Atypical eosinophilic angiocentric fibrosis on nasal septum

Atypical eosinophilic angiocentric fibrosis on nasal septum

Auris Nasus Larynx 33 (2006) 355–358 www.elsevier.com/locate/anl Atypical eosinophilic angiocentric fibrosis on nasal septum Noriko Watanabe *, Kazuh...

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Auris Nasus Larynx 33 (2006) 355–358 www.elsevier.com/locate/anl

Atypical eosinophilic angiocentric fibrosis on nasal septum Noriko Watanabe *, Kazuhiro Moriwaki Department of Otorhinolaryngology, Tondabayashi Hospital, 1-3-36, Koyodai, Tondabayashi, Osaka 584-0082, Japan. Received 24 October 2005; accepted 13 January 2006 Available online 10 March 2006

Abstract Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology and is most commonly found in the nasal septum and sinus mucosa. We report a case of EAF and present a review of the available literature. A 51-year-old man with progressive nasal obstruction was referred to our hospital. CT and MRI scans revealed a mass on the nasal septum; this was surgically excised. Histological analysis of the resected tumour showed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix and absence of eosinophilic vasculitis and onion skinning. Histologically, it resembled granuloma faciale. However, our case was considered to be an EAF although eosinophilic vasculitis and onion skinning were not observed. This was because a cutaneous lesion was absent and the lesion was limited to the nasal septal mucosa. # 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Eosinophilic vasculitis; Granuloma faciale; Onion skinning; Fibrosis

1. Introduction Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology. It was first described by Roberts and McCann [1], and 22 cases have been reported in literature since then [1–16]. EAF typically presents as a slowly progressive upper airway obstruction in association with a submucosal inflammatory, fibrosing and tumour-like lesion. We report a case of EAF with atypical features and review the 22 previously reported cases. 2. Case report A 51-year-old man noticed a bilateral nasal obstruction in January 2002. He visited an ENT clinic in his neighbourhood and was suspected of having a nasal septal tumour; he was referred to our hospital on 25 July 2003 for further treatment. Anterior rhinoscopy revealed a bilateral mass with a smooth surface on the nasal septum, and the mass protruded into both nasal cavities. The patient had no other * Corresponding author. Tel.: +81 721 29 1121; fax: +81 721 28 3550. E-mail address: [email protected] (N. Watanabe).

otolaryngological abnormalities or facial cutaneous disorders. All laboratory tests, including complete blood counts, eosinophil counts, C-reactive protein (CRP), antineutrophilic cytoplasmic antibodies (ANCA), IgE serum levels and antinuclear antibodies, were unremarkable. In addition, a radioimmunoenzymatic assay (RAST test) to detect specific IgE antibodies, including IgE against various common fungal antigens, was negative. Chest X-rays did not reveal any abnormalities. CT and MRI scans showed a wellcircumscribed, symmetrical mass on the nasal septum. T1and T2-weighted MRI showed a low-intensity mass on the nasal septum, and following gadolinium administration, the mass was only slightly enhanced on the T1-weighted images (Fig. 1). All paranasal sinuses were clear. The patient had no significant past medical history, including asthma, and no significant family history. On 18 September 2003, the tumour was resected under general anaesthesia. An incision was made on the midportion of the upper gingivobuccal sulcus. The tumour extended through the entire length of the nasal septal cartilage and from the nasal bone to the palatine bone. The lesion was localized to the nasal cavity. The nasal bone, palatine bone, bridge of the nose, vomer and base of the skull were intact. The tumour penetrated the nasal septal cartilage

0385-8146/$ – see front matter # 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2006.01.009

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Fig. 1. Sagittal T1-weighted MRI scan showing a low-intensity mass on the nasal septum.

and a portion of the perpendicular plate of the ethmoid bone and replaced these portions. A wide excision of the tumour and septonasal mucosa was performed, thereby yielding a surgical specimen measuring 20 mm  35 mm  60 mm. No cerebrospinal fluid leakage was observed after the surgery. Histological analysis of the resected tumour revealed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix and the absence of eosinophilic

Fig. 2. Histological analysis showing fibrosis and eosinophils but no vasculitis or onion skinning (haematoxylin and eosin; original magnification, 400).

Fig. 3. Postoperative coronal T1-weighted MRI scan showing no residual disease.

vasculitis and onion skinning (Fig. 2). Granulomas, foreign body giant cells, Langerhans cells and geographic necrosis were not detected. To date, the patient has had no evidence of recurrence (Fig. 3).

3. Discussion EAF is a rare condition of unknown aetiology that was first described by Roberts and McCann [1]. To our knowledge, 22 cases have been reported in literature since then. Of the 23 known cases (including our case), 21 involved the nose [1–16] and 2 involved the larynx. Twenty patients with nasal EAF experienced nasal stuffiness and obstruction. Our case is the 14th report of nasal septal EAF. The ages of the 21 patients with nasal EAF ranged from 27 to 79 years (mean, 48.6 years), with a male to female ratio of 11:10. Although initial case reports suggested a female predominance, a review of all the reported cases does not demonstrate an evident gender bias (Table 1). The pathogenesis of EAF is not well elucidated. Roberts and McCann [1] proposed an allergic aetiology because of the predominance of eosinophils in the pathological examination. However, only seven of the 23 patients with EAF had a history of allergy, and EAF has been found to be refractory to steroids, antiallergic agents and other topical agents. These data do not support an allergic aetiology. The treatment of choice for patients with EAF has not been determined [2,6]. Fageeh et al. [4] reported that EAF appears to respond only to the surgical excision of the involved

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Table 1 Literature summary of patients with nasal EAF

Ref., reference number; EV, eosinophilic vasculitis; OS, onion skinning; Blank, pathology not reported; PD, persistent disease; FD, free of disease.

segment. In most cases reported in literature as well as in our case, the patients were treated surgically. EAF can be diagnosed only by histological examination. Over the natural course of this disease, as fibrosis progresses, the inflammatory infiltration of the lymphoplasmacytic component becomes progressively scantier, but the eosinophils remain. The differential diagnosis of EAF includes Churg–Strauss syndrome, Wegener’s granulomatosis,

Kimura’s disease, eosinophilic granuloma and granuloma faciale (GF). Churg–Strauss syndrome is characterized by fibrinoid necrosis and generally contains extravascular granulomas with eosinophilia, and it is often associated with asthma and sinusitis. Wegener’s granulomatosis often involves the upper respiratory tract, lungs and kidneys, and contains geographic necrosis, vasculitis and foreign body giant cells. A blood test positive for c-ANCA can support a

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suspected diagnosis of Wegener’s granulomatosis. Kimura’s disease is often associated with peripheral blood eosinophilia, elevated ESR and serum IgE levels and shows the presence of dense lymphoid aggregates with prominent germinal centres and fibrous tissue. Eosinophilic granuloma is characterized by single or multiple skeletal lesions that show proliferation of foamy and vacuolated histiocytes. GF is characterized by a polymorphous infiltrate that predominantly comprises neutrophils and eosinophils, mainly in the dermis, and fibrosis is also present. The clinical features of our patient and the absence of any characteristic pathological findings ruled out Churg–Strauss syndrome, Wegener’s granulomatosis, Kimura’s disease, eosinophilic granuloma and GF. Eosinophilic vasculitis and onion skinning are also features of EAF. Eosinophilic vasculitis is a condition wherein the eosinophils cluster around and migrate through the vessel wall, accompanied by a variable number of plasma cells and lymphocytes in the perivascular space. Onion skinning consists of dense concentric rings of collagen accumulating around the capillaries and venules. Some case reports have mentioned the presence of both features (eosinophilic vasculitis and onion skinning), while others have mentioned only one feature [5,6]. We observed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix although eosinophilic vasculitis or onion skinning was not observed in all the areas. Although EAF and GF show pathological similarity, they are distinguished by the presence of the lesion in the submucosa or dermis and the detection of onion skinning. Six cases of mucosal lesion with onion skinning in the nose and cutaneous lesion without onion skinning on the face have been reported as cases with simultaneous occurrence of GF and EAF [1,7,10,11,15,16]. Roberts and McCann [1] claimed that the lesion was confined to the dermis in GF and to the submucosa in EAF, and both diseases were within the spectrum of the same entity. There is no consensus for considering EAF as a variant of GF. Most nasal septal tumour-like lesions that comprised an inflammatory infiltrate and fibrous matrix are accompanied by onion skinning or eosinophilic vasculitis. Onion skinning and eosinophilic vasculitis were not observed in our case; therefore, it resembled GF pathologically. However, we diagnosed it as EAF due to the absence of a cutaneous lesion. To date, our patient has not reported any recurrence of the disease. However, multiple recurrences have occurred in a majority of the published cases for which satisfactory follow-up information was available. EAF needs careful observation following surgery.

4. Conclusion We encountered a tumour-like lesion of the nasal septum. Histological analysis showed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix; histologically, it resembled GF. However, although eosinophilic vasculitis and onion skinning were not observed, our case was diagnosed as EAF although eosinophilic vasculitis and onion skinning were not observed. This was because a cutaneous lesion was absent and the lesion was limited to the nasal septal mucosa.

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