RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.
Adjuvant rIFNalpha2b is not indicated after radical nephrectomy for renal cell carcinoma. The protective effect in the small group of pN2/pN3 patients requires further investigation.
Background Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. Procedure Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. Results The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event‐free, relapse‐free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. Conclusions Teratomas show a good prognosis, especially the mature ones: surgery and follow‐up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses. Pediatr Blood Cancer 2015;62:1202–1208. © 2015 Wiley Periodicals, Inc.
This study confirms the rarity and poor prognosis of CRC in children and adolescents: advanced stage and an aggressive biology are hallmarks of this tumor in pediatric age, while clinical findings and outcome in young adults seem more similar to those observed in adult series. Therapeutic recommendations should stay the same as for adults. Surgery remains the mainstay of treatment and early diagnosis is crucial: it is important for pediatricians to be aware that CRC does occur in children, in order to refer suspected cases to expert physicians professionally dedicated to the management of this cancer in adults.
Although rare in children and adolescents, renal cell carcinomas (RCCs) raise important questions concerning the best treatment approach and accurate pathologic classification. The differences emerging between childhood and adulthood RCC probably prevent any direct generalized application of therapies to children that are validated for adults. The translocation type of RCC, which forms a distinct category characterized by translocations involving Xp11.2 or, less frequently, 6p21, has recently emerged as the predominant type of RCC in children and adolescents, whereas it is rarely diagnosed in adults. This new finding emphasizes how important it is to prospectively classify RCCs in children with standardized 'modern' diagnoses. The standard cornerstone of therapy for RCC in children and adolescents remains radical nephrectomy. Nephron-sparing surgery is currently recommended in adults for selected small-volume tumors, but additional data are needed before this experience can be extensively transferred to the pediatric population. The therapeutic value of complete retroperitoneal lymph node dissection is still controversial, especially in patients without suspected nodal involvement, be they adults or children. The backbone of systemic therapies for adult RCC has recently been changed by the introduction of drugs designed to target tumor-related angiogenesis and signal transduction. It is worth noting that the largest clinical efficacy trials on targeted molecules have been conducted on clear-cell RCC. While targeted drugs have become the standard of care for adult metastatic RCC, there are currently no published reports on their role in children, and their use should be considered for patients with unresectable metastatic or advanced-stage RCC. On the other hand, the utility of targeted therapies in the adjuvant setting remains to be seen for both adults and children.
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