Somatostatinoma Definition

Somatostatinomas are rare tumors of the pancreatic cells that secrete the hormone somatostatin. The pancreas is not the only location where somatostatin is produced but larger somatostatinomas are most likely to occur in the pancreas. Since this hormone has a suppressive action on most other hormones, a somatostatinoma tends to present with symptoms associated with the inhibition of these hormones. Somatostatinomas are malignant meaning that it is cancerous but is curable if the tumor can be surgically removed before the cancer has spread to other organs.

Somatostatinoma Incidence

Overall somatostatinomas are very rare. It occurs in only about 1 in 40 million people in the United States. The prevalence is equal in both men and women. Somatostatinomas are more frequently detected in the 40 to 60 year age group although some of the conditions it is associated with, like multiple endocrine neoplasia type 1 and neurofibromatosis, often start earlier in life. Since somatostatinomas are slow growing tumors, this may account for its diagnosis later in life.

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Definition

A glucagonoma is a tumor that secretes glucagon, a hormone normally produced in the pancreas and responsible for raising blood sugar (glucose) levels. Glucagonomas are almost entirely found in the pancreas, arising from the alpha-2 cells of the gland, and about 80% of these tumors are malignant (cancerous). The tumor can cause elevated blood glucose levels known as glucagonoma syndrome but this is not present in every case.

The elevated blood glucagon level (hyperglucagonemia) along with raised blood glucose levels (hyperglycemia) lead to a host of complications, marked by the 4Ds – diabetes mellitus, dermatitis (skin rash), deep vein thrombosis (DVT) and depression. The prognosis for glucagonomas are poor, especially once the cancerous cells spread to adjacent organs.

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What is parathyroid cancer?

Parathyroid cancer (carcinoma) is a malignant tumor of the parathyroid gland. It is a rare type of cancer that originates from the uncontrolled growth of the cells of the parathyroid gland. Since the cancer starts in the parathyroid cells itself, it is also known as a primary cancer. If cancer that starts elsewhere in the body and spreads to the parathyroid gland, then it is known as a secondary cancer or metastatic cancer. Parathyroid carcinoma is one of the rare cancers. It affects about 1 in 7.5million people and affects both males and females equally. Most cases of parathyroid cancer are seen in people over the age of 30 years.

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What is a mediastinal lymphoma?

Mediastinal lymphoma is a rare type of cancer. More popularly known as primary mediastinal B-cell lymphoma (or PMBL), it arises in a specialized organ of the immune system called the thymus. Mediastinal lymphoma or PMBL mostly affects young adults. It is a sub-type of diffuse large B-cell lymphomas (DLBC), which are the most common and aggressive types of all the lymphomas.

Lymphomas are the cancers of the lymphocytes, which are a type of immune cells. Both diffuse large B-cell lymphomas and primary mediastinal B-cell lymphomas affect B-lymphocytes of the immune system; however, the thymic mediastinal lymphoma has a better prognosis. Primary mediastinal B-cell lymphomas may also resemble another type of lymphoma, Hodgkin lymphoma, in their characteristics.

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What is Kaposi sarcoma?

Kaposi sarcoma (KS) is a type of cancer of the lymphatic vessels and blood vessels which is seen mostly on the skin. Less commonly it may also occur in other tissue like that of the mouth, other parts of the gut and even the lungs. Kaposi sarcoma is a rare cancer but has become more common in recent decades mainly due to its association with HIV/AIDS. It is considered an AIDS-defining illness. This does not mean that Kaposi sarcoma will not occur in a person who is HIV negative. It is caused by a certain type of herpes virus and can affect any person although some people are at greater risk.

What causes Kaposi sarcoma?

Kaposi sarcoma has been associated with the human herpesvirus 8 (HHV-8). This virus is therefore also known as Kaposi sarcoma herpesvirus (KSHV). The virus then infects cells lining the lymphatic vessels and blood vessels. It causes these cells to become cancerous possibly by inserting its genes into the cell which then disrupts normal cell proliferation. The cells then divide rapidly in an uncontrolled manner as is the case with cancer. Although HHV-8 is easily transmitted through saliva, it will have major effects in most people who are HIV negative or with a healthy immune system.

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