Signet ring adenocarcinoma is a rare and aggressive form of cancer that can occur in any organ lined with epithelium. It is characterized by the off-center nucleus of cancer cells, and symptoms are similar to those of more common tumors. Diagnosis is achieved through blood tests, imaging, and biopsy, and treatment usually involves surgery and chemotherapy. The prognosis is generally poor.
In signet ring adenocarcinoma, the skin cells lining the internal organs lose their structure and become unfit for their intended purpose. These cells reproduce rapidly and bind together to form cancerous growths. This form of cancer typically affects the organs of the digestive system, but signet ring cells, in theory, can occur in any organ lined with epithelium. Adenocarcinoma of this type is rare but very aggressive.
The distinctive physical appearance of individual cancer cells gives signet-ring adenocarcinoma its name. Normally, the portion of the cell that contains the deoxyribonucleic acid (DNA) needed for reproduction is found in the center of the cell. In signet ring cells, this structure, called the nucleus, is off-center and suspended in the mucus. The resulting structure is reminiscent of the crest rings traditionally used by royalty.
The presentation of signet-ring adenocarcinoma is identical to more common tumors of the same organ. In stomach cancer, these symptoms may include intestinal pain, stomach pain, and bloody stools. Although extremely rare, this condition can affect the lungs. In these cases, persistent cough, chest pain and bloody mucus are often reported.
Diagnosis of signet-ring adenocarcinoma is usually achieved by a combination of blood tests and diagnostic imaging. Abnormal blood cell counts can indicate the presence of cancer, and the patient’s symptoms can provide clues to its location, but finding the exact location of an abnormal growth requires ultrasound, X-ray, or magnetic resonance imaging (MRI) procedures. If a tumor is found, a surgeon usually takes a small sample of the growth in a procedure called a biopsy. Visual confirmation of signet ring cells is by examining the tissue under a microscope.
Depending on the area affected, surgical removal of a cancerous growth may be required. Although radiation therapy may be used briefly before surgery, postoperative radiation therapy is usually not recommended. Because of the aggressive nature of signet-ring adenocarcinoma, chemotherapy is almost always prescribed.
Frequently, signet ring tumors of the lower digestive tract are treated with a special type of chemotherapy called hyperthermic intraperitoneal chemotherapy (HIPEC). After any tumors in the stomach have been removed, the chemotherapy drugs are warmed to a slightly above normal body temperature and circulated through the abdominal cavity. In many cases, localized application of drugs can reduce the systematic side effects of traditional chemotherapy.
The prognosis for patients with signet ring adenocarcinoma is generally poor. Historically, the disease is extremely aggressive and has frequently spread to other areas of the body before a diagnosis is made. Also, the cancer typically does not respond to chemotherapy.
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