CLEAR CELL SARCOMA OF THE KIDNEY - CCSK

DISCLAIMER: Be aware that I'm not trained in medicine and any pronouncements I may make should be treated with appropriate caution
The information is intended to help you interpret information provided by trained medical staff

This page is under review and may contain factual errors..
Advice gratefully received from professionals and mortals alike.;-)

"Clear cell sarcoma (CCSK) of the kidney is not a Wilms' tumor variant, but it is an important primary renal tumor associated with a significantly higher rate of relapse and death than favorable histology Wilms' tumor". [ref]

That isn't as bleak as it sounds. The comparison is with a form of cancer which generally responds excellently, and CCSK survival rates are improving all the time.

THESE NEXT SECTIONS ARE CURRENTLY BEING REVISED Primary CCSK tumours occur in the same tissues as Wilms', but have a different genetic origin: Rather than a section of chromosome getting deleted, a piece of one chromosome gets spliced into another. It occurs twice as often in boys than girls and is most commonly, (50%), diagnosed between the first and third birthdays, (boys earlier than girls), [Argani, Perlman, et al]. It is not thought to run in families, [ibid].

The relatively common occurrence of the majority Wilm's type has let the medical profession gain the experience and reliable statistics needed to give the young victims a good chance of survival:
"About 70 children develop a Wilms' tumour each year in Britain", (pop. ca.55mil), [CCLG (was UKCCSG)], spread round 22 specialist units.

About 10 per cent of these are of "unfavourable histology", of which group clear cell sarcoma is only one of three types, amounting to 2.8% of all cases submitted prior to Aug '95 on National Wilms' Tumor Studies 1 through 4, [ref]. It is therefore not difficult to see why most UK paediatric oncology staff will not have met many, if any, CCSK victims before; and why major authorities on Wilm's etc. admited that the management of the rarer variants "lags disappointingly behind", [exact reference to be added].

According to Argani, Perlman, et al. there was a "view that CCSK disseminates early so that apparent low-stage patients have micrometastases at presentation", [ref]. In other words: Any sites of spreading are too small to detect at diagnosis, which is why "all patients with CCSK on NWTS trial 5 are now treated with doxorubicin regardless of stage", [ibid]. However, survival rates are puzzlingly good for very early stage disease. This tumour is most likely to relapse, (come back), in the bones and lungs and near to the original site. To a lesser extent it can recur in the brain, liver and general "soft tissue", [ibid]. Most relapses by far, (80%), will happen within three years of diagnosis though later relapses have been reported, [ibid].

On National Wilms Tumor Study (NWTS) 5, a new protocol has been introduced to reflect the more aggressive nature of the tumour. This augments the Doxorubicin/Vincristine combination, replacing Actinomycin-D regimen with Cyclophosphamide and Etoposide; DOX appearing to be the most important element in the chemotherapy . END OF SECTIONS BEING REVISED

Here is a glossary of terms, abbreviations and drug names The NWTS now also has a glossary.

RESOURCES:

• UKCCSG Online Parents guide, (+ mirror)
• CancerBACUP Wilms' section
• CancerNet
• Oncology glossary at CancerPoint
• Body Surface Area (BSA) Calculator from www.halls.md
  Included here for those curious about how drug doses are worked out.