Primary Lung Metastases in Pediatric Malignant Non-Wilms Renal Tumors: Data from SIOP 93-01/GPOH and SIOP 2001/GPOH

Abstract

Malignant non-Wilms renal tumors (NWRT) are a small but relevant subgroup of renal neoplasms in children. In this study we analyzed corresponding data from the trials SIOP 93-01/GPOH and SIOP 2001/GPOH of the Society of Pediatric Oncology and Hematology.Data of 22 patients with NWRT and primary lung metastases were retrospectively reviewed. Analyses included epidemiology, tumor characteristics, chemotherapy, local treatment, and outcome.The following diagnoses were registered: Malignant Rhabdoid tumor of the kidney (MRTK, n=15), Renal-cell carcinoma (RCC, n=3), Clear-cell sarcoma of the kidney (CCSK, n=3), and primitive neuro ectodermal tumor (PNET, n=1). Median age of patients at diagnosis was 14 months. Overall survival was 36.36% (8/22). Of the 15 children with MRTK 3 survived, 3/3 patients with RCC, 1/3 patients with CCSK, and 1/1 patient with PNET survived. Lung metastases disappeared in 6 patients after initial chemotherapy, 6/8 patients undergoing local treatment of lung metastases (surgery, irradiation, or both) achieved complete remission. Only patients with complete clearance of lung lesions, either through neoadjuvant chemotherapy or subsequent local treatment, survived. Mean Follow up was 31 months (1-137).Survival of patients with stage IV NWRT is dismal. Complete removal of lung metastases seems mandatory for survival. An aggressive diagnostic and therapeutic approach seems justified in affected children.

Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

“…The most discriminating criteria were associated with a 33%–75% probability of non‐WT (i.e., extrapulmonary and extrahepatic metastases, lung metastases occurring before two years of age, and the total absence of visible renal parenchyma), but occurred very scarcely (less than 3% in our series). Lung metastases are commonly observed in MRTK, which occurs in young children . For this reason, the SIOP recently proposed a systematic biopsy of all renal tumors with lung metastases occurring in children under the age of two years at diagnosis .…”

“…Lung metastases are commonly observed in MRTK, which occurs in young children. [26][27][28][29][30] For this reason, the SIOP recently proposed a systematic biopsy of all renal tumors with lung metastases occurring in children under the age of two years at diagnosis. 19 In our series, one third of the nine patients positive for this criterion had MRTK, the remaining having a WT.…”

Spontaneous Regression of Pulmonary Nodules Presenting as Epstein-Barr Virus-related Atypical Infectious Mononucleosis

“…[15][16][17] The patient described herein was noted to have an evidence of EBV infection with sore throat, lymphadenopathy, and splenomegaly. He was diagnosed as having atypical IM that may be categorized as one of EBV-related LPD.…”

Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

“…Wilms tumor is the most common pediatric renal tumor and accounts for approximately 6%-7% of all pediatric malignancies [1]. Pediatric non-Wilms renal tumors (NWRTs) constitute less than 10% of all renal tumors and have significantly higher mortality rates compared to childhood Wilms tumors [2][3][4][5][6][7][8]. There are controversies about the optimal follow-up and treatment plans for this rare and heterogeneous group of tumors.…”