroids, dapsone, azathioprine) have been unsuccessful. As such, surgical resection appears to be the treatment of choice. However, recurrences are common, requiring multiple surgeries [5,6,11,13,15,17].
Fig. 1. (A) Low-power view of EAF. Note numerous fibrotic nodules consisting of concentrically layered perivascular fibrosis and no vasculitis (hematoxylin-eosin, 200). (B) At medium power, there are several nodules with onion-skin–type perivascular fibrosis. At this stage, the blood vessel lumen is obliterated. (C) In contrast to the previous illustration, this high-power view of the perivascular fibrosis does contain a blood vessel.
The treatment of choice for a patient with EAF remains unclear. Attempts at decreasing the inflammatory response both locally (nasal and intranasal steroids) and systemat-
Fig. 2. (A) The early inflammatory lesion consists of dense inflammatory infiltrate with lymphocytes, plasma cells, scattered neutrophils, and numerous eosinophils. (B and C) These high-power views demonstrate the inflammatory infiltrate with numerous eosinophils on a background of chronic inflammatory cells.
270
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
Table 2 Summary of 28 reported cases of EAF Age (y)
Sex
Site of lesion
Management
Long-term follow-up
25 27 33 37 45 49 50 50 54 54 57 59 58 64 65 66 72 28 38 42 45 45 45 51 52 61 68 79
Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Male Male Male Male Male Male Male Male Male Male Male
Larynx Nasal septum/turbinate Larynx Nasal cavity/orbit Maxillary antrum/lamina papyracea Nasal septum Nasal cartilage Nasal cartilage Nasal septum Nasal septum/lateral wall Nasal cavity/orbit Nasal septum/lateral wall Nasal cavity Nasal septum/maxillary sinus Nasal septum Nasal septum Nasal septum Nasal septum/maxillary sinus Nasal septum/pyramid Nasal septum Nasal septum Nasal septum/pyramid Nasal septum/middle meatus Nasal septum Nasal septum Orbit Nasal septum/orbits Nasal septum/lateral wall
Surgical excision Surgical excision Surgical excision Surgical excision/steroids Surgical excision/steroids Surgical excision/steroids Surgical excision Surgical excision/steroids Surgical excision/radiotherapy Surgical excision Surgical excision/steroids Surgical excision/steroids Surgical excision Surgical excision/steroids Surgical excision Surgical excision No surgical excisiona Surgical excision/steroids Surgical excision Surgical excision Surgical excision No surgical excision/no therapy Surgical excision/steroids Surgical excision Surgical excision Steroidsa Surgical excision Surgical resection
Residual disease Residual disease Free of disease Residual disease Residual disease Residual disease Residual disease Residual disease Not available Residual disease Residual disease Residual disease Free of disease Residual disease Improved symptoms Not available Residual disease Residual disease Residual disease Not available Not available Residual disease Not available Improved symptoms Residual disease Residual disease Free of disease Not available
[5] [1] [1] [13] [11] [6] [9] [2] [2] [3] [13] [1] [13] [6] [18] [11] [12] [6] [15] [8] [16] [4] [10] [14] [5] [17] [13] [7] a
The patient declined surgical treatment.
In conclusion, EAF is a rare, benign condition that may be progressive and may cause local tissue destruction. The etiology and pathogenesis remain unknown. The diagnosis is based on specific pathohistologic findings.
References [1] 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. [2] Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: a postscript. Histopathology 1997;31: 385 - 6. [3] Altemani AM, Pilch BZ, Sakano E, Altemani JM. Eosinophilic angiocentric fibrosis of the nasal cavity. Mod Pathol 1997;10: 391 - 3. [4] Slovik Y, Putterman M, Nash M, Sion-Vardy N. Eosinophilic angiocentric fibrosis of the sinonasal tract in a male patient with chronic bowel inflammation. Am J Rhinol 2006;20:91 - 4. [5] Pereira EM, Millas I, Reis-Filho JS, Maeda SA, et al. Eosinophilic angiocentric fibrosis of the sinonasal tract: report on the clinicopathologic features of a case and review of the literature. Head Neck 2002;24:307 - 11. [6] Thompson LDR, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis. Am J Clin Pathol 2001;115:243 - 8. [7] Tabaee A, Zadeh MH, Proytcheva M, LaBruna A. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2003;117:410 - 3.
[8] 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 - 1. [9] Goldman NC. Angiocentric eosinophilic fibrosis. Otolaryngol Head Neck Surg 2003;128:445 - 6. [10] Nguyen DB, Alex JC, Calhoun B. Eosinophilic angiocentric fibrosis in a patient with nasal obstruction. Ear Nose Throat J 2004;83:183 - 6. [11] Yung A, Wachmuth R, Ramnath R. Eosinophilic angiocentric fibrosis—a rare mucosal variant of granuloma faciale which may present to the dermatologist. Br J Dermatol 2005;152:574 - 6. [12] Narayan J, Dougles-Jones AG. Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol 2005;119:35 - 42. [13] Paun S, Lund VJ, Gallimore A. Nasal fibrosis: long term follow up of four cases of eosinophilic angiocentric fibrosis. J Laryngol Otol 2005;119:119 - 24. [14] Matai V, Baer S, Barnes S, Boxer M. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2000;114:563 - 4. [15] Burns BV, Roberts PF, De Carpentier J, Zarod AP. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale. J Laryngol Otol 2001;115:223 - 6. [16] Onder A, Sungur A. Eosinophilic angiocentric fibrosis an unusual entity of the sinonasal tract. Arch Pathol Lab Med 2004;128:90 - 1. [17] Leibovitch I, Craig LJ, Wormald PJ. Orbital eosinophilic angiocentric fibrosis. A case report and review of the literature. Ophthal 2006;113:148 - 52. [18] Yavuzer R, Pandolfi PJ, Herschman BR, Jackson LT. An unusual cause of nasal airway obstruction: eosinophilic angiocentric fibrosis. Eur J Plast Surg 2000;23:49 - 51.
Annals of Diagnostic Pathology 12 (2008) 267 – 270
Eosinophilic angiocentric fibrosis of the sinonasal tract Ognjen Kosarac, MDa, Mario A. Luna, MDb, Jae Y. Ro, MD, PhDa, Alberto G. Ayala, MDa,4 a
b
Department of Pathology, The Methodist Hospital, Houston, TX 77030, USA Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA
Abstract
Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion of the upper respiratory tract and orbit that occurs mainly in young to middle-aged women. The etiology of EAF is unknown. To our knowledge, approximately 28 cases have been previously reported in the English literature. We report here 3 additional cases of EAF of the sinonasal tract; 2 in women aged 19 and 31 years, and 1 in a man aged 49 years. The 19-year-old woman is the youngest patient with EAF ever described. The patients presented with a nasal cavity mass, face pain, or nasal obstructive symptoms of long duration. D 2008 Elsevier Inc. All rights reserved.
Keywords:
Eosinophilic angiocentric fibrosis; Nasal cavity; Onion-skin pattern
1. Introduction Eosinophilic angiocentric fibrosis (EAF) is a rare, benign, obstructive lesion affecting the respiratory mucosa of the nasal cavity, larynx, and orbit. Since its first description in 1985 by Roberts and McCann [1], approximately 28 cases of EAF have been described in the English literature. The specific histologic features include a perivascular inflammatory cell infiltration (mainly eosinophils), which is gradually replaced with progressive fibrosis (bonion-skinQ pattern) around small blood vessels [1-17]. The etiology of EAF is unknown, and diagnosis is based on specific histologic findings. We report 3 cases of EAF, including one of a 19-year-old woman, the youngest patient described so far with this disease. 2. Case reports The pertinent clinical history, therapy, and follow-up of the 3 patients with EAF of the sinonasal tract are summarized in Table 1. 2.1. Case 1 A 19-year-old woman presented with recurrent right nasal congestion and face pain. Computed tomography 4 Corresponding author. Tel.: +1 713 441 1339; fax: +1 713 793 1603. E-mail address: [email protected] (A.G. Ayala). 1092-9134/$ – see front matter D 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.anndiagpath.2007.02.002
showed a complete opacification of the right maxillary sinus, extending to the adjacent middle meatus. Right endoscopic sinus surgery was performed, and a large fibrotic mass measuring 4 3 cm was found within the right maxillary sinus. 2.2. Case 2 A 31-year-old woman presented with nasal obstruction and a mass involving the right side of the nasal cavity at 5 years evolution. A physical examination showed a flat tumor forming a plaque measuring 3 2 cm. 2.3. Case 3 A 49-year-old man presented with nasal obstruction and a mass in the nasal septum at 3 years evolution. A physical examination showed a plaque in the nasal septum measuring 2 1.6 cm. 3. Pathologic findings The histologic appearance of the EAF was almost identical for all 3 cases. The normal architecture of the maxillary sinus (case 1), the right side of the nasal cavity (case 2), and the nasal septum (case 3) were completely distorted by fibrous-collagenous proliferation with a variable dense inflammatory infiltrate. The fibrous component displayed a concentrically layered onion-skin–type perivascular arrangement (Fig. 1A, B, and C), which is character-
268
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
Table 1 Summary of 3 new cases of EAF Age (y)
Sex
Clinical history
Site of lesion
Management
Follow up
19 31 49
Female Female Male
Nasal congestion and face pain Nasal obstruction Nasal obstruction
Right maxillary sinus Right nasal cavity Nasal septum
Surgical excision Surgical excision Surgical excision
Free of disease—1 year Free of disease—7 years Free of disease—12 years
istic of EAF. The inflammatory infiltrate consisted of lymphocytes, plasma cells, scattered neutrophils, and numerous eosinophils (Fig. 2A, B, and C). Other areas of the lesion demonstrated dense fibrosis with scanty inflammatory infiltrate. There were no granulomas, foreign-body giant cells, or fibrinoid necroses. 4. Treatment and follow-up The lesions were completely curettaged, and the patients were free of disease at 1, 7, and 12 years, respectively (see Table 1). 5. Discussion Although most of the 28 reported cases of EAF occurred in the nasal cavity (25 cases), including 3 cases with extension into the orbit, 2 cases occurred in the larynx, and 1 case occurred in the orbit (Table 2). Patient ages ranged from 25 to 79 years (median, 50.5 years), with a slight female preponderance (female-male ratio, 1.5/1). Eosinophilic angiocentric fibrosis is a slowly progressing disease that can exist for more than 4 years before it is diagnosed [1-3,6]. The etiology of EAF remains unknown. Some researchers have suggested that the association of EAF with allergic and atopic disorders and the predominance of an eosinophilic infiltrate represent an abnormal inflammatory response to a nonspecific stimulus in predisposed individuals [7,10]. Trauma may play a role in this condition because most patients had a history of nasal surgery or trauma to the nose before the onset of EAF [1,2,4,5,14,15]. Eosinophilic angiocentric fibrosis is also considered the mucosal variant of granuloma faciale because its histology is similar to that of this condition and because it has a rare association with granuloma faciale [1,12,15,16]. Symptoms are nonspecific and include nasal obstruction, nasal discharge, epistaxis, sinusitis, pain, and a mass [1-6,11]. A recent patient with orbital involvement presented with periorbital edema and painless proptosis [17]. The imaging findings are usually nonspecific and include clouding and opacification of the sinuses, bony sclerosis, and focal destruction of the surrounding sinusoidal or nasal cavity bony tissue [6,17]. Histologically, EAF covers the spectrum of lesions from an early inflammatory lesion to a late fibrotic lesion. The early lesion consists of an eosinophilic vasculitis without fibrinoid necrosis involving small blood vessels (capillaries and/or venules). The eosinophilic infiltrate predominates but
is invariably accompanied by plasma cells and lymphocytes. Furthermore, a granulomatous-type picture may be present but without true granulomas consisting of epithelioid cells or multinucleated giant cells. In contrast, the late lesion shows a dense fibrous thickening of the subepithelial stroma with obliterative perivascular onion-skin whorling of collagen and reticulin fibers. The inflammatory infiltrate is scanty, but eosinophils remain in the lesion [1,13]. The differential diagnosis of EAF includes infectiousinflammatory processes, including granulomatous diseases, Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, angiolymphoid hyperplasia with eosinophilia, erythema elevatum diutitum, inflammatory pseudotumor, and granuloma faciale. It may rarely simulate neoplastic (neurogenic, vascular, mesenchymal tumors) conditions. An initial approach to the differential diagnosis is to exclude some of these conditions by usual laboratory diagnostics tests, such as antineutrophil cytoplasmic antibodies, erythrocyte sedimentation rate, immunoglobulin E serum titers, and central and peripheral eosinophilic counts [5]. Nasal disease affects the mucosa of the lateral wall and septum, and this may help to clinically differentiate EAF from midline granulomatous diseases [13,15]. In addition, the absence of granulomas, negative staining with special stains (Gomori methenamine silver, acid-fast bacilli), and the characteristic whorling and onion-skin pattern of perivascular fibrosis strongly favor EAF. The limited form of Wegener granulomatosis, which is restricted to the nose and upper respiratory tract, should be considered in the differential diagnosis [8]. However, Wegener granulomatosis contains substantial necrobiosis, vasculitis, and scattered foreign-body–type giant cells. Churg-Strauss syndrome has fibrinoid necrosis and usually contains granulomas [6]. It is not difficult to rule out Kimura disease, a rare lesion in the Western hemisphere, which shows a mixed inflammatory infiltrate with a prominent fibrosis but without onion-skin perivascular fibrosis [5,6]. Angiolymphoid hyperplasia with eosinophilia is a lesion of the skin of the head and neck that characteristically displays eosinophilic infiltrate and should be differentiated from EAF. Furthermore, in the hyperplastic stage of this disease, there is a conspicuous vascular proliferation with prominent epithelioid endothelial cells that are not seen in typical cases of EAF [3,5,6]. Granuloma faciale is a rare inflammatory condition that affects the skin and may coexist with EAF [9,12,15]. Although some researchers consider EAF to be an extracutaneous lesion of granuloma faciale [11,12,15], others believe it is a separate clinical and histologic entity [7].
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
269
ically (steroids, dapsone, azathioprine) have been unsuccessful. As such, surgical resection appears to be the treatment of choice. However, recurrences are common, requiring multiple surgeries [5,6,11,13,15,17].
Fig. 1. (A) Low-power view of EAF. Note numerous fibrotic nodules consisting of concentrically layered perivascular fibrosis and no vasculitis (hematoxylin-eosin, 200). (B) At medium power, there are several nodules with onion-skin–type perivascular fibrosis. At this stage, the blood vessel lumen is obliterated. (C) In contrast to the previous illustration, this high-power view of the perivascular fibrosis does contain a blood vessel.
The treatment of choice for a patient with EAF remains unclear. Attempts at decreasing the inflammatory response both locally (nasal and intranasal steroids) and systemat-
Fig. 2. (A) The early inflammatory lesion consists of dense inflammatory infiltrate with lymphocytes, plasma cells, scattered neutrophils, and numerous eosinophils. (B and C) These high-power views demonstrate the inflammatory infiltrate with numerous eosinophils on a background of chronic inflammatory cells.
270
O. Kosarac et al. / Annals of Diagnostic Pathology 12 (2008) 267 – 270
Table 2 Summary of 28 reported cases of EAF Age (y)
Sex
Site of lesion
Management
Long-term follow-up
25 27 33 37 45 49 50 50 54 54 57 59 58 64 65 66 72 28 38 42 45 45 45 51 52 61 68 79
Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Female Male Male Male Male Male Male Male Male Male Male Male
Larynx Nasal septum/turbinate Larynx Nasal cavity/orbit Maxillary antrum/lamina papyracea Nasal septum Nasal cartilage Nasal cartilage Nasal septum Nasal septum/lateral wall Nasal cavity/orbit Nasal septum/lateral wall Nasal cavity Nasal septum/maxillary sinus Nasal septum Nasal septum Nasal septum Nasal septum/maxillary sinus Nasal septum/pyramid Nasal septum Nasal septum Nasal septum/pyramid Nasal septum/middle meatus Nasal septum Nasal septum Orbit Nasal septum/orbits Nasal septum/lateral wall
Surgical excision Surgical excision Surgical excision Surgical excision/steroids Surgical excision/steroids Surgical excision/steroids Surgical excision Surgical excision/steroids Surgical excision/radiotherapy Surgical excision Surgical excision/steroids Surgical excision/steroids Surgical excision Surgical excision/steroids Surgical excision Surgical excision No surgical excisiona Surgical excision/steroids Surgical excision Surgical excision Surgical excision No surgical excision/no therapy Surgical excision/steroids Surgical excision Surgical excision Steroidsa Surgical excision Surgical resection
Residual disease Residual disease Free of disease Residual disease Residual disease Residual disease Residual disease Residual disease Not available Residual disease Residual disease Residual disease Free of disease Residual disease Improved symptoms Not available Residual disease Residual disease Residual disease Not available Not available Residual disease Not available Improved symptoms Residual disease Residual disease Free of disease Not available
[5] [1] [1] [13] [11] [6] [9] [2] [2] [3] [13] [1] [13] [6] [18] [11] [12] [6] [15] [8] [16] [4] [10] [14] [5] [17] [13] [7] a
The patient declined surgical treatment.
In conclusion, EAF is a rare, benign condition that may be progressive and may cause local tissue destruction. The etiology and pathogenesis remain unknown. The diagnosis is based on specific pathohistologic findings.
References [1] 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. [2] Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: a postscript. Histopathology 1997;31: 385 - 6. [3] Altemani AM, Pilch BZ, Sakano E, Altemani JM. Eosinophilic angiocentric fibrosis of the nasal cavity. Mod Pathol 1997;10: 391 - 3. [4] Slovik Y, Putterman M, Nash M, Sion-Vardy N. Eosinophilic angiocentric fibrosis of the sinonasal tract in a male patient with chronic bowel inflammation. Am J Rhinol 2006;20:91 - 4. [5] Pereira EM, Millas I, Reis-Filho JS, Maeda SA, et al. Eosinophilic angiocentric fibrosis of the sinonasal tract: report on the clinicopathologic features of a case and review of the literature. Head Neck 2002;24:307 - 11. [6] Thompson LDR, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis. Am J Clin Pathol 2001;115:243 - 8. [7] Tabaee A, Zadeh MH, Proytcheva M, LaBruna A. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2003;117:410 - 3.
[8] 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 - 1. [9] Goldman NC. Angiocentric eosinophilic fibrosis. Otolaryngol Head Neck Surg 2003;128:445 - 6. [10] Nguyen DB, Alex JC, Calhoun B. Eosinophilic angiocentric fibrosis in a patient with nasal obstruction. Ear Nose Throat J 2004;83:183 - 6. [11] Yung A, Wachmuth R, Ramnath R. Eosinophilic angiocentric fibrosis—a rare mucosal variant of granuloma faciale which may present to the dermatologist. Br J Dermatol 2005;152:574 - 6. [12] Narayan J, Dougles-Jones AG. Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol 2005;119:35 - 42. [13] Paun S, Lund VJ, Gallimore A. Nasal fibrosis: long term follow up of four cases of eosinophilic angiocentric fibrosis. J Laryngol Otol 2005;119:119 - 24. [14] Matai V, Baer S, Barnes S, Boxer M. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2000;114:563 - 4. [15] Burns BV, Roberts PF, De Carpentier J, Zarod AP. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale. J Laryngol Otol 2001;115:223 - 6. [16] Onder A, Sungur A. Eosinophilic angiocentric fibrosis an unusual entity of the sinonasal tract. Arch Pathol Lab Med 2004;128:90 - 1. [17] Leibovitch I, Craig LJ, Wormald PJ. Orbital eosinophilic angiocentric fibrosis. A case report and review of the literature. Ophthal 2006;113:148 - 52. [18] Yavuzer R, Pandolfi PJ, Herschman BR, Jackson LT. An unusual cause of nasal airway obstruction: eosinophilic angiocentric fibrosis. Eur J Plast Surg 2000;23:49 - 51.