A CUP syndrome is used when the organism has metastasized (tumor colonization) and the primary tumor cannot be identified. About two to five percent of cancer patients are affected by CUP syndrome, which in most cases is lethal (ie fatal).
What is CUP Syndrome?
According to AbbreviationFinder, the CUP syndrome (Cancer of Unknown Primary – cancer of unknown origin) is the metastasis of the organism through secondary tumors (metastases) for which no original tumor can be localized within the framework of diagnostic procedures.
The majority of malignant (malignant) tumors develop metastases when tumor cells settle via the lymphatic system (lymphogenic metastasis) or the blood system (hematogenic metastasis). With lymphogenic metastasis, carcinoma cells enter the lymphatic system, penetrate the tissue there with tumor cells and form lymph node metastases.
If, on the other hand, carcinoma cells penetrate the blood system through a destroyed vascular wall, they are carried over this and can form distant metastases. If the source of this process cannot be identified, CUP syndrome is present.
Various factors are thought to be the cause of CUP syndrome. On the one hand, the primary tumor can be too small to be identified using conventional diagnostic methods, which is attributed to a faster division of the metastatic cells with a higher potential for degeneration at the same time.
On the other hand, the primary tumor may already have been decomposed by the organism after metastasis has taken place. A possible surgical removal (e.g. when removing an intestinal polyp) must also be taken into account in the case of a CUP syndrome. In the majority of cases of CUP syndrome (20 to 30 percent), the metastasis is caused by a lung cancer, in around 15 to 25 percent by a pancreatic tumor (pancreatic cancer).
In the CUP syndrome, however, metastasis can also originate from tumor diseases in other organs (liver, kidneys, stomach, uterus, etc.), whereby common cancers (large intestine, prostate, mammary gland) rarely trigger a CUP syndrome.
Symptoms, ailments & signs
The symptoms and symptoms of CUP syndrome can vary widely. They depend very much on the extent of the metastasis and the exact primary tumor. A general prediction of the complaints is therefore not possible. However, those affected suffer from the formation of metastases, which can usually develop in regions of the body alone.
This leads to severe pain, nausea or vomiting. Even coughing or severe headaches may occur when CUP syndrome and adversely affect the quality of life of the patient. The CUP syndrome always leads to a premature death of the person concerned and therefore to a significantly reduced life expectancy of the patient. As a rule, there are severe restrictions on movement and restrictions in everyday life.
The CUP syndrome is often associated with severe psychological complaints, so that the patients and their relatives also suffer from depression or other psychological disorders. Furthermore, the CUP syndrome leads to a loss of consciousness or a comatose state. As the cause of the syndrome itself cannot be treated, there is only symptomatic relief of the symptoms. In most cases, people only have about twelve months to pass away.
Diagnosis & course
Since a CUP syndrome only occurs when the primary tumor cannot be identified, the localization of the initial focus takes on a special position. In addition to a physical examination, a blood analysis (including for tumor markers) and saliva, stool and urine examinations, the results of the biopsy (removal and histological analysis) of the metastatic tissue are decisive in the presence of a CUP syndrome.
Since metastases usually have the histological structure (fine tissue structure) of the original tumor, conclusions about the primary tumor are possible here. In addition, the results are crucial for the treatment of CUP syndrome. In addition, imaging procedures (sonography, X-ray, MRT, CT) as well as endoscopic and nuclear medicine examination procedures are used for diagnosis.
The course of a CUP syndrome depends on the underlying tumor, the degree of metastasis and the general condition of the person affected. On average, however, a life expectancy of 6-13 months can be expected, with only about 33 to 40 percent of those affected by the CUP syndrome still alive after 12 months.
In most cases, CUP syndrome leads to patient death. However, it cannot be predicted how much the life expectancy of the person affected will be reduced. The symptoms and complications also depend on the region and the type of tumor. Therefore, no universal statement is possible here either. The person concerned generally feels sick and weak and suffers from exhaustion.
There is also severe pain that can affect the entire body. Furthermore, there is nausea and vomiting and also headaches. The quality of life of the person affected is extremely limited and reduced by the CUP syndrome. In many cases, patients are dependent on a stay in hospital and on the help of other people in everyday life in order to be able to do most of it.
As a rule, those affected remain alive for another year. The complaints often lead to psychological complaints and depression. The patient’s relatives can also suffer from these symptoms. Treatment of CUP syndrome is not possible in all cases. The exact risks and opportunities depend on the cancer.
When should you go to the doctor?
In most cases, the CUP syndrome is recognized during other examinations, so that an additional diagnosis by a doctor is not necessary. If the metastases have already spread in the body, treatment is usually no longer possible, so that only the symptoms of the tumor disease can be weakened. Those affected should then contact a doctor if the tumor causes pain or other symptoms.
This could also be nausea or a cough. In many cases, those affected have to be hospitalized and die prematurely. Psychological treatment is also often necessary, which can affect not only those affected but also relatives. A psychologist can be consulted for this. Treatment of the symptoms depends heavily on the affected area and is carried out by a specialist doctor. In most cases, the syndrome will significantly reduce the patient’s life expectancy.
Treatment & Therapy
In the case of CUP syndrome, therapeutic approaches that are generally used for cancer diseases are usually recommended. These include surgical therapy, radiation, hormone and chemotherapy. In the case of CUP syndrome, the therapeutic measures depend on the histological findings of the biopsy, the degree of metastasis, the suspected primary tumor and the general condition of the person affected.
In the case of locally limited metastasis, surgical procedures and / or radiation therapy are possible. If there are multiple metastases in different locations, chemotherapy is recommended in most cases.
If the histological findings indicate a hormone-sensitive initial tumor, hormone therapy is used. If there is a strong suspicion of a primary tumor in the lungs, large intestine, kidney or liver, tyrosine kinase inhibitors or antibodies can be used, whereby these so-called targeted molecular forms of therapy for CUP syndrome are still being tested in the context of scientific treatment studies.
In addition, the symptoms accompanying the illness are always treated. If, for example, the bones are metastasized, active ingredients to strengthen the bones (bisphosphonates) are also used. If the metastasis is already very advanced or if the person affected by the CUP syndrome has a poor constitutional condition, the therapeutic measures are primarily aimed at alleviating symptoms, improving quality of life and preventing complications.
Outlook & forecast
In most cases, the CUP syndrome causes the patient to die prematurely. Since the tumor has already spread throughout the body and metastasized, the disease can usually no longer be fully treated. Only the individual symptoms can be restricted.
Since the primary tumor cannot be identified either, the prognosis for CUP syndrome is very poor. The syndrome is treated with the help of various therapies. When using chemotherapy, various side effects occur that can have a negative impact on the patient’s quality of life. A radiation therapy can not completely cure doing some tumors removed, but the syndrome. The further course is therefore very much dependent on the extent of the tumors and their position and can therefore not be universally predicted.
If there is no treatment for the CUP syndrome, the life expectancy of the patient will continue to be shortened enormously. The syndrome often leads to severe psychological complaints, which can also occur in the patient’s relatives.
CUP syndrome cannot be prevented by specific measures. In general, however, risk factors that can contribute to cancer should be avoided. These include an unhealthy lifestyle characterized by a sedentary lifestyle, an unbalanced diet, and excessive alcohol and / or nicotine consumption.
In CUP syndrome, medical follow-up depends on the individual health of the patient. Follow-up care is often based on the symptoms, since causal therapy is usually not possible. In the case of a causal treatment, a 3-month follow-up is carried out in the first two years after recovery.
Afterwards, checks are carried out every six months, with a focus on physical examinations. As a rule, there is no psychological follow-up. The examinations are discontinued after the fifth year if no recurrences are found in the patient. In addition to medical follow-up care, the patient must take some measures himself to ensure a full recovery.
It is particularly important to keep a complaint diary in which all unusual symptoms and any side effects of the prescribed medication are noted. Although there should be no more major health problems after the treatment has been completed, detailed documentation helps to identify any symptoms and thus to diagnose recurrences promptly.
Aftercare may also include vocational rehabilitation and building new hobbies and friendships. If the patient is treated with palliative care, therapeutic measures must be taken to deal with the fears and worries associated with the diagnosis. Talk therapy can relieve the psyche and thereby increase the patient’s quality of life.
You can do that yourself
The diagnosis of CUP syndrome brings challenges for everyday life and your own psyche. Those affected deal first with their environment, the last with themselves.
Relatives and friends are part of the environment. Together with them, we need to clarify to what extent they will help with necessary care at home. There is also the question of how easily accessible the domestic sanitary facilities are. Aids such as walking aids are to be purchased and must be usable in the apartment. Financial aspects in particular need to be reflected on. Any additional payments that may arise can be a burden. In addition, outpatient care services can be used if confidants are only able to provide care services in part or not at all.
Self-reflection is often the hardest part. Patients themselves know exactly what is important to them. Those close to you can be both a help and a burden. Please note: The psychological effects of diagnosing CUP syndrome are comparatively great because doctors cannot find a known primary tumor. The associated desperation can be discussed in self-help groups.
Some patients deal with feelings such as fear by working creatively. Pictures or sculptures, like literary texts, allow the situation to be processed. In addition, it is advisable to consult psychosocial cancer counseling centers. You can find them in many German cities. They work for free or for a small contribution towards expenses.