What are the other Names for this Condition? (Also known as/Synonyms)

• Basal-Cell Cancer of Skin with Adnexal Differentiation
• BCC of Skin with Adnexal Differentiation

What is Basal Cell Carcinoma of Skin with Adnexal Differentiation? (Definition/Background Information)

• Basal Cell Carcinoma (BCC) of Skin is a malignant condition affecting the skin. It is a slow-growing tumor generally observed in older individuals, in both men and women
• This malignant carcinoma, which may be present as a lesion on the sun-exposed areas of the body, has the potential to metastasize (spread) to the lymph nodes
• Basal Cell Carcinoma of Skin with Adnexal Differentiation is an uncommon type of BCC that can present sebaceous differentiation, eccrine differentiation, or apocrine differentiation, when examined under the microscope by a pathologist
• There are three different types of sweat glands in the skin. These include the following:
  • Apocrine sweat glands
  • Eccrine sweat glands
  • Sebaceous oil glands
• In BCC of Skin with Adnexal Differentiation, there is a combination of basal cell carcinoma and histological features of any of the three adnexal glands; any of the sweat gland morphological features may be mixed up with BCC
• Some lesions may grow to large sizes and ulcerate. They can also infiltrate into the adjoining soft tissues and nerves. Larger tumors also have a greater tendency to recur after treatment
• The cause of Basal Cell Carcinoma of Skin with Adnexal Differentiation is unknown, but factors, such as chronic sun exposure, smoking, and ionizing radiation, etc., are known to contribute towards its development. Also, fair-skinned Caucasians have a greater risk for the condition than dark-skinned Africans and Asians
• Any combination of chemotherapy, radiation therapy, and invasive procedures (surgery) are used to treat Basal Cell Carcinoma of Skin with Adnexal Differentiation. Small-sized tumors and tumors that have not metastasized may be curable through appropriate skin surgery
• The prognosis for metastatic tumors depends upon many factors including the stage of the tumor, health status of the individual, and their treatment response. The prognosis may be guarded

Who gets Basal Cell Carcinoma of Skin with Adnexal Differentiation? (Age and Sex Distribution)

• Basal Cell Carcinoma of Skin with Adnexal Differentiation is an uncommon skin cancer that generally affects elderly or older adults; some cases may rarely develop in children
• It can occur in both males and females; however;
  • Among the older age group, males are affected more than females
  • In the younger age group, females are affected more than males, which might be attributable to their tendency to acquire sun-tanned bodies or visit skin tanning parlors (more than males)
• This condition is observed worldwide across all racial and ethnic groups; however, lighter-skinned individuals (especially Caucasians) are more prone to BCC of Skin with Adnexal Differentiation than darker-skinned individuals (such as Asians, Africans, etc.)

What are the Risk Factors for Basal Cell Carcinoma of Skin with Adnexal Differentiation? (Predisposing Factors)

The risk factors that contribute to Basal Cell Carcinoma of Skin with Adnexal Differentiation formation include:

• Prolonged sun exposure, exposure to ultraviolet (UV) light
• Use of tanning beds, tanning parlors
• Arsenic exposure
• Ionizing radiation
• Smoking
• The presence of certain genetic syndromes, such as basal cell nevus syndrome (a rare autosomal dominant disorder), is known to increase the risk
• Caucasians are more vulnerable compared to other darker-skinned individuals

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Basal Cell Carcinoma of Skin with Adnexal Differentiation? (Etiology)

• The exact cause of development of Basal Cell Carcinoma of Skin with Adnexal Differentiation is not completely understood in a majority of cases
• Although, genetic mutations have been detected in basal cell carcinomas that are currently being characterized
• Most BCCs are sporadic in origin i.e., they occur in a random fashion

What are the Signs and Symptoms of Basal Cell Carcinoma of Skin with Adnexal Differentiation?

Basal Cell Carcinoma of Skin with Adnexal Differentiation signs and symptoms may include:

• BCC of Skin with Adnexal Differentiation is a slow-growing malignant tumor. The tumor is a typical skin lesion that presents papular and nodular appearance
• The surface of the papule may be red, if intact. Else, it may appear as an ulcer, if the surface is eroded
• It is typically observed on sun-exposed areas of the body; common sites include the head and neck region, arms and legs, etc.
• The tumor may be solitary or many in number. In children, if it is associated with basal cell nevus syndrome, then multiple lesions may be observed
• In some individuals, BCC of Skin with Adnexal Differentiation  show a pigmented appearance and may resemble a melanoma
• Most lesions are less than 1-2 cm, but some may grow to larger sizes of even 10 cm
• There may be itching sensation, ulceration, and bleeding

How is Basal Cell Carcinoma of Skin with Adnexal Differentiation Diagnosed?

Some of the tests that may help in diagnosing Basal Cell Carcinoma of Skin with Adnexal Differentiation include:

• Complete physical examination with detailed medical history evaluation
• Examination by a dermatologist using a dermoscopy, a special device to examine the skin
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin or tissue biopsy: A skin or tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Differential diagnosis of other tumors should be ruled out; hence, biopsy is an important diagnostic tool

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Basal Cell Carcinoma of Skin with Adnexal Differentiation?

The complications of Basal Cell Carcinoma of Skin with Adnexal Differentiation could include:

• If the tumor becomes big, develops into a firm mass and ulcerates, it can get secondarily infected with bacteria or fungus
• Metastasis to regional lymph nodes can occur. The tumor can also infiltrate into the surrounding structures
• BCC of Skin with Adnexal Differentiation can cause cosmetic issues, since these tumors can cause large ulceration (termed rodent ulceration)
• Recurrence of the tumor after a period of time; recurrence is frequently common with large tumors
• Side effects of chemotherapy (such as toxicity) and radiation may be noted

How is Basal Cell Carcinoma of Skin with Adnexal Differentiation Treated?

In general, the treatment of Basal Cell Carcinoma of Skin depends upon a variety of factors including:

• The subtype of BCC
• The location of the tumor
• The number of tumors
• The size of the tumor
• Whether the tumor has metastasized

A combination of treatment methods may be used to treat Basal Cell Carcinoma of Skin with Adnexal Differentiation. The type of surgery may include:

• Curettage and electrodessication: This procedure is used for small tumors. There is no requirement of sutures (stitches) after the surgery
• Excision of tumor: In this procedure, the tumor and surrounding tissue are removed with clear margins. Depending upon the amount of skin removed, surgical sutures may be necessary
• Mohs surgery: In this procedure, the tumor is removed layer by layer precisely, until clear margins are achieved. Each layer removed is examined under a microscope through a ‘frozen section’ procedure, for the presence of residual tumor

In most cases, a surgical removal of the entire tumor is the preferred treatment option. This can result in a cure.

• If the tumor has metastasized (in rare cases), then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
• Targeted therapy medications are generally used for locally infiltrated or metastatic BCCs. This therapy destroys the tumor cells by acting against the proteins that are responsible for tumor growth
• Large tumors that have infiltrated surrounding structures can be treated with radiation therapy (the use of high-energy beams to kill cancer cells)
• Large tumors after complete skin excision may need skin grafting
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals

Other techniques to treat this skin cancer (sometimes, when surgery may not be an option) may include:

• Photodynamic therapy: In this procedure, a chemical cream is applied onto the tumor, which sensitizes the tumor to light (photosensitization). After the cream application, the tumor is exposed to light. This combination of chemical therapy and light therapy kills the tumor. Usually, this technique results in good cosmetic outcomes
• Treatment with oral hedgehog pathway inhibitors vismodegib and sonidegib
• Cryotherapy: Here the tumor tissue is destroyed through a freezing technique. Typically liquid nitrogen is used to freeze the tumor
• Topical creams, such as 5-fluorouracil cream and imiquimod cream, are two examples that can be used for topical treatment. These creams may be applied for several weeks, which slowly destroys the tumor

• Note: If multiple lesions occur in children, then the possibility of basal cell nevus syndrome should be considered.

Generally, proper follow-up care with regular screening and check-ups are important and encouraged. In the case of large lesions, close follow-up and periodic observation is essential, since they have a tendency to recur.

How can Basal Cell Carcinoma of Skin with Adnexal Differentiation be Prevented?

Currently, Basal Cell Carcinoma of Skin with Adnexal Differentiation is a malignant skin cancer that has no preventive measures. However, the following factors may help reduce the risk for the condition:

• Avoid or minimize sun exposure
• Limit the use of tanning beds, tanning parlors
• Smoking cessation
• If it is caused by certain underlying disorders, then treating the underlying condition may help in the treatment and early resolution of BCC of Skin with Adnexal Differentiation
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory, due to its metastasizing potential and high possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Basal Cell Carcinoma of Skin with Adnexal Differentiation? (Outcomes/Resolutions)

• In general, the prognosis of Basal Cell Carcinoma of Skin with Adnexal Differentiation is excellent, if it is detected and treated early. However, if it metastasizes to the local lymph nodes, the prognosis is guarded or unpredictable
• In such cases of metastatic BCC, its prognosis depends upon a set of several factors that include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The surgical resectability of the tumor (meaning if the tumor can be removed completely)
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared to those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have a poorer prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond so well to treatment
• Without treatment (or with delayed treatment), Basal-Cell Cancer of Skin with Adnexal Differentiation can metastasize and this may result in a poor prognosis
• Close and regular follow-up and long-term monitor for recurrence of BCC has to be maintained

Additional and Relevant Useful Information for Basal Cell Carcinoma for Skin with Adnexal Differentiation:

There are multiple types of Basal Cell Carcinoma of Skin:

• Superficial Basal Cell Carcinoma of Skin
• Nodular Basal Cell Carcinoma of Skin
• Infiltrating Basal Cell Carcinoma of Skin
• Micronodular Basal Cell Carcinoma of Skin
• Fibroepithelial Basal Cell Carcinoma of Skin
• Basal Cell Carcinoma of Skin with Adnexal Differentiation
• Basosquamous Carcinoma
• Keratotic Basal Cell Carcinoma of Skin