Paraneoplastic Syndrome

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Classification according to ICD-10
C80 Malignant neoplasm without specifying the location
Paraneoplastic syndrome
ICD-10 online (WHO version 2019)

A paraneoplastic syndrome , also called paraneoplasia ( para - Greek next , neo - Greek new , plasia - Greek education ) or paraneoplastic disease , is understood to mean accompanying symptoms of cancer (neoplasia) that are not primarily caused by the neoplasm ( solid tumors or leukemia ) . The term encompasses all symptoms that are neither caused by the space required by the tumor nor by the destruction of tissue through the ingrowth of neoplastic cells and which are often typical of cancer at the same time.

Paraneoplastic syndromes usually occur in the area of ​​the glands (as in endocrinopathy ), the nervous system (as in polyneuropathy ), the skin, the joints or the blood-forming system or coagulation system. Cancer diseases in which paraneoplastic syndrome occurs more frequently: small cell lung cancer, breast cancer, cancer of the female genital organs, blood cancer.

The cause can be an immune reaction of the body that was provoked by the cancer or a release of messenger substances ( cytokines and hormones ) caused by the tumor .

Some paraneoplastic syndromes are so characteristic that if they occur without an existing tumor diagnosis, all available diagnostic methods should be used to search for the neoplasm; in some syndromes, for example, a tumor in the lungs can be suspected.

A large number of paraneoplastic diseases are now known. The compilation in this article is therefore far from complete.

history

The term "paraneoplastic" was first used by Guichard and Vignon in 1949 to describe multiple nerve failures in a patient with cervical cancer.

General paraneoplastic symptoms

Cancer sufferers are often accompanied by symptoms such as anorexia , tumor cachexia , hyperthermia , taste disorders, night sweats (see B symptoms ) as well as anemia , leukocytosis and thrombosis . Insofar as these occur individually, one must correctly speak of paraneoplastic symptoms, since syndromes always make up an interplay of several symptoms.

Special paraneoplasms

Special paraneoplasms are caused by the production of hormone-like substances or immunological mechanisms. Special paraneoplastic syndromes occur in 2 to 15% of cancer patients, most frequently in small cell lung cancer (up to 40%).

Endocrinological paraneoplastic syndromes

The production of a hormone or hormone precursors by tumor tissue simulates the presence of an endocrinological disease . The exact causes are still unknown.

Examples of endocrinological paraneoplasias

Antibody-mediated paraneoplasms

Antibody- mediated paraneoplasias arise when the immune system produces antibodies against the tumor, but these antibodies also attack healthy tissue at the same time ( cross-reactivity ). Removal of the tumor does not necessarily stop the immune response and the antibodies that are still produced can continue to damage healthy tissue. However, the prognosis of patients with antibody-associated paraneoplastic syndromes is in many cases better than the prognosis of patients without antibodies against the tumor, because on the one hand the antibodies develop their own disease value, on the other hand they also fight the tumor. Experiments are currently being carried out with immunosuppressive therapies .

Examples of antibody-mediated paraneoplasms

blood

Skin / muscles

Gastrointestinal tract

Brain / nervous system

eyes

Other paraneoplasms

The skin can be damaged by hyperpigmentation , hyperkeratosis , hypertrichosis , acanthosis nigricans , erythema gyratum or bullous pemphigoid as well as hypertrichosis as in the Herzberg-Potjan-Gebauer syndrome , the heart by "non-bacterial thrombotic endocarditis ", the vascular system by repeated thromboses (in pancreatic cancer) and the kidney may be affected by glomerulonephritis . On the skeleton, hypertrophic osteoarthropathy is often paraneoplastic in nature.

treatment

The therapy of paraneoplastic syndromes consists primarily in the treatment of the underlying tumor disease. If there is no or insufficiently rapid treatment option, additional or alternative forms of treatment that alleviate the symptoms can be used.

See also

literature

Individual evidence

  1. ↑ Lorraine C. Pelosof, David E. Gerber: Paraneoplastic syndromes: an approach to diagnosis and treatment . In: Mayo Clinic Proceedings . tape 85 , no. 9 , September 1, 2010, p. 838-854 , doi : 10.4065 / mcp.2010.0099 , PMID 20810794 , PMC 2931619 (free full text).
  2. ^ A. Guichard, G. Vignon: La polyradiculonévrite cancéreuse métastatique; paralysies multiple des nerfs craniens et rachidiens par généralisation microscopique d'un épithélioma du colutérin . In: Journal De Médecine De Lyon . tape 30 , no. 700 , March 5, 1949, pp. 197-207 , PMID 18115298 .
  3. ↑ Roland Kath and others: Treatment of paraneoplastic syndromes. 2012.