A case report of chest wall desmoplastic small round cell tumor in children.

INTRODUCTION: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma predominantly afflicting young males. CASE PRESENTATION: In this current report, a two-year-old boy was admitted to the hospital for the evaluation of a left chest wall mass. Imaging revealed the tumor's presence in the left chest, compressing lung tissue. Subsequently, histological analysis confirmed the DSRCT diagnosis following a biopsy. The patient underwent a comprehensive management strategy centered around surgery, successfully completing the entire treatment course without experiencing relapse during subsequent follow-up assessments. DISCUSSION: When chest wall tumors are inoperable upon initial diagnosis, a biopsy is essential to clarify the pathology and assist in the diagnostic process. If a patient is diagnosed with DRSCT and conventional chemotherapy fails with surgical resection still not feasible, timely adjustment of the chemotherapy regimen coupled with targeted drug administration can reduce the tumor, enable complete resection, and improve the overall prognosis. CONCLUSION: DSRCT is a rare malignancy associated with a generally poor prognosis. The administration of a combined treatment approach involving oral targeted medication (anlotinib), chemotherapy, radiotherapy, and aggressive surgical resection holds the potential to enhance the prognosis for pediatric patients with this condition.

Pediatric adrenocortical carcinoma: clinical features and application of neoadjuvant chemotherapy.

OBJECTIVE: To summarize the clinical characteristics of children with adrenocortical carcinoma (ACC) and preliminarily explore the indications for and efficacy of neoadjuvant chemotherapy in certain patients. METHODS: The data of 49 children with adrenocortical tumors (ACT) in the past 15 years were retrospectively analyzed, and after pathology assessment using Weiss system grading, 40 children diagnosed with ACC were included. Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 and three-dimensional (3D) reconstruction of contrast-enhanced computed tomography data were used to evaluate the response to neoadjuvant chemotherapy. RESULTS: Forty patients (17 males, 23 females) with ACC were enrolled. Abnormal hormone levels were common in children with ACC (n = 31), and in terms of clinical presentation, sexual precocity was the most common (n = 14, 35.0%), followed by Cushing's syndrome (n = 12, 30.0%). Seven of 40 children received neoadjuvant chemotherapy due to a maximum lesion diameter greater than 10 cm (n = 4), invasion of surrounding tissues (n = 2), intravenous tumor thrombus (n = 2), and/or distant metastasis (n = 2); 2 patients achieved partial response, and 5 had stable disease according to the RECIST 1.1 standard. Furthermore, 3D tumor volume reconstruction was performed in 5 children before and after neoadjuvant chemotherapy. Tumor volumes were significantly reduced in all 5 children, with a median volume reduction of 270 (interquartile range, IQR 83, 293) (range: 49-413) ml. After surgery with/without chemotherapy, the 5-year overall survival rate for all children was 90.0% (95% CI-confidence interval 80.0-100.0%), and the 5-year event-free survival rate was 81.5% (95% CI 68.0-97.7%). CONCLUSION: In the diagnosis and treatment of pediatric ACC, a comprehensive endocrine evaluation is necessary to facilitate early diagnosis. Surgery and chemotherapy are important components of ACC treatment, and neoadjuvant chemotherapy should be considered for children with ACC who meet certain criteria, such as a large tumor, distant metastases, or poor general condition.

Hyperthermic Intraperitoneal Chemotherapy for Recurrent Nephroblastoma in Children.

CONCLUSION: For children with recurrent nephroblastoma, intraoperative HIPEC has little impact on the body, can significantly improve the effectiveness and reduce the recurrence rate, and does not increase the adverse reactions. KEY WORDS: Children, Recurrence, Nephroblastoma, Hyperthermic perfusion. METHODOLOGY: Sixty children with recurrent nephroblastoma treated by HIPEC in the Department of Surgical Oncology were analysed and divided into group A and group B, according to different perfused drugs. Additionally, 30 children without a history of HIPEC were selected as the control group (group C). The changes in routine blood indices, albumin, and hepatic and renal function of the three groups were observed before and after treatment. The clinical efficacy, frequency of adverse reactions, as well as 6-month and 1-year tumour recurrence in the three groups were compared. OBJECTIVE: To investigate the clinical efficacy and safety of hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of recurrent nephroblastoma in children. PLACE AND DURATION OF STUDY: Department of Oncology, Baoding Children's Hospital, from August 2018 to November 2021. RESULTS: The efficacy in groups A and B was significantly higher than that in group C (p<0.05). Changes in routine blood indices, albumin, and hepatic and renal function showed no statistically significant differences among the three groups during each observation period after treatment (all p>0.05). No significant differences were found in the incidence of adverse reactions among the three groups during treatment (all p>0.05). Six months after treatment, the tumour recurrence rate presented no significant differences among the three groups. However, at 12-months after treatment, the recurrence rate in groups A and B was lower than that in group C (p<0.05). STUDY DESIGN: Randomised controlled trial.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.

Background: Peritoneal malignant tumors in children are rare but commonly associated with disease progression and poor outcome. The successful treatment experience of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in adult peritoneal carcinoma has been applied to pediatric peritoneal malignancy in recent years. However, patients with desmoplastic small round cell tumor (DSRCT) accounted for the majority of patients treated with CRS and HIPEC in previous studies. The role of CRS and HIPEC remains controversial due to the rarity of the disease and the limited sample size of studies. Additionally, the cases using CRS and HIPEC except DSRCT were mainly small case reports with unclear outcomes. We present our experience in the treatment of pediatric peritoneal malignancies using CRS and HIPEC, with more emphasis on the safety, feasibility, and short-term outcome. Methods: A retrospective query from December 2019 to February 2022 identified 19 children with peritoneal malignancies who underwent CRS and HIPEC in our institution. Clinical characteristics, therapies, and outcomes were summarized and analyzed. Results: The median age of the patients was 6.4 years (range, 0.7-13.9 years). The histologic types included rhabdomyosarcoma (7), Wilms tumor (2), clear cell sarcoma of the kidney (2), undifferentiated sarcoma (2), immature teratoma (1), peritoneal serous carcinoma (1), malignant rhabdoid of the kidney (1), malignant germ cell tumor (1), neuroblastoma (1), and epithelioid inflammatory myofibroblast sarcoma (1). Seven patients underwent initial operation, and 12 patients received reoperation for tumor recurrence. The median peritoneal carcinomatosis index was 5 (range, 2-21). There were no perioperative deaths or life-threatening complications of CRS and HIPEC. Two patients had grade 3 complications of wound infection and wound dehiscence. With a median follow-up time of 14 months (range, 1.5-31 months), 14 patients were alive, and 5 died of tumor recurrence. Of the 14 patients who were alive, 2 relapsed after CRS and HIPEC and then received radiotherapy and molecular-targeted therapy or chemotherapy. Conclusions: CRS and HIPEC are safe and feasible in children, without increasing serious complications in the peri- and postoperative periods. The complication is acceptable. The short-term outcome shows possible effectiveness in pediatric peritoneal malignant tumors. The long-term effectiveness needs to be verified by additional cases and long-term follow-ups.

Congenital Bilateral Wilms Tumor: A Case Report.

Compare to congenital mesoblastic nephroma in fetus, congenital wilms tumor is extremely rare. Herein we report a case of congenital bilateral solid masses on antenatal ultrasound. The mass was evaluated by ultrasonography and contrast computed tomography scan in postnatal period, and the patient was undergoing tumor enucleation separately in short period after neoadjuvant chemotherapy. The diagnosis was confirmed by histology analysis for each side, and the treatment was taken according to the International Society of Pediatric Oncology.