[A long-term follow-up report of pediatric relapsed Wilms tumor after retreatment].

Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children's Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist's assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively.Survival data were analyzed by Kaplan-Meier.Log-Rank analysis was used for univariate analysis. Result: One case was excluded because of giving up the therapy even though no disease progress was identified. A total of 15 cases (5 males and 10 females) were included in this study. The median age at diagnosis was 3.8 years (range 0.5-9.1 years). The tumor staging at diagnosis included one case of stageⅠ, 7 cases of stageⅡand 7 cases of stage Ⅲ. Among cases of stage Ⅲ, 6 cases had radiation therapy history. The pathology of all patients' recurrent tumor was favorable histology (FH). The median follow-up time was 34.6 months (range 12.5-132.7 months) until March 21, 2017. The time from initial diagnosis to relapse was 7.9 months (range 3.1-17.9 months). Four cases experienced local recurrence, 9 cases relapsed with metastases (6 cases in lungs, 2 in livers, 1 in mediastinum) and 2 cases relapsed in both local site and with metastases. Except to 2 cases received irregular retreatment, 13 cases received regimen I (doxorubicin, vincristine, epoposide and cyclophosphamide for 25 weeks) as relapsed chemotherapy. Five cases received autologous bone marrow transplantation (ABMT). Until the last follow-up, 8 cases achieved continuous complete remission (range 6.7-104.3 months), 3 cases had relapse again or progressing and 4 cases died. The estimated 5-year overall survival (OS) rate and event free survival (EFS) rate were (70±15)% and (52±15)%. According to whether received ABMT or not, the 5-year EFS rate were 51% and 53%. According to whether relapsed within 6 months after diagnosis or not, the 5-year EFS rate were 38% and 56% respectively. Conclusion: The 5-year EFS rate of pediatric relapsed FH WT have reached above 50% by multi-disciplinary treatment in our experience and we encourage patients and doctors to receive retreatment.

[A follow-up report of childhood hepatoblastoma from 74 cases in a single center].

Objective: To investigate the efficacy and the prognostic factors in pediatric hepatoblastoma according to the standard diagnostic and therapeutic regimen. Method: Eighty-four consecutive patients were enrolled in this study between June 2000 and June 2015. Diagnosis and staging was decided by the multi-disciplinary team including oncologists, surgeons, pathologists and sub-specialized radiologists refering to protocol of Children's Oncology Group(COG) and International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL) in a case observational study. Univariate analysis was tested by the log-rank and multivariate analysis by COX regression. All consecutive cases were divided into low risk group and high risk group according to grouping criteria. Complete remission was defined as both imaging negative and α fetoprotein (AFP) normalization. Retrospective analysis was performed in clinical features, long-term outcomes and prognostic factors. Result: Ten patients were excluded because of giving up after less than or equal to three cycles of treatment. A total of 74 cases were included in this study; 45 males and 29 females. The median age at diagnosis was 1.7 years(range 0.2-14.8 years). Untill August 30, 2016, the median follow-up time was 24.2 months (range 4.1-135.3 months); 59 cases achieved complete remission.The estimated five years overall survival (OS) and event free survival(EFS) were 90%(68/74)and 72%(58/74). AFP could be normalized after 5 circles of treatment or 2 circles of postoperation.In univariate analysis , the five years OS and EFS in low risk group were both 100%(18/18), and those in high risk group were 88%(50/56)and 68%(40/56), respectively. The five years OS rates were 75%(15/19) and 95%(53/55) in patients with or without distant metastasis (P=0.016). After 3 cycles of chemotherapy post tumor resection, we divided these patients into 2 groups according to AFP recover or not, the five years OS were 100%(43/43)and 81%(22/26), respectively (P=0.011). Conclusion: The result of this protocol is reasonable when comparing with other worldwide research. Except for staging, metastasis, pathological subtypes, postoperative AFP recover or not is a prognostic factor after 3 cycles of chemotherapy.

[Clinical analysis of 51 cases with rare childhood soft tissue sarcomas].

Objective: To analyze the clinical characteristics and prognosis of rare soft tissue sarcomas. Method: Clinical data of 51 patients with rare soft tissue sarcomas including fibrosarcoma, synovial sarcoma, extrarenal rhabdoid tumor, alveolar soft part sarcoma, desmoplastic small round cell tumor and undifferentiated sarcoma in children and adolescents, diagnosed at Shanghai Children's Medical Center from June 1998 to December 2013, were retrospectively analyzed. All types were treated with the same strategy and chemotherapy regimens. Their clinical features, treatment and prognosis were discussed. Result: Seventeen patients with fibrosarcoma, 10 with synovial sarcoma, 9 with extrarenal rhabdoid tumor, 6 with alveolar soft part sarcoma, 3 with desmoplastic small round cell tumor and 6 with undifferentiated sarcoma were included. The mean age at initial diagnosis was 5 years(range from 1 month to 13.5 years). The most common primary site of tumors was limbs, followed by the thoracic and abdominal cavity, accounting for 41% and 24% respectively. Twelve cases presented distant tissue or organ involvement in which bone metastases occupied the first place. Seven cases(accounting for 14%)were at stage Ⅰ, 13 cases were at stage Ⅱ(accounting for 25%), 19 cases were at stage Ⅲ(accounting for 37%) and 12 cases were at stage Ⅳ(accounting for 24%). The median follow-up period was 36 months(range from 1 month to 123 months). Forty-four patients achieved complete remission and 3 patients achieved partial remission after initial treatment, the overall response rate was 92%. Subsequent follow-up showed 29 patients remained relapse-free while 13 patients had relapsed disease. Overall survival and event-free survival at 2 years were 88% and 57%.Postoperative surgical staging was the main prognostic factors. Patients with stage Ⅲ+ Ⅳ had poorer results than those with Ⅰ+ Ⅱ (χ2=4.909, P=0.027). Conclusion: These 6 types of soft tissue sarcomas are rare in children and adolescents. The tumor can occur anywhere in the body but commonly presents in the extremities. Complete resection of tumor remains the most important modality of treatment and is directly related to prognosis. Neoadjuvant chemotherapy helps improve the resection rate of some unresectable tumors at diagnosis. Radiation therapy is primarily adopted for focal tumor control.