Clear Cell Sarcoma of the Kidney Follow-up

Pediatricians often refer patients with an abdominal mass to a pediatric general surgeon or urologist (if a known renal mass is present). These specialists should involve pediatric oncologists preoperatively. Chest CT scans should be obtained before surgery to eliminate confusion regarding areas of atelectasis that may be difficult to separate from metastases. In addition, if abnormal findings are revealed on chest CT scans, a biopsy of lung tissue can be planned at the time of surgery.

Chemotherapy may be administered on an outpatient (depending on the facilities) or inpatient basis.

Fever and neutropenia may occur, requiring hospitalization for intravenous antibiotics and monitoring.

After completing chemotherapy, patients should continue to have regular blood work and radiographic scans on an outpatient basis, which decreases in frequency over time. The ideal follow up schedule has not been established particularly for the length of follow up. Generally, these visits occur every 1-3 months for the first year, every 3-6 months for the second and third years, then yearly thereafter. Investigators in Europe and in North America have reported an increase in number of CNS recurrences of clear cell sarcoma of the kidney (CCSK). The brain should be routinely scanned with other areas (such as lungs).

Clear cell sarcoma of the kidney tumors are associated with late recurrence; the most common site of recurrence is the brain and then the lung. Unlike Wilms tumors, patients remain at risk for recurrence after 2 years posttherapy. Tumors may recur as long as 10 years after completion of treatment. However, with the recent treatment approaches, most recurrences occur within 3 years of the completion of therapy.

Treatment of patients with recurrent clear cell sarcoma of the kidney depends on initial therapy. Cyclophosphamide and carboplatin should be considered if not used initially. Patients with recurrent clear cell sarcoma of the kidney that involves the brain have responded to treatment with ifosfamide, carboplatin, and etoposide (ICE), coupled with local control consisting of either surgical resection and/or radiation. Patients with recurrent clear cell sarcoma of the kidney should be considered for treatment on available pediatric phase I and phase II clinical trials.

Patients require follow-up evaluation in a late-effects clinic and monitoring with appropriate tests because they have a single kidney. Patients are assessed for toxic effects resulting from chemotherapy, radiotherapy, or both. Follow-up visits should include renal, psychosocial, cardiac, and hormonal evaluations.

Trimethoprim-sulfamethoxazole is indicated in patients who have undergone irradiation therapy to the lung. This should continue throughout the course of treatment and for 6 months posttherapy.

Although the major therapy for cancer should occur at a center staffed by pediatric oncologists, referring physicians should continue to play an important role in the child's care throughout treatment. The referring physician can be critical in performing the first evaluation of an illness, particularly if the child lives far from the oncology center.

Patients may be referred to pediatric general surgeons and urologists.

No preventive measures for childhood cancers currently are known.

Cardiomyopathy results primarily from anthracycline (doxorubicin) use. Patients should obtain routine follow-up echocardiograms after the completion of therapy.

Patients are at risk for renal failure because they have a single kidney.

Radiation effects have decreased but, in the past, have consisted of asymmetry of the muscle mass in the back.

Secondary malignant neoplasms may arise as a result of chemotherapy (particularly alkylating agents in combination with radiotherapy).

Infertility may occur as a result of the alkylating agents.

Patients who have stage I tumors, are aged 2-4 years, and have no tumor necrosis tend to have a better prognosis.

Patients who present with distant metastases or multifocal disease have a poor prognosis, with a 50% long-term 6-year survival rate.

Treatment with doxorubicin has resulted in a 66% reduction in the tumor-related mortality rate.

Parents and patients must undergo formal chemotherapy instruction to learn about the adverse effects of medication. They must be encouraged to call with any questions and to become educated regarding the expectations of chemotherapy.

Parents must be taught regarding flushing schedules and how to maintain and care for the central venous catheter if it exits the skin and the procedure to follow if the patient develops a fever.

Patients who have undergone abdominal surgery are at risk for developing intestinal obstructions or scar tissue related to the surgery. Families must be educated to call when abdominal pain or vomiting develop that are not related to an infectious cause. All members of the pediatric oncology team also must have a heightened awareness of the risk of obstruction in these patients so that an abdominal radiograph is obtained if any suggestion of obstruction exists.

For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Renal Cell Cancer.