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Pathology Quiz Case 2: Diagnosis

Pathology Quiz Case 2: Diagnosis Diagnosis: Eosinophilic angiocentric fibrosis (EAF) Eosinophilic angiocentric fibrosis is a rare cause of nonneoplastic mucosal thickening of the upper respiratory tract that predominantly affects the nasal airway. In 1985, Roberts and McCann1described the condition as a mucosal variant of granuloma faciale. Since then, there have been fewer than 30 cases of EAF reported in the world literature. Twenty-five of the cases involved the sinonasal airway; 2 cases involved only the larynx; and 1 case involved the orbit and the ethmoidal sinus unilaterally. The etiology of EAF is unknown. Allergy, atopy, and trauma have all been suggested as predisposing factors, but the evidence for any of these origins is slim.2,3In the series of cases reported by Thompson and Heffner,4there was no history of allergy, trauma, previous surgery, self-medication, or known immunological disease. The patient may present with nonspecific symptoms such as nasal obstruction, epistaxis, nasal discharge, facial pain, or the presence of a mass in the nasal airway. The disease is slowly progressive, and the patient will often describe a worsening of symptoms over a number of years. The findings of clinical examination are nonspecific, with chronic inflammatory changes of the nasal mucosa and crusting. There may be an inflammatory mass in the nasal airway or diffuse inflammation, as in this case. Imaging, the findings of which are also nonspecific, serves only to identify the extent of disease process and to rule out other lesions. Eosinophilic angiocentric fibrosis is a histologic diagnosis. The absence of true granulomas is the characteristic that sets EAF apart from granulomatous disease of the sinonasal tract. The early features include a vasculitis without fibrinoid necrosis that is predominated by eosinophils (Figure 1). As the inflammation progresses, the number of eosinophils increases, with a corresponding decrease in the numbers of other inflammatory cells. The late lesion involves perivascular fibrosis that displays a whorling of collagen fibers in a characteristic onionskin pattern (Figure 2). Different “phases” in the evolution of EAF can be present in the same biopsy specimen. This finding is particularly relevant because the eosinophilic preponderance may not be as obvious in areas of tissue that contain the late fibrotic changes. Figure 1. View LargeDownload Figure 2. View LargeDownload The differential diagnosis includes inflammatory and granulomatous diseases of the sinonasal tract, including Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, and granuloma faciale.4Wegener granulomatosis may involve other anatomical sites, particularly the lungs and kidneys, and is characterized by a necrotizing vasculitis of medium-sized vessels with infiltration by neutrophils and multinucleated giant cells. Immunofluorescence studies to identify antineutrophilic cytoplasmic antibodies and further confirmatory enzyme-linked immunosorbent assay testing for antiproteinase 3 and antimyeloperoxidase are highly specific for Wegener granulomatosis. Churg-Strauss syndrome is characterized by a history of asthma and peripheral eosinophilia. Histologically, there is an eosinophilic infiltrate, but unlike EAF, there are also granulomas and a necrotizing vasculitis. Kimura disease is a chronic inflammatory disease that usually presents as painless lymphadenopathy or subcutaneous masses of the head and neck. Histologic examination shows lymphoid aggregates with prominent germinal centers and fibrosis that is unlike the angiocentric whorling pattern seen in EAF. The rich angiolymphoid hyperplasia with eosinophilia is not seen in EAF.4It is also important to rule out the presence of neoplasms with a fibroproliferative nature and histologic appearance, eg, schwannoma, angiofibroma, and fibrosarcoma. Because of the paucity of cases of EAF, it is difficult to make definitive conclusions regarding optimal management. However, immunosuppressant therapy, including oral corticosteroids, intralesional triamcinolone, and azathioprine, has not shown any significant benefit.5The disease runs an indolent self-limiting course, which is relatively unchanged once the EAF is fully developed. Surgical excision is the treatment of choice for the symptomatic patient but is often not curative owing to the indolent natural history and multifocality of the disease. Box Section Ref ID Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters to the Archivescommenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (http://archotol.ama-assn.org/misc/ifora.dtl). Material for CLINICALPROBLEMSOLVING: PATHOLOGYshould be submitted electronically via the online submission and review system at http://manuscripts.archoto.com. Reprints are not available from the authors. Return to Quiz Case 2. References 1. Roberts PFMcCann BG Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? a report of three cases. Histopathology 1985;9 (11) 1217- 1225PubMedGoogle ScholarCrossref 2. Tabaee AZadeh MHProytcheva MLaBruna A Eosinophilic angiocentric fibrosis. J Laryngol Otol 2003;117 (5) 410- 413PubMedGoogle ScholarCrossref 3. Nguyen DBAlex JCCalhoun B Eosinophilic angiocentric fibrosis in a patient with nasal obstruction. Ear Nose Throat J 2004;83 (3) 183- 184, 186PubMedGoogle Scholar 4. Thompson LDHeffner DK Sinonasal tract eosinophilic angiocentric fibrosis: a report of three cases. Am J Clin Pathol 2001;115 (2) 243- 248PubMedGoogle ScholarCrossref 5. Watanabe NMoriwaki K Atypical eosinophilic angiocentric fibrosis on nasal septum. Auris Nasus Larynx 2006;33 (3) 355- 358PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Pathology Quiz Case 2: Diagnosis

Archives of Otolaryngology - Head & Neck Surgery , Volume 135 (7) – Jul 1, 2009

Pathology Quiz Case 2: Diagnosis

Abstract

Diagnosis: Eosinophilic angiocentric fibrosis (EAF) Eosinophilic angiocentric fibrosis is a rare cause of nonneoplastic mucosal thickening of the upper respiratory tract that predominantly affects the nasal airway. In 1985, Roberts and McCann1described the condition as a mucosal variant of granuloma faciale. Since then, there have been fewer than 30 cases of EAF reported in the world literature. Twenty-five of the cases involved the sinonasal airway; 2 cases involved only the larynx; and 1...
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Publisher
American Medical Association
Copyright
Copyright © 2009 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archoto.2009.63-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Eosinophilic angiocentric fibrosis (EAF) Eosinophilic angiocentric fibrosis is a rare cause of nonneoplastic mucosal thickening of the upper respiratory tract that predominantly affects the nasal airway. In 1985, Roberts and McCann1described the condition as a mucosal variant of granuloma faciale. Since then, there have been fewer than 30 cases of EAF reported in the world literature. Twenty-five of the cases involved the sinonasal airway; 2 cases involved only the larynx; and 1 case involved the orbit and the ethmoidal sinus unilaterally. The etiology of EAF is unknown. Allergy, atopy, and trauma have all been suggested as predisposing factors, but the evidence for any of these origins is slim.2,3In the series of cases reported by Thompson and Heffner,4there was no history of allergy, trauma, previous surgery, self-medication, or known immunological disease. The patient may present with nonspecific symptoms such as nasal obstruction, epistaxis, nasal discharge, facial pain, or the presence of a mass in the nasal airway. The disease is slowly progressive, and the patient will often describe a worsening of symptoms over a number of years. The findings of clinical examination are nonspecific, with chronic inflammatory changes of the nasal mucosa and crusting. There may be an inflammatory mass in the nasal airway or diffuse inflammation, as in this case. Imaging, the findings of which are also nonspecific, serves only to identify the extent of disease process and to rule out other lesions. Eosinophilic angiocentric fibrosis is a histologic diagnosis. The absence of true granulomas is the characteristic that sets EAF apart from granulomatous disease of the sinonasal tract. The early features include a vasculitis without fibrinoid necrosis that is predominated by eosinophils (Figure 1). As the inflammation progresses, the number of eosinophils increases, with a corresponding decrease in the numbers of other inflammatory cells. The late lesion involves perivascular fibrosis that displays a whorling of collagen fibers in a characteristic onionskin pattern (Figure 2). Different “phases” in the evolution of EAF can be present in the same biopsy specimen. This finding is particularly relevant because the eosinophilic preponderance may not be as obvious in areas of tissue that contain the late fibrotic changes. Figure 1. View LargeDownload Figure 2. View LargeDownload The differential diagnosis includes inflammatory and granulomatous diseases of the sinonasal tract, including Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, and granuloma faciale.4Wegener granulomatosis may involve other anatomical sites, particularly the lungs and kidneys, and is characterized by a necrotizing vasculitis of medium-sized vessels with infiltration by neutrophils and multinucleated giant cells. Immunofluorescence studies to identify antineutrophilic cytoplasmic antibodies and further confirmatory enzyme-linked immunosorbent assay testing for antiproteinase 3 and antimyeloperoxidase are highly specific for Wegener granulomatosis. Churg-Strauss syndrome is characterized by a history of asthma and peripheral eosinophilia. Histologically, there is an eosinophilic infiltrate, but unlike EAF, there are also granulomas and a necrotizing vasculitis. Kimura disease is a chronic inflammatory disease that usually presents as painless lymphadenopathy or subcutaneous masses of the head and neck. Histologic examination shows lymphoid aggregates with prominent germinal centers and fibrosis that is unlike the angiocentric whorling pattern seen in EAF. The rich angiolymphoid hyperplasia with eosinophilia is not seen in EAF.4It is also important to rule out the presence of neoplasms with a fibroproliferative nature and histologic appearance, eg, schwannoma, angiofibroma, and fibrosarcoma. Because of the paucity of cases of EAF, it is difficult to make definitive conclusions regarding optimal management. However, immunosuppressant therapy, including oral corticosteroids, intralesional triamcinolone, and azathioprine, has not shown any significant benefit.5The disease runs an indolent self-limiting course, which is relatively unchanged once the EAF is fully developed. Surgical excision is the treatment of choice for the symptomatic patient but is often not curative owing to the indolent natural history and multifocality of the disease. Box Section Ref ID Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters to the Archivescommenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (http://archotol.ama-assn.org/misc/ifora.dtl). Material for CLINICALPROBLEMSOLVING: PATHOLOGYshould be submitted electronically via the online submission and review system at http://manuscripts.archoto.com. Reprints are not available from the authors. Return to Quiz Case 2. References 1. Roberts PFMcCann BG Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? a report of three cases. Histopathology 1985;9 (11) 1217- 1225PubMedGoogle ScholarCrossref 2. Tabaee AZadeh MHProytcheva MLaBruna A Eosinophilic angiocentric fibrosis. J Laryngol Otol 2003;117 (5) 410- 413PubMedGoogle ScholarCrossref 3. Nguyen DBAlex JCCalhoun B Eosinophilic angiocentric fibrosis in a patient with nasal obstruction. Ear Nose Throat J 2004;83 (3) 183- 184, 186PubMedGoogle Scholar 4. Thompson LDHeffner DK Sinonasal tract eosinophilic angiocentric fibrosis: a report of three cases. Am J Clin Pathol 2001;115 (2) 243- 248PubMedGoogle ScholarCrossref 5. Watanabe NMoriwaki K Atypical eosinophilic angiocentric fibrosis on nasal septum. Auris Nasus Larynx 2006;33 (3) 355- 358PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Jul 1, 2009

Keywords: fibrosis

References

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