History
Patients presenting with basal cell carcinoma (BCC) often report a slowly enlarging lesion that does not heal and that bleeds when traumatized. As tumors most commonly occur on the face, patients often give a history of an acne bump that occasionally bleeds.
People who sunburn are more likely to develop skin cancer than those who do not; however, sunlight damages the skin with or without sunburn. Consider BCC in any patient with a history of a sore or skin anomaly that does not heal within 3-4 weeks and occurs on sun-exposed skin, especially if it is dimpled in the middle. These tumors may take many months or years to reach even 1 cm in diameter.
Patients often have a history of chronic sun exposure, including recreational sun exposure (eg, sunbathing, outdoor sports, fishing, boating) and occupational sun exposure (eg, farming, construction).
History of any prior treatment to the index tumor should be elicited, as well as history of any prior non-melanoma skin cancer. In patients with recurrent tumors, deeper invasion should be expected. Recurrence following radiation therapy is often biologically more aggressive.
Occasionally, patients have a history of exposure to ionizing radiation. X-ray therapy for acne was commonly used until 1950. Though not common, patients may have a history of arsenic intake; arsenic is found in well water in some parts of the United States.
Physical Examination
Characteristic features of BCC tumors include the following:
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Waxy papules with central depression
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Pearly appearance
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Erosion or ulceration, often central
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Bleeding, especially when traumatized
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Crusting
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Rolled (raised) border
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Translucency
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Telangiectases over the surface
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Slow growing (0.5 cm in 1-2 y)
Basal cell carcinoma occurs mostly on the face, head (scalp included), neck, and hands. [4] It rarely develops on the palms and soles. BCC usually appears as a flat, firm, pale area that is small, raised, pink or red, translucent, shiny, and waxy, and the area may bleed following minor injury. BCCs may have one or more visible and irregular blood vessels, an ulcerative area in the center that often is pigmented, and black-blue or brown areas. Large BCCs may have oozing or crusted areas. The lesion grows slowly, is not painful, and does not itch.
Periocular tumors most commonly involve the lower eyelid (48.9-72.1%), followed by the medial canthus (25-30%), the upper eyelid (15%), and the lateral canthus (5%). Examples are shown in the images below.
Biopsy-proven basal cell carcinoma of the upper lid margin. Note the loss of cilia (madarosis) in the area of the tumor.
Medial canthal/lower lid basal cell. Note the pearly nodular surface with characteristic telangiectatic vessels. Proximity to the lacrimal system will impact its treatment and reconstruction.
Though a literature review showed all authors agreed that periocular BCC most commonly occurs in the lower eyelid, the remaining anatomical locations and the incidence of occurrence differ among the studies.
Younger patients (< 40 y) may have a lower prevalence of BCC on the head and neck and a higher prevalence on the trunk, with greater tendency to superficial BCC, than in older patients. [57] Childhood BCC is exceedingly rare in the absence of other underlying conditions. Only 107 cases of de novo childhood BCC have been reported in the literature, but the majority (90%) occurred on the head and neck, and aggressive subtypes were observed in 20% of the total cases. [58]
Clinical presentation of BCC varies by type. Physical examination of the skin aids in determination of tumor extent, subtype, and involvement of important cosmetic and functional structures. Matted BCCs may indicate deeper tumor invasion and involvement of deeper underlying structures. In patients with recurrent or deeply infiltrative tumors, involvement of the facial nerve or branches of the trigeminal nerve should be investigated. Facial nerve function can be monitored by comparing facial symmetry during voluntary facial movements with that at rest. Sensory nerve function can be tested and compared to the nonaffected side by means of light touch and pinprick. Orbital invasion can cause diplopia, proptosis, and ophthalmoplegia. Any limitation in ocular movements and/or diplopia should be tested.
BCC seldom causes regional or distant metastasis, with the exception of the metatypical basosquamous type. To evaluate for lymph node metastasis, particular attention should be taken to examine the parotid posterior auricular, suboccipital, and upper cervical groups of lymph nodes.
Several different clinicopathologic types of BCC exist, each with distinct biologic behavior:
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Nodular - Cystic, pigmented, keratotic
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Infiltrative
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Micronodular
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Morpheaform
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Superficial
Nodular basal cell carcinoma
Nodular basal cell carcinoma is the most common type of basal cell carcinoma and usually presents as a round, pearly, flesh-colored papule with telangiectases. More than 60% of BCCs belong to this subtype. As it enlarges, it frequently ulcerates centrally, leaving a raised, pearly border with telangiectases, which aids in making the diagnosis. Fine vessels may bleed, resulting in hemosiderin deposition.
The tumor may present as a cyst, which can be mistaken for inclusion cysts of the eyelid. Cystic BCC is an uncommon variant of nodular BCC and is often clinically indistinguishable from nodular basal cell carcinoma, although it might have a polypoid, cystic appearance. Typically, a bluish-gray cystlike lesion is observed. The cystic center of the tumor is filled with clear mucin that has a gelatinlike consistency. Often, one can see the typical features of a nodular basal cell carcinoma in addition to the cystic features.
Most tumors are observed on the face, although the trunk and extremities also are affected. See the images below.
Nodular basal cell carcinoma appearing as a waxy, translucent papule with central depression and a few small erosions.
Pigmented basal cell carcinoma (see the images below) is an uncommon variant of nodular basal cell carcinoma that usually has brown-black macules in some areas or affecting nearly the entire tumor, occasionally making it difficult to differentiate from melanoma.
Typically, some areas of these tumors do not retain pigment, and pearly, raised borders with telangiectases that are typical of a nodular basal cell carcinoma can be observed. This aids clinically in differentiating this tumor from a malignant melanoma. See the images below.
Pigmented basal cell carcinoma has features of nodular basal cell carcinoma with the addition of dark pigmentation from melanin deposition. The pigmentation often has the appearance of dark droplets in the lesion, as shown here.
Keratotic BCC is a variant of nodular BCC and is usually clinically indistinguishable from nodular BCC histologically.
Infiltrative basal cell carcinoma
With this variant of BCC, tumor infiltrates the dermis in thin strands between collagen fibers, making tumor margins less clinically apparent. Mohs micrographic surgery is the treatment of choice for infiltrative basal cell carcinoma. Because of its growth pattern, electrodesiccation and curettage has a significantly higher recurrence rate when used to treat infiltrative BCC compared to the treatment of nodular BCC; other treatment methods should be sought.
Micronodular basal cell carcinoma
This aggressive BCC subtype has the typical BCC distribution. It is not prone to ulceration, it may appear yellow-white when stretched, and it is firm to the touch. It may have a seemingly well-defined border.
Morpheaform (sclerosing) basal cell carcinoma
Morpheaform basal cell carcinoma is an uncommon variant in which tumor cells induce a proliferation of fibroblasts within the dermis and an increased collagen deposition (sclerosis) that clinically resembles a scar. This form accounts for 10% of lesions.
Such lesions appear as flat or slightly depressed, fibrotic, and firm. The tumor appears as a white or yellow, waxy, sclerotic plaque that rarely ulcerates. The morpheaform (sclerosing) type of basal cell carcinoma is often the most difficult type to diagnose, as it bears little resemblance to the typical nodular BCC.
Because the tumor infiltrates in thin strands between collagen fibers, treatment is difficult because the clinical margins are difficult to distinguish from normal, uninvolved skin. Mohs micrographic surgery is the treatment of choice for morpheaform basal cell carcinoma because recurrence is more likely with other treatment modalities.
Ulceration, bleeding, and crusting are uncommon and these tumors are commonly mistaken for scar tissue (see the image below).
Superficial basal cell carcinoma
Superficial basal cell carcinomas are seen mostly on the upper trunk or shoulders. This type of BCC grows slowly, has minimal tendency to be invasive, and appears clinically as an erythematous, well-circumscribed patch or plaque, often with a whitish scale. Occasionally, minute eschars may appear within the patch or plaque. The tumor often appears multicentric, with areas of clinically normal skin intervening among clinically involved areas.
A threadlike border is common but not always present. Erosion is less common in superficial BCC than in nodular BCC, although pinpoint areas of hemorrhage or eschar may be present. The papules may mimic psoriasis or eczema, but they are slowly progressive and are not prone to fluctuate in appearance. Numerous superficial BCCs may indicate arsenic exposure. See the images below.
Scale, erythema, and a threadlike raised border are present in this superficial basal cell carcinoma on the trunk.
Gorlin syndrome or basal cell nevus syndrome
Basal cell carcinoma (BCC) is also a feature of basal cell nevus syndrome (ie, Gorlin syndrome), [59] an autosomal dominant inherited condition. The lesions in these patients cannot be distinguished histologically from ordinary BCCs. The gene responsible for this syndrome is located on arm 9q, and chromosome abnormalities develop in some patients. The number of BCCs in patients with this syndrome may number from one to hundreds. Multiple BCCs begin to appear after puberty on the face, trunk, and extremities. In many cases, the tumors are highly invasive and may involve areas around the eyes and nose. [60]
Other features associated with Gorlin syndrome (fortunately, uncommon) include the following [61] :
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Mental retardation
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Congenital agenesis of the corpus callosum and medulloblastoma
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Odontogenic jaw cysts
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Bifid ribs and pectus excavatum
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Absent or undescended testes
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Mesenteric lymphatic cysts
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Palmar and plantar pits
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Ectopic calcification (particularly of the falx cerebri)
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Ocular and skeletal abnormalities (eg, hypertelorism, shortening of the fourth and fifth metacarpals)
Other basal cell carcinomas
Other types of basal cell carcinoma include the following:
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Metatypical
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Infundibulocystic
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Follicular
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Pleomorphic
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A pink, scaly lesion on the skin. Superficial basal cell carcinoma (BCC) is often misdiagnosed as eczematous dermatitis or guttate psoriasis and is often difficult to distinguish clinically from Bowen disease (squamous cell carcinoma in situ). Features that suggest the diagnosis of superficial BCC are the absence of significant white, adherent scale and a history of the lesion remaining unchanged for several months or years. Treatment options for this tumor include electrodesiccation and curettage, surgical excision, cryosurgery, 5-fluorouracil, 5% imiquimod cream, and superficial radiotherapy. Electrodesiccation and curettage is the modality most commonly used, with a cure rate of approximately 95%.
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Basal cell carcinoma.
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A 68-year-old patient presenting with an advanced basal cell carcinoma (BCC) of the right periorbital region, frontal view. Courtesy of M Abraham Kuriakose, DDS, MD.
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Lateral view of face showing extent of tumor. Courtesy of M Abraham Kuriakose, DDS, MD.
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Basal cell carcinoma of the right lower lid.
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Biopsy-proven basal cell carcinoma of the upper lid margin. Note the loss of cilia (madarosis) in the area of the tumor.
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Medial canthal/lower lid basal cell. Note the pearly nodular surface with characteristic telangiectatic vessels. Proximity to the lacrimal system will impact its treatment and reconstruction.
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Nodular basal cell carcinoma.
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Nodular basal cell carcinoma appearing as a waxy, translucent papule with central depression and a few small erosions.
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Scale, erythema, and a threadlike raised border are present in this superficial basal cell carcinoma on the trunk.
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Large, superficial basal cell carcinoma.
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Basal cell carcinoma. Courtesy of Hon Pak, MD.
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Pigmented basal cell carcinoma.
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Pigmented basal cell carcinoma.
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Pigmented basal cell carcinoma has features of nodular basal cell carcinoma with the addition of dark pigmentation from melanin deposition. The pigmentation often has the appearance of dark droplets in the lesion, as shown here.
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This infiltrating basal cell cancer has ill-defined borders and telangiectases.
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This translucent pink papule has telangiectases and a crusted erosion, characteristic of nodular basal cell carcinoma.
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Large, scarlike morpheaform basal cell cancer.
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Nodular basal cell carcinoma. Nodular aggregates of basalioma cells are present in the dermis and exhibit peripheral palisading and retraction artifact. Melanin is also present within the tumor and in the surrounding stroma, as seen in pigmented basal cell carcinoma.
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Histologic pattern of a well-differentiated basal cell carcinoma (original magnification X140). Courtesy of Prof Pantaleo Bufo, University of Foggia, Italy.
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Histologic pattern of a well-differentiated basal cell carcinoma (original magnification X250). Courtesy of Prof Pantaleo Bufo, University of Foggia, Italy.
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Micronodular basal cell carcinoma often has an absence of retraction artifact. The characteristic histology is small size and uniformity of the tumor nodules. Courtesy of Shang I Brian Jiang, MD.
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Infiltrative basal cell carcinoma. Tumor cells are arranged in narrow strands, and mucin-rich stroma is often present. Courtesy of Shang I Brian Jiang, MD.
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Keratotic basal cell carcinoma. Rare type characterized by keratocysts. Courtesy of Shang I Brian Jiang, MD.
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Basosquamous basal cell carcinoma. Foci of neoplastic cells with squamous differentiation are present. Courtesy of Shang I Brian Jiang, MD.
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Histology of superficial basal cell carcinoma. Nests of basaloid cells are seen budding from the undersurface of the epidermis. Courtesy of Michael L Ramsey, MD.
