What checks can other children at home do to rule out the risk of malignant tumors?

What checks can other children at home do to rule out the risk of malignant tumors?

Disclaimer:  

The online Q&A service is not a recommended treatment plan. As it is not possible to understand the patient's detailed condition or conduct face-to-face diagnoses, expert opinions are for reference only. For specific treatment plans, please visit a formal hospital.

This edition of the "Expert Q&A" column features Director Dong Kuiran from the Pediatric Oncology Surgery Department of Fudan University Affiliated Children’s Hospital to answer questions.

Director Dong specializes in the standardized comprehensive treatment and surgery of pediatric embryonal malignant tumors and is skilled in diagnosing and treating neuroblastoma, hepatoblastoma, nephroblastoma, and various soft tissue tumors. He also has rich experience in minimally invasive surgical treatments for conditions such as choledochal cysts, abdominal tumors, esophageal atresia, and severe diaphragmatic hernia.

01  

Q: A 1-year-old boy has been diagnosed with alveolar rhabdomyosarcoma in the calf, with the primary site in the calf muscle. Pre-chemotherapy PET scan showed an SUV of 3.3. After one round of major chemotherapy and one round of minor chemotherapy, follow-up ultrasound and enhanced CT scans found nothing. The attending physician is uncertain whether there is metastasis. They are currently treating him according to a high-risk protocol. The child's first surgery achieved complete resection, and the doctor did not perform a margin resection. Now they are considering whether to perform a margin resection. The child's genetic tests show no issues. Are there any targeted medications available? Under what circumstances does alveolar rhabdomyosarcoma require radiotherapy? If needed, when should it start, and what type of radiotherapy is best? The child has a monozygotic twin sister; what checks can be done to rule out the risk of malignant tumors?

A: For this type of soft tissue tumor, if the first surgery was performed by a non-pediatric surgical oncologist, I would generally recommend a margin resection. Currently, there are no targeted drugs specifically for alveolar rhabdomyosarcoma, but combining it with anti-angiogenic targeted therapy may yield some results. Rhabdomyosarcoma occurring in the calf is considered a poor prognostic site; treatment protocols typically include radiotherapy scheduled for 12 weeks after chemotherapy. One may also consider brachytherapy, which is a form of localized radiotherapy. For those in good condition, proton therapy could also be attempted. This tumor is not hereditary, so the twin sister only needs routine check-ups.

02  

Q: A 16-year-old boy has been diagnosed with sacral Ewing sarcoma. He has undergone three cycles of first-line chemotherapy (VDC+IE), but the tumor has progressed. He also received two cycles of second-line chemotherapy (irinotecan + temozolomide), and the current size is 8.4×5.5×7.2 cm. We understand that if surgery is performed, the entire sacrum must be removed. My question is: for sacral Ewing sarcoma, can radiotherapy replace surgery when the surgical resection margin is too extensive?

A: Ewing sarcoma is a tumor that is sensitive to chemotherapy and radiotherapy. Generally, we recommend that patients with soft tissue masses undergo chemotherapy combined with surgery and radiotherapy for satisfactory results. There are also isolated radiotherapy treatment options for bone Ewing sarcoma. Thus, for this sacral Ewing sarcoma, if the tumor significantly shrinks after chemotherapy, it may be considered to replace surgery with radiotherapy. However, radiotherapy to the sacrum can easily affect normal pelvic tissues, which is another factor to consider. It is best to consult with a radiation oncologist.

03  

Q: A girl diagnosed with stage 3 right kidney Wilms tumor at age 2 underwent three cycles of chemotherapy followed by right nephrectomy. After seven additional cycles of chemotherapy, she has now been in remission for four months. Upon diagnosis, her alpha-fetoprotein (AFP) level was 200,000; it decreased to 73 before surgery and was 103 on the fourth day after surgery, and 68 on the tenth day post-operation. After three cycles of chemotherapy, her AFP levels were 20, 11.5, and 10.7, but they have not dropped to normal levels, making it impossible to assess remission. No biopsy was performed prior to surgery, and the tumor biopsy results post-operation indicated 99% necrosis and 1% fetal type. What could be the reasons for the suboptimal decrease in AFP after surgery? What is the standard for determining remission based on AFP levels?

A: Elevated alpha-fetoprotein can be related to the liver's growth and repair processes, and infants generally have higher AFP levels than adults. AFP is a sensitive monitoring indicator for children with hepatoblastoma, and a rapid decrease in AFP post-surgery is generally a good prognostic indicator. This 1-year-old child’s current AFP level is slightly above normal, which does not necessarily indicate a poor outcome. Continued observation is recommended. If there are concerns, testing for AFP isoforms can be performed; if the ratio is normal, it would indicate that the elevation is not tumor-related.