teratoider, rhabdoider Hirntumor

In meinem Freundeskreis wurde bei einem 5 jährigen Jungen ein teratoider, rhabdoider Hirntumor diagnostiziert. Ich würde mich sehr gern über diese Tumorart informieren, jedoch finde ich im Internet keinerlei Informationen über diesen sehr seltenen Tumor. Kann mir jemand diesbezüglich helfen.

Hierzu ein (leider nicht so ermutigender) Artikel der Arbeitsgruppe meiner Frau:

Childs Nerv Syst. 2000 Apr;16(4):228-34.


Rhabdoid tumors of the central nervous system.

Reinhardt D, Behnke-Mursch J, Weiss E, Christen HJ, Kuhl J, Lakomek M, Pekrun A.


Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherapy. Four of these were treated in addition with both neuroaxis radiotherapy and a local boost directed to the tumor region, while two patients received local radiotherapy only. The therapy was reasonably well tolerated in most cases. Despite the aggressive therapy, eight of the nine patients died from progressive tumor disease, and one patient died from hemorrhagic brain stem lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the fatal prognosis of central nervous system rhabdoid tumors. As these neoplasms are so rare, a coordinated register would probably be a good idea, offering a means of learning more about the tumor´s biology and possible strategies of treatment

Gruß

PD Dr. Mursch
Neurochirurgie
Zentralklinik Bad Berka

Im Internet kannst du dich informieren z.B. bei:
1. http://www.pubmed.org dort z.B. als Suchbegriff eingeben: teratoid rhabdoid. Dort sind sehr viele Zusammenfassungen von medizinischen Fachartikeln zu finden, halt alles englisch.
2 http://www.virtualtrials.com/ -> Find a treatment -> keyword search und dort eingeben: teratoid oder rhabdoid. Ist auch eine gute Seite.
3. Einer online Diskussionsliste z.B: http://health.groups.yahoo.com/group/Pediatricbraintumors/
Viele Grüsse Peter


In meinem Freundeskreis wurde bei einem 5 jährigen Jungen ein teratoider, rhabdoider Hirntumor diagnostiziert. Ich würde mich sehr gern über diese Tumorart informieren, jedoch finde ich im Internet keinerlei Informationen über diesen sehr seltenen Tumor. Kann mir jemand diesbezüglich helfen.

http://www.kinderkrebs-hamburg.de/fi/fobefi05.pdf

Vielleicht hilft Dir dieser Link weiter.

Liebe Grüße
Anne

Brain Tumor: Atypical Teratoid / Rhabdoid Tumor (ATRT)
Alternative Names: ATRT

Definition

This rare, high-grade tumor occurs most commonly in children younger than 2. It is generally found in the cerebellum, which is the lower, back part of the brain that controls balance. These tumors tend to be aggressive and frequently spread through the central nervous system.


Incidence

This diagnosis has been classified only in the last three to five years; the tumor is a subset of medulloblastomas. They occur in about 1-2 percent of children with brain tumors.


Survival Rates

Even after surgery and chemotherapy treatment, the survival rate for children younger than 3 at diagnosis is less than 10 percent. It appears that older children, when treated with chemotherapy and radiation therapy after surgery, do somewhat better long-term, nearing 70 percent.


Treatment Strategies

Treatment generally involves surgical removal of the tumor followed by chemotherapy. Radiation therapy may be considered depending on the age of the child and whether the tumor has recurred.


Current Research

Researchers are investigating new, more effective methods of treating brain tumors of infants and young children. High doses of radiation can't be used because it may cause permanent problems with thinking, learning, and growing when given to very young children. It has been standard therapy to administer chemotherapy in an attempt to delay giving radiation therapy until the child is older and thus giving the brain more time to develop. However, chemotherapy alone has not been effective in fighting brain tumors.

New radiation techniques that minimize damage to healthy tissue that surrounds brain tumor tissue are under investigation.

Stem cell transplantation as a part of treatment continues to be under study.

Scientists continue to study chromosomal abnormalities, genes, and proteins that may have a role in the development and metastasis (spread to other parts of the central nervous system) of pediatric brain tumors.

Clinical trials are underway to develop chemotherapy drugs effective against this tumor.