anybody know anything about ccsk?

Question

my friend was diagnosed recently, shes a lot older than most who get it [shes 14]. i have no idea what to expect. if anybody knows anything about ccsk [clear cell sarcoma of the kidney] i would really appreciate some information...especially if its about people her age because i cant find anything thats not written in medical words that i cant understand

Answer 1

Treatment for this disease depends upon the stage of the cancer at diagnosis. A stage 1 cancer is often treated differently than a stage 4 . . with stage 4 being the most difficult, but not impossible to treat. CCSK is generally more difficult to treat than Wilms tumor, but each patient is an individual. CCSK is treatable and survival rates have increased.

"Clear cell sarcoma of the kidney ([CCSK] the 8-year relapse-free survival rate for localized CCSK stages I-III is 88%, but late relapses have been known to occur

Nephrectomy, abdominal radiation using 1,080 cGy for all patients, whole-lung radiation for patients with pulmonary metastasis, and 24 weeks of chemotherapy with vincristine, doxorubicin, etoposide, and cyclophosphamide."

My son was diagnosed at 17 with a stage IV abdominal sarcoma that is considered a cross between Wilms tumor and Ewings sarcoma. He does not have CCSK, but his cancer is very rare so we understand what you are going through. There is also a disparity between childhood cancers and adult cancers . . he is an older adolescent, but is being treated by pediatric oncologists. Teenagers have more difficulties being treated because they are neither children or adults. The teen group has only recently become recognized as a neglected group when it comes to cancer. There is some progress being made into treating adolescent patients as a separate group from pediatrics and adult cancers.

You should check with the Dana Farber institute which sponsors an online teen support group called 'Plantet Cancer' http://www.planetcancer.org/

Teens Living with Cancer
http://www.teenslivingwithcancer.org

Group Loop
http://www.grouploop.org

As for the specific cancer, you can find information at the National Cancer Institute that describes the various childhood kidney cancers including CCSK:
Clear Cell Sarcoma of the Kidney
http://www.cancer.gov/cancertopics/pdq/treatment/wilms/HealthProfessional/page11

Good luck.

Answer 2

I don't know if this will help but try going onto the Mayo Clinic website. You can get a lot of answers there. I have used it many times and it helped me to learn about non-hodkins lymphoma that my husband had and some weird stuff I had.
I think it is www.mayoclinic.com
Just type it into your web search.
I hope your friend will be alright. I'm rooting for her.

Answer 3

Medical Care: The approach for treating clear cell sarcoma of the kidney (CCSK) is different from the approach for Wilms tumor because the overall survival of children with CCSK remains considerably lower than that of patients with favorable-histology Wilms tumor. In the NWTS-3 study, the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival in patients with CCSK.

NWTS-4 showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival rate compared with patients treated for 6 months (87.5% vs 60.6% at 8 y). The overall survival has improved for patients with CCSK from NWTS-3 to NWTS-4 (83% vs 66.9% at 8 y). The 8-year relapse-free survival rate for localized CCSK stages I-III is 88%, but late relapses have been known to occur. In the NWTS-5 protocol, patients with all stages of CCSK are treated with the same regimen used in patients who have Wilms tumor with diffuse anaplasia (excluding stage I); this treatment consists of a radical nephrectomy followed by radiotherapy and chemotherapy with cyclophosphamide, etoposide, vincristine, and doxorubicin for 24 weeks.

In the NWTSG series that was reviewed by Argani et al, a better prognosis was indicated in the subset of patients with CCSK that was characterized by stage I tumors in patients aged 2-4 years in whom no tumor necrosis was identified.

In the current Children's Oncology Group protocol, all patients with CCSK, except patients with stage IV, continue treatment as in NWTS-5. However, patients with stage I who undergo lymph node sampling do not undergo radiation therapy to the tumor bed. Any patient with stage I who has not undergone lymph node sampling is upstaged to stage II. Patients with stage IV undergo treatment with irinotecan and vincristine in an upfront window approach before treatment with cyclophosphamide, etoposide, vincristine, doxorubicin, and cyclophosphamide.

Surgical Care: At presentation, radical nephrectomy is the initial treatment of choice if the lesion is resectable. If any question exists regarding the size or extension of the lesion, a biopsy is performed and chemotherapy is administered, followed by surgical resection after a response has been obtained.

Consultations:

Radiotherapist: Once the tumor has been resected, the tumor bed and any other sites of disease are irradiated.
Pediatric oncologist: Primary care physicians should consult with a pediatric oncologist to determine standard and investigational treatment protocols.
Diet: No special diet is required.

Activity: Patients with CCSK are advised not to participate in contact sports, especially football. Other activity recommendations are made on an individual basis.