Uribe RK1*, Ruiz AN2, Morante DC3, Cruz R4, Hernández E5, Garcia LJ5, García EM6 and Destefano UV3
1Department of Surgical Oncology, National Cancer Institute (INEN), Peru
2Urology Oncology Fellow, National Cancer Institute (INEN), Peru
3Department of Urological Surgery, National Cancer Institute (INEN), Peru
4Pathology Oncology Fellow, National Cancer Institute (INEN), Peru
5Department of Pediatric Oncology, National Cancer Institute (INEN), Peru
6Department of Clinical Oncology, Regional Hospital of Lambayeque, Peru
Background: Rhabdoid kidney tumors are a rare infant entity. We present a case series of 10 patients with this diagnosis in a period of 19 years at the National Cancer Institute of Peru.
Methods and Results: Descriptive and retrospective study. We analyzed patients with rhabdoid kidney tumor in the Department of Urological Surgery of the National Cancer Institute (Instituto Nacional de Enfermedades Neoplasicas: INEN) in Lima-Peru, from January 2000 to December 2019. The results: 10 cases were reported with anatomopathological diagnosis of Rhabdoid kidney tumor.
According to clinical characteristics: Seven male patients (70%) and three females (30%), the proportion of population related to age group was: Seven infants (between 1 to 23 months) (70%), two preschool (between 24 to 71 months) (20%), one child (between 6 to 12 years) (10%). About symptoms six patients present hematuria (60%), three patients with palpable tumor mass (30%) and one patient with fever (10%). Paraneoplastic hypercalcemia was infrequent with two cases (20%). According to surgical characteristics: Left kidney was the most frequent presentation in six cases (60%). Average tumor size was 10 cm. Of the ten patients, surgical treatment was made in nine patients (90%), of which seven (70%) had primary surgery, two (20%) had surgery post neoadjuvant treatment and one case did not have surgical treatment (due to progressive disease). R0 in seven cases (70%) and R1 in two cases (20%). Related to multimodal treatment, at the chemotherapy arm, eight patients (80%) received systemic chemotherapy; three of them (30%) had neoadjuvant therapy, two of them had complementary surgery (of these two patients, one had complementary adjuvant chemotherapy and the other one surgical management post neoadjuvant treatment without adjuvant treatment); the other one patient did not have surgical management in relation of progressive disease. The remaining five patients (50%) had adjuvant treatment posterior primary surgical management. One patient had adjuvant
radiotherapy (10%) and one patient (10%) had surgical management without complementary treatment. Prevalent clinical stage was III in five cases (50%), and medial global survival of seven months.
Conclusion: This entity is predominantly in young children, most of cases with advanced stage disease and poor survival even if the diagnosis is on early stage.
Rhabdoid kidney tumor; Children kidney cancer; Radical nephrectomy
Uribe RK, Ruiz AN, Morante DC, Cruz R, Hernández E, Garcia LJ, et al. Case Series: Rhabdoid Kidney Tumor and Literature Review. Clin Oncol. 2021;6:1834..