|Year : 2014 | Volume
| Issue : 3 | Page : 388-391
Periorbital epidermoid cyst in an elderly male
Sahana Ashok, Priya Sahni, Meghanand T Nayak
Department of Oral and Maxillofacial Pathology, Vyas Dental College and Hospital, Kudi Haud, Pali Road, Jodhpur, Rajasthan, India
|Date of Web Publication||18-Mar-2014|
Department of Oral and Maxillofacial Pathology, Vyas Dental College and Hospital, Kudi Haud, Pali Road, Jodhpur, Rajasthan
Source of Support: None, Conflict of Interest: None
Developmental cysts of the head and neck are rare, but are the most frequent cause of cystic masses of the head and neck. Of these, thyroglossal duct cysts are the most common, comprising about 70%. Branchial cleft cysts are the next most common, with most other lesions being very rarely encountered. The incidence of dermoid and epidermoid cysts in the head and neck region is 7%. This report describes a case of a dermoid cyst localized in the right lateral aspect of middle third of the face, showing signs of gradual enlargement and progression. Histologically, a diagnosis of epidermoid cyst was made and surgically treated with no evidence of recurrence.
Keywords: Dermoid cyst, developmental cyst, epidermoid cyst, germ layers
|How to cite this article:|
Ashok S, Sahni P, Nayak MT. Periorbital epidermoid cyst in an elderly male. Med J DY Patil Univ 2014;7:388-91
| Introduction|| |
Dermoid cysts of the head and neck region comprise only 7% of all body dermoid cysts  and account for 23-34% of all head and neck cysts.  According to the study of New and Erich, among 103 patients with head and neck dermoid and epidermoid cysts, the most common location was the orbit (46.6%), followed by the floor of the mouth and submental region (23.3%), nose (12.6%), neck (10.7%), and lip (2.9%). 
Epidermoid cysts are less common than dermoid cysts in the head and neck region.  Epidermoid cysts occur at any age from birth to 72 years; they usually become apparent in patients between 15 and 35 years of age.  There is no predominant sex predilection in the recent literature, regardless of some evidence which showed a male prevalence.  Common location sites are the orbit, calvarial diploic space, and intracranially.  Nearly 10% of the dermoid and epidermoid cysts of head and neck are localized in the orbital and periorbital region.  When present in the oral cavity, these cysts are seen in decreasing frequency in the following order: floor of mouth, tongue, sublingual, and submental. 
Dermoid and epidermoid cysts generally present as painless, asymptomatic, well-encapsulated, and slow-growing mass, which typically feel "dough like" on palpation.  Clinically, these cysts are divided into superficial or simple and deep or complicated cysts. Because of their superficial localization, simple cysts rarely develop complications and are easily handled surgically. It is not possible to differentiate between these cysts clinically. A histopathologic examination shows the difference between these two entities. 
In 1955, Meyer classified these cysts histologically as epidermoid (lined with simple squamous epithelium), dermoid (when skin adnexa are found in the cyst wall), or teratoid (when other tissues such as muscle, cartilage, and bone are present).  Although dermoid cysts represent a separate entity, the term "dermoid" is typically used to indicate all three categories clinically. 
Epidermoid cysts are rare, slow-growing, benign, developmental cysts that are derived from aberrant ectodermal tissue. An epidermoid cyst is defined as "a simple cyst lined with stratified squamous epithelium, and the lumen is filled with cystic fluid or keratin and no other specialized structure." 
In this paper, we describe a case of a dermoid cyst localized in the right lateral aspect of middle third of the face, which was a gradually increasing swelling. Histopathologic examination represented features of an epidermoid cyst. The swelling was surgically excised.
| Case Report|| |
A 46-year-old male patient presented to the outpatient department with a solitary swelling on the right lateral aspect of cheek. The swelling gradually increased in size over a period of two decades and the present size was a cause of cosmetic concern to the patient.
He was moderately built, conscious, and cooperative. On maxillofacial examination, a well-defined, painless, solitary swelling measuring 3 × 3 cm in size, extending 5 mm from the lateral border of orbital rim and 1 cm from the tragus of ear toward cheek was present. The swelling was non-pulsatile and spherical in shape having normal overlying skin with indistinct edges [Figure 1]. On palpation, the surface of the swelling was smooth, non-tender, compressible, non-reducible, and soft in consistency with diffuse borders, and was freely movable. On the basis of clinical presentation, a provisional diagnosis of dermoid cyst with a differential diagnosis of lipoma was given.
All hematological investigations were within normal limits. Surgical excision was planned under local anesthesia. Blunt dissection was carried out around the lesion and cystic enucleation was done [Figure 2]a. After the excision of the cyst [Figure 2]b, the wound was surgically sutured with adequate hemostasis. Postoperative course was uneventful without any inflammatory signs and with aesthetic results.
The resected specimen was sent for histopathologic examination. The macroscopic features revealed one piece of soft tissue mass which was soft to firm in consistency, brown in color, and with dimensions of 3 × 2.5 × 1.2 cm. A small portion of the gross specimen showed skin attached to it [Figure 3]a. On cut section, the lumen of the cystic mass contained cheesy white material [Figure 3]b. After clearing the contents of the lumen, a wet cotton pellet was placed in the lumen and tightened with a thread to maintain its patency during tissue processing.
Histopathologic examination with hematoxylin and eosin stain revealed cyst lined by hyperorthokeratinized stratified squamous epithelium supported by a fibrous connective tissue wall with few vascular spaces [Figure 4]a. Abundant amount of keratin was seen within the cyst lumen [Figure 4]b. On the basis of these features, the final diagnosis of epidermoid cyst was made.
| Discussion|| |
Dermoid cysts usually result from dysontogenic (defective embryonic development) anomaly.  These cysts may be classified into two major categories based on their origin as congenital and acquired, although there is no disparity between the two clinically or histologically.  Three theories have been proposed with regard to the origin of these cysts. The first and most prevalent theory states the pathogenesis for congenital dermoid cysts. These cysts originate from embryonic cells of the 1 st and 2 nd branchial arches entrapped in the mesenchyme of that region during the 3 rd or 4 th week of embryonic life. The second theory explains the pathogenic mechanism of acquired dermoid cysts. The acquired cysts may be due to the implantation of epithelial cells subsequent to accidental or surgical injury (traumatic causes, iatrogenic antecedents, or an occlusion of a sebaceous gland duct). The third theory states that these cysts are considered a variation of the cyst of the thyroglossal anomaly. 
One misconception is that an epidermoid cyst contains tissues derived from one of the three embryonic germ layers (ectoderm), a dermoid cyst has tissues from two germ layers (ectoderm and mesoderm), and a teratoma has tissues from all three germ layers (ectoderm, mesoderm, and endoderm). The most important point is that a teratoma is not necessarily composed of tissues derived from all the three germ layers [Table 1]. Instead, a teratoma can be defined as a true neoplasm that contains tissues that either are foreign to the primary site of origin or are histologically diverse and represent derivatives of more than one of the embryonic germ layers. The designation of teratoma can be appropriate even for a lesion with the tissues derived from only a single embryonic germ layer, if the tumor shows histologically divergent differentiation. 
Although these cysts may be present at birth and in elderly patients, the majority occur in the age range of 15-45 years. There is a report showing male:female ratio of 3:1, but few authors have reported no sex predilection. Dermoid and epidermoid cysts are usually present in the midline of the head and neck region. When present in the oral cavity, they are most commonly seen in the floor of mouth and extraorally in the periorbital region.  The present case was of a 46-year-old male patient presenting with a periorbital epidermoid cyst. All these features were in accordance with previous reports.
The differential diagnoses for dermoid cysts should include developmental lesions, congenital, salivary gland lesions, and benign tumors [Table 2]. Needless to say, in all cases, the confirmative diagnosis is given after histopathologic examination. 
There is always a difficulty in making a correct diagnosis of these cysts with clinical examinations and conventional radiography. To achieve a diagnosis and to develop correct surgical strategy, specialized imaging techniques such as ultrasonography, computed tomography, magnetic resonance imaging, and fine-needle aspiration should be carried out. 
The basic treatment for epidermoid cysts is total excision, but Ronald  suggests that the contents of extremely large cysts should be reduced via fine-needle aspiration and then the excision should be performed. Ochiai et al.  reported that such palliative treatments may lead to infection, which potentially causes pain and exacerbation. Care should be taken not to rupture the cyst, as the cystic contents may act as irritants to fibrovascular tissues, causing postoperative inflammation. 
Prognosis of these cysts is good with rare recurrence.  But a 5% rate of malignant transformation of dermoid cysts, particularly of teratoid type, to squamous carcinoma and basal cell carcinoma have been reported. , Patients are often elderly and present with advanced disease. Prognosis of such cases tends to be poor. 
| Conclusion|| |
Epidermoid cysts of the head and neck region are quite rare and need to be differentially diagnosed from several other diseases and conditions of the area. For diagnosis, a detailed clinical examination of their size and anatomical location is important. Furthermore, valuable assistance is provided by the imaging techniques. Surgical enucleation provides a good treatment with excellent prognosis. The final confirmatory diagnosis of the disease is always through histopathologic examination.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]