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Unusual Cancers of Childhood

Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes and Carney Complex

Multiple endocrine neoplasia (MEN) syndromes

Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system. The endocrine system is made up of glands and cells that make hormones and release them into the blood. MEN syndromes may cause hyperplasia (the growth of too many normal cells) or tumors that may be benign (not cancer) or malignant (cancer).

There are several types of MEN syndrome and each type may cause different conditions or cancers. Patients and family members with an increased risk of these syndromes should have genetic counseling and tests to check for the syndromes.

The two main types of MEN syndromes are MEN1 and MEN2:

Tests used to diagnose and stage MEN syndromes depend on the symptoms and the patient's family history. They may include:

See the General Information section for a description of these tests and procedures.

Other tests and procedures used to diagnose MEN syndromes include the following:

Treatment

There are several types of MEN syndrome, and each type may need different treatment:

Skin Cancer (Squamous Cell Cancer, Basal Cell Cancer, Melanoma)

Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the body’s largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells:


Anatomy of the skin, showing the epidermis, dermis, and subcutaneous tissue. Melanocytes are in the layer of basal cells at the deepest part of the epidermis.
Anatomy of the skin, showing the epidermis, dermis, and subcutaneous tissue. Melanocytes are in the layer of basal cells at the deepest part of the epidermis.

There are three types of skin cancer:

Squamous Cell and Basal Cell Skin Cancer

The risk of squamous cell or basal cell cancer is increased by the following:

Symptoms of squamous cell and basal cell skin cancer include the following:

Tests that examine the skin are used to diagnose and stage squamous cell and basal cell skin cancer include the following:

Treatment of Squamous Cell and Basal Cell Skin Cancer

Treatment for squamous cell and basal cell cancer is usually surgery to remove the tumor.

Melanoma

Melanoma is the most common skin cancer in children. It occurs more often in children aged 10 to 19 years. Melanoma rates in the United States have slowly increased since 1975.

The risk of melanoma is increased by the following:

Risk factors for melanoma in all age groups include:

Symptoms of melanoma include the following:

Tests that examine the skin are used to diagnose and stage melanoma. They may include:

See the General Information section for a description of these tests and procedures.

Other tests and procedures used to diagnose melanoma include the following:

Treatment of Melanoma

Treatment for melanoma that has spread only to lymph nodes may be surgery to remove the tumor and lymph nodes with cancer, followed by biologic therapy with high-dose interferon alpha-2b.

Treatment for melanoma that has spread beyond the lymph nodes may include the following:

Chordoma

Chordoma is a very rare type of bone tumor that forms anywhere along the spine from the base of the skull to the tailbone. In children and teenagers, chordomas develop more often in the base of the skull, making them hard to remove completely with surgery.

Symptoms

Chordoma may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

Other conditions that are not chordoma may cause these same symptoms.

Chordomas may recur (come back), usually in the same place, but sometimes they recur in other areas of bone or in the lungs.

Treatment

Treatment for chordoma in children is usually surgery to remove as much of the tumor as possible, followed by radiation therapy. Proton beam radiation therapy may be used.

Cancer of Unknown Primary Site

Carcinoma of unknown primary (CUP) is a rare disease in which malignant (cancer) cells are found in the body but the place the cancer began is not known. Cancer can form in any tissue of the body. The primary cancer (the cancer that first formed) can spread to other parts of the body. This process is called metastasis. Cancer cells usually look like the cells in the type of tissue in which the cancer began. For example, breast cancer cells may spread to the lung. Because the cancer began in the breast, the cancer cells in the lung look like breast cancer cells.

Sometimes doctors find where the cancer has spread but cannot find where in the body the cancer first began to grow. This type of cancer is called a cancer of unknown primary (CUP) or occult primary tumor.

Tests are done to find where the primary cancer began and to get information about where the cancer has spread. When tests are able to find the primary cancer, the cancer is no longer a CUP and treatment is based on the type of primary cancer.

Because the place where the cancer started is not known, many different tests and procedures may be needed to find out what type of cancer it is. If tests show there may be cancer, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist. The pathologist views the tissue under a microscope to look for cancer cells and to find out the type of cancer. The type of biopsy that is done depends on the part of the body being tested for cancer. One of the following types of biopsies may be used:

When the type of cancer cells or tissue removed is different from the type of cancer cells expected to be found, a diagnosis of CUP may be made. The cells in the body have a certain look that depends on the type of tissue they come from. For example, a sample of cancer tissue taken from the breast is expected to be made up of breast cells. However, if the sample of tissue is a different type of cell (not made up of breast cells), it is likely that the cells have spread to the breast from another part of the body.

Adenocarcinomas, melanomas, and embryonal tumors are common tumor types that appear and it is not known where the cancer first formed. Embryonal tumors such as rhabdomyosarcomas and neuroblastomas are most common in children.

Treatment

Treatment depends on what the cancer cells look like under a microscope, the patient's age and symptoms, and where the cancer has spread in the body. Treatment is usually chemotherapy or radiation therapy.