A Rare Case of Epitheloid Hemangioma Cheek: Case Report

Abstract

Epithelioid hemangioma is a very rare lesion arising from vascular structures. We encountered a case in very atypical location i.e. on the anterior maxillary wall. The underwent excision of tumour twice elsewhere and presented in our hospital with reccurence of mass. Patient was managed in our hospital with good cosmetic outcome with no disease recurrence till date.

Introduction

Epithelioid hemangioma (EH), also termed as Angiolymphoid hyperplasia with eosinophilia, is a rare, benign, neoplastic, endothelial cell lesion. It is a vascular tumour recognized by WHO [1]. Although the etiology remains unclear, surgical excision with negative margins has proven to be the treatment of choice [2]. EH is seen in wide range, most common between 20–50 years of age [3]. The most common sites in head and neck are the forehead, preacuricular area and scalp [3]. We report a case in very atypical location, i.e. on the anterior maxillary wall. The patient underwent excision of tumour twice elsewhere and presented in our hospital with reccurence of mass. The EH was successfully excised in our hospital through sublabial approach.

Case Report

A 15 year old female was admitted with chief complaint of swelling over the right cheek. She was treated for same at local hospital where they excised 6 months ago. She then came to our hospital with recurrence of swelling. Physical examination showed 5 *5 cm swelling over the right cheek. Scar was present across the swelling. Firm to hard, non-tender well-defined swelling which was non reducible, not fixed to overlying skin and mucosa. No bruits were heard.

Mri scan showed soft tissue tumour in subcutanous plane.

USG examination of right cheek swelling was also performed which showed well defined solid iso echoic lesion of size 27*12 mm in right cheek deep to subcutanous plane overlying maxillary bone. No obvious _{destruction of underlying bone was noted.} Vascularity was noted along wall of the lesion. The patient underwent excision of mass in toto through the gingivo-buccal sulcus approach. Post operative the patient was followed upto 18 months and is symptom free till date.

Specimen was then sent of histopathological examination which showed well circumscribed, an encapsulated tumor proper. The tumor proper was composed of lobular proliferation of capillary sized vessels lined by epithelioid endothelial cells. In addition, there are solid sheets of oval to spindle shaped cells with mild nuclear pleomorphism.

Immunohistochemical studies was advised. IHC stain showed the presence of vascular proliferation markers CD31 + and CD34 + . In accordance with these findings, the lesion was diagnosed as epitheloid hemangioma.

Discussion

EH is an uncommon, benign, neoplastic lesion of vascular endothelium that usually involves the subcutaneous tissue of head and neck.

It is most commonly seen in forehead, preauricular region and scalp [3]. In our case it presented in cheek [4]. EH is a rare disease typically affecting skin and subcutis [5] but in our case it was attached to anterior wall of right maxilla. EH is witnessed more often among Asians and Caucasians and reported among patients aged 20–50 years [6] but in our case patient was 15 year old teenager. This specific condition is slightly more prevalent in females [6] and same is scenario in our case.

The central dogma of this lesion is that it is a benign tumor whose pathogenesis still remains unclear. Th reported literature that it may be a result of unusual reactive process secondary to an artery or vein damage following traumatic insults or hormonal imbalance [6].

Clinical diagnosis can be difficult as in most cases the only symptom is a progressive tender swelling. Skin discoloration may take place over the swelling [2] and in our case also there was discoloration over the swelling. Imaging can be useful for studying tumor extension, vascular involvement and any other simultaneous presentations, but histopathology and immunohistochemistry studies are mandatory in order to obtain a definitive diagnosis.

Histopathological picture of epithelioid haemangioma shows prominent proliferation of vessels lined by plump “epithelioid” endothelial cells with abundant eosinophilic cytoplasm and central oval nucleus. The distinctive endothelial cells have been described as having cobblestone appearance. The stroma of the tumor is formed by fibromyxoid tissue with inflammatory cells that surround the vessels. The inflammatory cell infiltrate is composed of eosinophils, plasma cells, lymphocytes, macrophages and mast cells our patient also tumour was composed of lobular proliferation of capillary sized vessels lined by epithelioid endothelial cells. In addition, there are solid sheets of oval to spindle shaped cells with mild nuclear pleomorphism.

The immunohistochemistry of epithelioid haemangioma with a vascular marker will show that these epithelioid cells are vascular in origin [2]. CD 31 and CD 35 are vascular markers [2] which were used in our studies and results were positive for these epithelioid cells.

The primary treatment for EH is surgical excision in all cases of EH [2].