Angiolymphoid hyperplasia with eosinophilia of the maxillary sinus and orbit

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an inflammatory disorder characterized by benign vasoproliferative lesions. Although these lesions are typically found around the ear and external auditory canal, there are several studies that present cases with ocular involvement. In this report we present the first case of ALHE with ocular and sinus involvement in Turkish literature.


INTRODUCTION
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign disorder that presents with subdermal nodules that show an affinity for the head and neck region with the typical lesion involving the external auditory canal.Although previously frequently used as a synonym with Kimura's disease, today the two are generally accepted to be two different diseases.ALHE shows a definite predilection for the external ausitory canal, it may present in the maxillary sinus, ethmoid sinus and orbita.Removal of these lesions often results in recurrence and the need for secondary surgery.In this report we present an unusual case of both maxillary and orbital involvement in a male patient with ALHE.

CASE REPORT
A 31-year old Caucasian male presented with right-sided eye swelling, diplopia, limitation of eye movements and a feeling of fullness in his right cheek area.His physical examination revealed restricted right globe movements and proptosis.Optical evaluation showed diminished sight.
MRI showed a lobulated, well circumscribed lesion, measuring 25 x 38 x 39 mm in the right inferior orbita, displacing the globe and other orbital structures anteromedially and superiorly.Invasion findings were present in the neighboring inferior, medial and lateral walls of the orbita, inferior rectus muscle, roof of the maxillary sinus and the lesion seemed to extend into the right middle ethmoid cells and superior part of the right maxillary sinus (Figure 1).A CT scan was obtained and showed a lesion arising from the right maxillary bone wıth extension into the right orbital cavity.The floor, medial and posterior walls of the orbital bone were eroded but there was no evidence of globe involvement.The lesion also had extension in to the nasal cavity (Figure 2).A CBC showed minimally elevated leukocyte levels yet the eosinophil count was normal.A biopsy from the maxillary extension of the lesion was performed; the histological evaluation showed a spindle cell tumor with epiteloid cells later designated as an epiteloid hemangioendothelioma.A second operation for total excision was performed however, due to the lesions localization, a small residual tumor was left to preserve eyesight.The microscopic assessment was reported as angiolymphoid hyperplasia with eosinophilia.

DISCUSSION
Although Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura disease have very similar clinical and histopathological properties they represent two different disease processes.The two terms have been used interchangeably for the similar clinical spectrum in the previous literature (1) the two entities both show a predilection for the head and neck, frequently recur after treatment, and most often present as subcutaneous masses with vascular proliferation and eosinophilia.However after a study by Rosai et al. (2), the two processes were divided into two distinct diseases.
Angiolymphoid hyperplasia with eosinophilia (ALHE) also termed epithelioid hemagioma is a benign, rare disorder which presents with pink to red-brown, dome shaped, dermal or subdermal papules or nodules of the head and neck with a predilection for the ear, especially the external auditory canal (3).ALHE is accepted to be a benign vasoproliferative formation with inflammation, however there is no consensus regarding the causative predisposer for the development of this disease although trauma, low grade infections, allergy and pregnancy have all been implicated (4).The pathogenesis is believed to begin with the formation of an arteriovenous malformation (5).Microscopic evaluation of these nodules shows a proliferation of vessels surrounded by a lympho-eosinophilic predominant infiltrate.The vessels have an endothelial covering which appears to be like an epithelioid and some may produce epithelioid endothelium, causing obstruction of these vessels.The treatment for epithelioid hemangiomas is surgical.Spontaneous regression is still a matter of debate although a consensus has been reached stating that recurrence is common.
The typical presentation of ALHE is a middle-aged Caucasian woman with multiple subdermal or dermal, matted red lesions, predominantly located in the head and neck region.The behavior of lesions differ and show a range; from asymptomatic bumps to pruritic and painful swellings (6).Lesions are occasionally reported in different parts with the orbital tissues, parotid, chest and lingual mucosa being some of the areas presented (4,7,8,9).Orbital involvement of ALHE is very rarely bilateral and is generally treated with surgery.Be it de-bulking or complete removal, several studies have shown that the lesions involute more rapidly when the lesion is excised (8,10,11).Other treatment modalities initiated in the treatment of ALHE are intralesional steriods, cryotherapy, cytotoxic drug injections, phototherapy, radiation therapy and alpha-2a interferon therapy (12).Kimura's disease is described as a chronic inflammatory disease that presents as deep, subcutaneous masses in the head and neck, especially around the ear and on the scalp.Involvement of salivary glands and lymph nodes is frequently seen and blood analysis shows increased levels of both immunoglobulin E (IgE) and peripheral blood eosinophilia (13).Microscopy shows lymphoid tissue with reactive follicular hyperplasia with deposits of eosinophilic proteinaceous  material in the germinal center, hence the appearance of a granuloma.Eosinophilia and elevated IgE levels are characteristic of KD and it has been postulated that the level of eosinophilia may be correlated with the size of the lesion (14).KD has also been linked with allergic diseases such as asthma, eczema and rhinitis.The stereotypic KD patient an Asian, young male with a single subdermal lesion, accompanying lymphadenopathy with elevated levels of blood eosinophils and IgE (15) .Although there is a small chance of spontaneous involution making it a viable option to observe asymptomatic cases, the treatment of choice is surgery in symptomatic cases (16).KD has a higher recurrence rate than ALHE and for this reason; incomplete removal of the lesion is generally not an accepted method of treatment.

CONCLUSION
Kimura's disease and ALHE are two different pathologies with very similar appearances.Where ALHE is considered a benign proliferative disorder, KD is accepted as a chronic allergic process.The differences in age, race and sex may give clues as to which disease is being dealt with but definitive categorization is done histochemically.Orbital involvement may be seen in both diseases and should be treated similarly to dermal lesions.Total excision is the preferred method but if not possible, to speed along the process of spontaneous regression, especially in the case of epitheloid hemangioma, de-bulking is the next best option.

Figure 1 :
Figure 1: a: Precontrast T1 weighted axial plane MR image depicts a well circumscribed lesion in the right orbita displacing the globe anteromedially.b: Postcontrast T1 weighted coronal plane MR image shows that the lesion enhances homogeneously.Globe is displaced superomedially and the lesion extends into the neighboring maxillary and middle ethmoid sinuses Figure 2: a: The lesion with erosion of the inferior orbital wall and extension into the nasal cavity.Note the deplacement of the inferior and lateral rectus muscles.b: The lesion extending posteriorly and eroding the posterior maxillary wall together with the posterolateral orbital floor

Figure 2 :
Figure 1: a: Precontrast T1 weighted axial plane MR image depicts a well circumscribed lesion in the right orbita displacing the globe anteromedially.b: Postcontrast T1 weighted coronal plane MR image shows that the lesion enhances homogeneously.Globe is displaced superomedially and the lesion extends into the neighboring maxillary and middle ethmoid sinuses Figure 2: a: The lesion with erosion of the inferior orbital wall and extension into the nasal cavity.Note the deplacement of the inferior and lateral rectus muscles.b: The lesion extending posteriorly and eroding the posterior maxillary wall together with the posterolateral orbital floor