Wilms tumor treatment

The treatment of Wilms tumor consists of a multimodal approach, including surgery, chemotherapy and radiation therapy. All patients undergo nephrectomy and cytostatic therapy. The question of the optimal sequence of surgery and chemotherapy remains controversial. Radiation therapy is performed in the adjuvant mode, with a high prevalence of the tumor process, as well as in the presence of adverse factors of disease progression. The treatment of Wilms tumor is determined based on the stage of the disease and tumor anaplasia.

In North America, the standard approach to treating Wilms tumor is immediate nephrectomy followed by chemotherapy with or without post-operative radiotherapy.

Treatment of Wilms tumor depending on the stage and histological structure of the tumor

Stage of tumor	Histology	Operation	Chemotherapy	Radiation therapy
I, II	Favorable	Nephrectomy	Vincristine, dactinomycin (18 weeks)	No
I	Anaplasia
III, IV	Favorable	Nephrectomy	Vincristine, dactinomycin, doxorubicin (24 weeks)	Yes
II, III, IV	Focal anaplasia
II, III, IV	Diffusive anaplasia	Nephrectomy	Vincristine, doxorubicin, cyclophosphamide, etoposide (24 months)	Yes

* Total focal doses: on the bed of the removed kidney - 10.8 Gy. On all lungs in patients with metastases in the lungs -12 Gy.

In Europe, patients with Wilms tumor receive pre-operative chemotherapy with vincristine and dactinomycin followed by nephrectomy and post-operative treatment, whose regimen does not differ significantly from the North American standards given above. The European protocol for the treatment of Wilms tumor involves the use of higher doses of radiation (15-30 Gy).

Chemotherapy, used in the Wilms tumor, is based on regimens with the inclusion of dactinomycin. Vincristine and doxorubicin as first-line therapy and cyclophosphamide, etoposide - as a salvage treatment for Wilms tumor with tumor recurrence or in patients with a poor prognosis group. Doses of antitumor drugs depend on the stage and surface area of the child's body.

Calculated doses of antitumor drugs used in the Wilms tumor

Stage	A drug	Dose
I	Dactinomycin	1000 μg / m 2
	Vincristine	1.5 mg / m 2
II	Dactinomycin	1000 μg / m 2
	Vincristine	1.5 mg / m 2
	Doxorubicin	40 mg / m 2
	Cyclophosphamide	100 mg / m 2
	Etoposide	400 mg / m 2
III	Dactinomycin	1.2 mg / m 2 (not more than 2 mg)
	Vincristine	1.5 mg / m 2
	Doxorubicin	50 mg / m 2
	Cyclophosphamide	600 mg / m 2
	Etoposide	100 mg / m 2
IV	Dactinomycin	1.2 mg / m 2 (not more than 2 mg)
	Vincristine	1.5 mg / m 2
	Doxorubicin	50 mg / m 2
	Cyclophosphamide	600 mg / m 2
	Etoposide	100 mg / m 2

Further management

With the purpose of timely detection and adequate treatment of Wilms tumor, tumor recurrence for all patients is carefully monitored, the frequency and type of which are determined by the stage and histological structure of the tumor.

Tactics of dynamic observation of patients with Wilms tumor

Stage and histological structure	Type of examination	Mode
All patients	Chest X-ray	6 weeks and 3 months after the operation, then every 3 months (5 times), every 6 months (3 times), annually (2 times)
Stages 1 and II, favorable histological structure	Ultrasound of the abdominal cavity and retroperitoneal space	Annually (6 times)
Stage III, favorable histological structure	Ultrasound of the abdominal cavity and retroperitoneal space	6 weeks and 3 months after the operation. Then every 3 months (5 times). Each 6 months (3 times), annually 12 times)
All stages, unfavorable histological structure	Ultrasound of the abdominal cavity and retroperitoneal space	Every 3 months (4 times), then every 6 months (4 times)

Prognosis of Wilms tumor

The prognosis in children suffering from Wilms tumor is favorable. Three-modal treatment of Wilms tumor leads to recovery of 80-90% of cases.

With a favorable histological variant of the tumor, the total and disease-free survival of patients with stage I is 98 and 92%, stage II - 96% and 85%, stage III - 95% and 90%, stage IV - 90% and 80%, respectively.

Patients with synchronous bilateral tumors have a long-term survival rate, reaching 70-80%, metachronous 45-50%. Wilms tumor with relapses has a moderate prognosis (overall survival is 30-40%).