CHAPTER 2

LITERATURE REVIEW

A. Basal Cell Carcinoma (BCC)

1. Definition
BCC is malignant neoplasm derived from nonkeratinizing cells originating
in the basal layer of the epidermis. Basal cell carcinoma was first described in 1824
by Jacob who called it "ulcus rodens"; its current nomenclature was proposed by
Krompecher in 1903.6 In 1900, Krompecher described BCC as a malignant, locally
invasive, and destructive cancer and named it Carcinoma epitheliale adenoides;
he then went on to pioneer the classification of skin tumors using histogenetic
principles, three years later coining the term Basalzellenkrebs, a term indicating
that the tumor originated in the basal layer of the epidermis or hair follicle (HF). In
contrast, in 1910, Mallory used the term hair matrix Tumor to specify the
follicular origin of BCC, illustrating the long-standing controversy and uncertainty
about the cellular origin of BCC.7 BCC originates as a neoplastic transformation of
the basal cells of the epidermis that proliferate and invade into the dermis as bulbous
nodules or invasive strands.8
Despite being malignant histologically, these tumors show a benign
progression, with metastasis being very rare. In most cases, these tumors are painless
and nodular, with an ulcerative central area. Although most do not show distinct
borders of subcutaneous infiltration, the borders of these tumors are grossly distinct,
the tumors grow slowly and distant and local metastases are rare.5
2. Anatomy of the ear
The external section is called the auricle and is composed of a single thin
plate of elastic cartilage covered with a thin layer of skin, which contains sebaceous
glands as well as fine hairs. The cartilage is composed of numerous elastic fibers and

is known for its ability to tolerate distortion without damage. The auricle is an ovalshaped structure and is directed slightly forward with a concave surface. Many
defined ridges and valleys shape the lateral surface of the auricle. The prominent
outer ridge is called the helix, which gives the overall shape of the ear. The helix
curves down and connects to the lobe, which is the inferior portion of the auricle.
The lobe contains no cartilage and is composed of tough areolar and adipose tissue,
making it the most vascularized portion of the external ear. The antihelix is also a
curved ridge, which lies interior and parallel to the helix. The antihelix splits and
forms a Y as it approaches the superior portion of the auricle; this split forms the
fossa triangularis. The valley between the helix and antihelix is called the scapha.
The deepest depression, which leads directly to the external auditory canal, or
acoustic meatus, is called the concha. The tragus (superior) and antitragus (inferior)
are separated by the intertragic notch located in front of the concha and mark the
beginning of the external auditory canal.9

Figure 2.1. Showing the surface anatomy of the external ear.2

The ear lobule was the most common site of auricular masses, followed by
the tragus, crus of the helix, triangular fossa, concha-crus of the antihelix and
antitragus-scapha. Frequent development in the lobule may be related to its being the
area of the auricle with the greatest abundance of soft tissue, as well as due to the
wearing of earrings and other jewelry, which may induce scars or inflammation.5
3. Prevalence
Basal cell carcinoma (BCC) is the most common human malignancy,
amounting to 90% of skin cancers. 2 BCC, first described by Jacob in 1824, most

commonly occurs in adults, especially in the elderly population and are more
prevalent in males than in females.1,5 Basal Cell Carcinomas (BCCs) are locally
destructive malignancies of the skin. They are the most common type of skin cancer
in Europe, Australia and the U.S.A. BCC is a locally invasive and slowly growing
malignant epidermal skin tumor mainly aff ecting the Caucasians.10
4. Risk Factors
Risk factors for BCC include fair skin type, sun exposure, ionizing radiation,
advanced age, immunesuppression, and a personal history of non melanoma skin
cancer. The most important risk factor for basal cell carcinoma is exposure to UVradiation.6 Ultraviolet (UV) and ionizing radiation can induce DNA damage that
leads to the development of skin cancer, and defective DNA repair is associated with
advanced age. In addition, studies of long-term immune-suppression after organ
transplantation and in patients with chronic lymphocytic leukemia showed increased
risks for cancers. Hereditary predisposition to BCC occurs among individuals with
albinism, xeroderma pigmentosum, nevoid basal cell carcinoma syndrome, and
Darrier disease2 .

5. Etiology and pathogenesis

Its etiology is still unclear, but both constitutional and environmental factors
and genetic predispotion are accused in BCC etiopathogenesis.6 Chronic exposure to
ultraviolet light is the most significant etiological factor and consequently exposed
areas such as the head and neck are the most commonly involved sites. 10 Almost all
basal cell carcinomas occur on parts of the body excessively exposed to the sun
especially the face, ears, neck, scalp, shoulders, and back.6
Basal cell carcinoma arises from basal keratinocytes of the epidermis, hair
follicles, and eccrine sweat ducts. Histologically, the cells are basophilic with large
nuclei. Because basal cell carcinoma requires surrounding stroma for support during
growth, it is virtually incapable of metastasizing via blood or lymphatics.13

Associated risk factors for nonmelanoma skin cancers are directly related to a
persons overall sun exposure or susceptibility to solar radiation including
environmental and occupational sun exposures, having fair skin that easily burns, or
having an inherited condition of increased susceptibility to UV radiation. Additional
factors

include

chronic

arsenic

exposure,

therapeutic

exposure

to

photochemotherapy (PUVA; especially for SCC), smoking, exposure to ionizing

radiation, chronic infection with human papillomavirus, immunosuppression, and
chronic nonhealing wounds.9
6. Sign and Symptom
Early tumor can be recognized as small, translucent, light colored papules of the skin,
completely covered by a thin epidermis through which telangiectases are noticeable or
erosion and ulceration. So different types of BCC nodular, keratotic, infiltrative, morphea
form, micronodular or superficial on dermoscopy. Sometimes the lesion is