[Laparoscopic nephrectomies and kidney resections in children]. / Nachal'nyi opyt laparoskopicheskikh nefrektomii i rezektsii pochek u detei.

OBJECTIVE: To present the experience of laparoscopic nephrectomies and kidney resections in children. MATERIAL AND METHODS: There were 28 minimally invasive surgeries for renal tumors between July 2015 and March 2023 (92 months). There were 16 (57%) boys and 12 (43%) girls who underwent 22 nephrectomies and 6 kidney resections. The median age of patients was 54 (38; 76.5) months. RESULTS: In the laparoscopic nephrectomy group, the median surgery time was 135 (108-188) min, blood loss - 10 (3.75-15) ml. Total resection was confirmed in all patients. In the group of minimally invasive kidney resections, these values were 182.5 (157.5; 265) min and 50 (42.5; 117.5) ml, respectively. Histological examination confirmed total resection in all patients. In both groups, none patient developed postoperative complications. Event-free survival was 86.72% with a median follow-up of 82 months, and local recurrence-free survival was 95.8% with a median follow-up of 89.8 months. CONCLUSION: Minimally invasive nephrectomies and resections are safe in children in case of careful patient selection.

[Autologous kidney transplantation in a high-risk patient with locally advanced retroperitoneal neuroblastoma]. / Autologicheskaya transplantatsiya pochki u patsienta s mestnorasprostranennoi neiroblastomoi zabryushinnogo prostranstva gruppy vysokogo riska. Klinicheskoe nablyudenie.

Surgery of locally advanced neuroblastoma with risk factors is one of the most difficult in pediatric surgery. Incidence of nephrectomy during subtotal or complete tumor resection is higher due to common involvement of renal vessels. We present a patient with locally advanced retroperitoneal neuroblastoma who underwent heterotopic kidney autotransplantation.

[Analysis of future liver remnant parameters in children after extended liver resection]. / Analiz parametrov budushchego ostatka pecheni u detei, perenesshikh ee obshirnye rezektsii.

OBJECTIVE: To analyze the initial data on future liver remnant volume and its function evaluated by 99mTc-Bromesida hepatobiliary scintigraphy in children with liver tumors. MATERIAL AND METHODS: Extended liver resections were performed in 58 patients aged 2 months - 208 months (median 26 months) for various neoplasms. Before hepatectomy, all children underwent contrast-enhanced CT with volumetry and hepatobiliary scintigraphy with 99mTc-Bromezida and subsequent quantitative assessment of its accumulation in the future liver remnant. All consecutive patients eligible for extended liver resection were retrospectively analyzed. RESULTS: The analysis included patients who underwent extended liver resection between June 2017 and March 2021. Among 91 liver resections, 58 (64%) procedures were extended hepatectomies including 2 ALPPS procedures. Median volume of future liver remnant was 44.5% (16.5-91.4), median future liver remnant function - 10.14%/min/m2 (1.8-30). Four patients with adequate liver function had insufficient volume of future liver remnant. Insufficient future liver remnant volume and its appropriate function were observed in 2 patients. Not life-threatening post-resection liver failure developed in 2 patients. CONCLUSION: Evaluation of future liver remnant function is the most sensitive method to predict post-hepatectomy liver failure in children. The cut off value of future liver remnant volume in children is below 25% and probably below 16.5%. Further data collection and research are warranted to determine significant values. These data will contribute to define the new indications for two-staged hepatectomies in children.

[Liver transplantation for hepatoblastoma in children]. / Transplantatsiya pecheni pri gepatoblastome u detei.

OBJECTIVE: To improve the outcomes in children with hepatoblastoma. MATERIAL AND METHODS: There were 160 children with focal liver lesions who underwent surgery at the department of liver transplantation in 2008-2019. Patients with malignant tumors made up 77% (n=123). Hepatoblastoma (HB) prevailed (86%, n=106). Liver transplantation was performed in 19 (18%) patients with HB. Median follow-up after transplantation was 24.3 months by December 2019. Follow-up period did not exceed 4 years in more than 2/3 of patients. RESULTS: Overall and disease-free 10-year survival was 87.1% and 82.7%, respectively. Similar values were observed after resections (91.1% and 86.6%). At the same time, actuarial 4-year survival after liver transplantation for HB was 68%. CONCLUSION: Improvement of treatment outcomes may be achieved through multidisciplinary interaction ensuring timely drug therapy and liver transplantation.

[The analysis of the association of the polymorphic variants of the TPMT, COMT, and ABCC3 genes with the development of hearing disorders induced by the cisplatin treatment]. / Analiz assotsiatsii polimorfnykh variantov genov TPMT, COMT i ABCC3 c razvitiem narusheniia slukha, indutsirovannogo priemom tsisplatina.

Cisplatin and its derivatives are widely used chemotherapeutic agents for the treatment of many cancers, including hepatoblastoma, brain tumors, and germ-cell tumors. This therapy contributed to the dramatic increase in the survival rate. However, its use is restricted by the high incidence of irreversible ototoxicity associated with cisplatin application (in more than 60% of the children receiving it). Some studies have reported that genetic variants of TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) are conferring increased risk of developing cisplatin-induced hearing loss. However, in other studies the results were not replicated. In the present study, we replicated the previous studies based on an independent cohort of Russian patients. SNP genotypes for rs 12201199, rs4646316 and rs 1051640 were determined in DNA samples obtained from 16 patients who developed hearing loss and a group of 34 patients whose hearing was retained. The association between TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) variants and the hearing loss was not observed in our cohort.

[Neurosurgical aspects of the treatment of neuroblastoma patients]. / Neirokhirurgicheskie aspekty lecheniya bol'nykh s neiroblastomoi.

Neuroblastoma (NB) is the most common extracranial solid tumor in children. The neoplasm grows from progenitor cells of the sympathetic nervous system and can be detected anywhere along the sympathetic neurological circuit: retroperitoneally, mediastinally, cervically, and pelvically. Examination of children with suspected neuroblastoma is comprehensive and performed in strict compliance with a therapeutic protocol. A decision on the treatment regimen is made based on the tumor staging and the risk group of the patient. The diagnosis and treatment of NB patients are comprehensive and can be fully carried out only at the pediatric oncology department. In 10-15% of cases, an hourglass tumor spreads to the intervertebral foramina or spinal canal at one or more levels. A tumor node is always located extradurally with respect to the spinal cord. Symptoms of spinal cord compression of various severity are observed in 5-7% of patients. We present several cases of patients with neuroblastoma with intraspinal extension. Despite apparent benefits of primary surgical decompression of the spinal cord, modern experience of treatment of children with intraspinal tumor extension does not reveal advantages of surgery over chemotherapy. Neurological disorders of various nature and severity persist in the majority of patients in the long-term period, regardless of primary treatment. A higher level of spinal deformities after surgical tumor resection is observed. The issue of spinal cord decompression should be discussed by the neurosurgeon and pediatric oncologist, and the most common method of choice may be chemotherapy. The article discusses the indications and contraindications for neurosurgical interventions in NB patients and addresses the issues of NB metastasis to the brain and cranial bones as well as the opsoclonus-myoclonus syndrome.

[Classical osteosarcoma in children and adolescent].

Classical osteosarcoma is one of the most common primary malignant bone tumors in children and adolescents. It more frequently occurs in the areas of the highest growth plate proliferation: limb long bones particularly in the distal femur (30%), proximal tibia (15%), and proximal humerus (15%). In the long bones, the tumor is located usually in the metaphysis (90%), less frequently in the diaphysis (9%), and very rarely in the epiphysis. This paper considers the clinical, radiological, and histological diagnosis, and prognosis in this pathology.