[Differentiated surgical approach for retroperitoneal non-organ liposarcoma]. / Differentsirovannyi podkhod v khirurgii zabryushinnykh neorgannykh liposarkom.

OBJECTIVE: To determine the indications for combined and organ-sparing surgery depending on malignancy grade of retroperitoneal liposarcoma. MATERIAL AND METHODS: A retrospective study included 190 patients with retroperitoneal liposarcoma. Influence of malignancy grade, lesion of adjacent organs and resection/excision of adjacent organs on the overall and recurrence-free survival was studied. Moreover, we analyzed the issue of kidney-sparing surgery and nephrectomy. RESULTS: Overall and recurrence-free survival were significantly worse in high grade (G2-3) compared to low-grade tumors (G1) (p=0.0001; log-rank test). Visceral invasion was revealed in 23% of patients with low-grade (G1) tumors and 53% of patients with high-grade (G2-3) neoplasms. Visceral invasion significantly impairs overall and recurrence-free survival in both low grade (G1) and high-grade (G2-3) tumors (p=0.0001; log-rank test). In case of low grade (G1) retroperitoneal liposarcoma, overall and recurrence-free survival was similar after combined surgery without histologically confirmed visceral invasion of liposarcoma and organ-sparing surgery (p=0.006; p=0.053; log-rank test). On the contrary, high grade (G2-3) tumor was followed by significantly better overall and recurrence-free survival after combined surgery without histologically confirmed visceral invasion compared to organ-sparing surgery (p=0.006; p=0.053; log-rank test). Recurrence-free survival was similar after kidney-sparing surgery and nephrectomy among patients with low-grade (G1) tumor (p=0.456; log-rank test). In case of high-grade retroperitoneal liposarcoma (G2-3), recurrence-free survival was significantly worse after kidney-sparing surgery compared to nephrectomy (p=0.039; log-rank test). CONCLUSION: Surgery is the only potentially radical treatment of patients with retroperitoneal liposarcoma. Organ-sparing including kidney-sparing surgery is advisable for low-grade liposarcoma (G1). On the contrary, organ-sparing surgery impairs long-term results and prognosis in patients with high-grade tumors (G2-3). Combined operations including nephrectomy are justified for high-grade retroperitoneal liposarcoma (G2-3).

[Surgical and combined treatment of patients with duodenal stromal tumors]. / Osobennosti khirurgicheskogo i kombinirovannogo lecheniya bol'nykh stromal'noi opukhol'yu dvenadtsatiperstnoi kishki.

OBJECTIVE: To evaluate the immediate and long-term results of surgical and combined treatment of patients with duodenal stromal tumors. MATERIAL AND METHODS: There were 47 patients with duodenal stromal tumors for the period 2002-2019. All patients underwent treatment at the Blokhin National Cancer Research Center. Six patients had metastatic disease, 2 ones - a rare syndrome of duodenal stromal tumor associated with neurofibromatosis type 1, other 39 patients had a localized and locally-advanced disease. Surgical treatment was performed in 37 patients (limited resections (LR) in 24 cases and gastropancreaticoduodenectomy in 13 cases).Incidence of postoperative complications was significantly lower after limited resections compared to gastropancreaticoduodenectomy (22.2% (6/24) vs. 61.5% (8/13), respectively). Severe complications (Clavien-Dindo grade 3) occurred in 4.2% (1/24) vs. 15.3% (2/13) of patients, respectively. Postoperative mortality was absent in both groups. We observed no significant differences in long-term results. Overall 5-year survival was 91% and 70% (p=0.5960), 5-year recurrence-free survival - 65 and 70% (p=0.6226), respectively. CONCLUSION: Considering similar survival rates, lower postoperative morbidity and better quality of life, limited duodenal resections are preferred for duodenal stromal tumors.

[Gastrointestinal stromal duodenal tumors: diagnostic mistakes and modern approaches to treatment]. / Gastrointestinal'nye stromal'nye opukholi dvenadtsatiperstnoi kishki: oshibki diagnostiki i sovremennye podkhody k lecheniyu.

Gastrointestinal stromal duodenal tumors are rare diseases of small intestine. Duodenal GISTs may be giant; these neoplasms can also simulate malignancies of other organs. These features result diagnostic and treatment mistakes. Neoadjuvant therapy with imatinib results tumor shrinkage and ensures organ-sparing surgery. We report duodenal GISTs in patients with primary diagnosis «retroperitoneal tumor¼, «pancreatic cyst¼ and «retroperitoneal abscess¼, who were treated at the Blokhin National Cancer Research Centre in 2019-2020.

[Retroperitoneal dedifferentiated liposarcomas: semi-quantitative assessment of the dedifferentiated component and prognosis]. / Zabryushinnye dedifferentsirovannye liposarkomy: polukolichestvennaya otsenka dedifferentsirovannogo komponenta i prognoz.

OBJECTIVE: To evaluate the impact of malignancy grade and the proportion of the dedifferentiated component (DC) in retroperitoneal dedifferentiated liposarcomas (DDLS) on the course and prognosis of the disease. MATERIAL AND METHODS: The retrospective study enrolled 74 patients with primary retroperitoneal DDLS who underwent radical surgical treatment in the N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia. Histological surgery specimens from all cases of DDLS were reexamined and reclassified. According to malignancy grades and the proportion of the dedifferentiated component in the tumor, the patients were divided into the comparison groups included in the intergroup analysis of overall and relapse-free survival (OS and RFS) rates. The authors also analyzed the relationship between the proportion of the dedifferentiated component in DDLS and the frequency of adjacent organ invasion. RESULTS: Patients with a more than 15% dedifferentiated component had significantly lower OS rates than those with a less than 15% one (p=0.0001; log-rank test). The median OS in the DDLS group with a less than 15% dedifferentiated component was 91 months (95% CI, 82-100); that in the DDLS group with a more than 15% dedifferentiated component was 29 months (95% CI 17-41). The 5-year overall survival rates in the groups with less than 15% and more than 15% dedifferentiated components were 69% and 2%, respectively. The group with a more than 15% dedifferentiated component had significantly lower RFS rates than that with a less than 15% one (p=0.0001; log-rank test). In the DDLS groups with less than 15% and more than 15% dedifferentiated components, the median RFS rates were 25 months (95% CI 23-27) and 13 months (95% CI 8-18), respectively. In these groups, the 2-year RFS rates were equal to 50% and 9%, respectively. In the DDLS groups with less than 15% and more than 15% dedifferentiated components, pathologically confirmed invasion into the adjacent organs was observed in 32% and 63% of cases, respectively. There were no statistically significant differences in the OS and RFS of patients with DDLS according to tumor grade (p=0.069; p=0.102). CONCLUSION: This investigation suggests that DDLS have a more aggressive course with an increasing proportion of the dedifferentiated component in the tumor. Considering the histological variability in the dedifferentiated component, which is demonstrated in the research and scientific literature, as well as lack of a prognostic impact of histological grade, the authors believe that semi-quantitative assessment of the proportion of the dedifferentiated component in DDLS is able to serve as a simple and efficient morphological marker for the course of the disease and prognosis in retroperitoneal DDLS.

[Distal duodenectomy: a new option of surgical treatment for duodenal tumors]. / Distal'naya duodenal'naya rezektsiya: novyi sposob khirurgicheskogo lecheniya pri opukholevom porazhenii dvenadtsatiperstnoi kishki.

OBJECTIVE: To assess severity, safety and functional advisability of distal pancreatectomy using original surgical technique developed in the Blokhin National Medical Research Centre of Oncology. MATERIAL AND METHODS: There were 10 patients with duodenal malignancies who have undergone distal pancreatectomy in the Blokhin National Medical Research Centre of Oncology for the period 2006-2018. Distal pancreatectomy for primary duodenal tumors was performed in 8 patients, 2 patients underwent surgery for external invasion of the duodenum. RESULTS: Postoperative complications Clavien-Dindo grade 1 and 2 occurred in 4 (40%) patients. Surgical complication grade 2 occurred in 1 (10%) patient (pancreatic fistula with effective conservative management). There were no cases of leakage of duodenal stump and duodenojejunostomy, impaired bile flow and stenosis of anastomosis with delayed stomach emptying. CONCLUSION: Distal duodenectomyis associated with low postoperative morbidity, good functionality and quality of life. This procedure is preferred for non-epithelial and neuroendocrine tumors, as well as with secondary malignant duodenal invasion.

[Surgical treatment of relapsing metastatic stromal tumor of the gastrointestinal tract].

The study included 63 patients with relapsing metastatic stromal tumor of the gastrointestinal tract (surgery - 34, tyrosinkinase inhibitors - 29). Cytoreductive surgery is indicated in patients showing positive response to target therapy. The highest survival rates were reported in cases of surgical treatment received in conjunction with pre- and postoperative therapy with tyrosinkinase inhibitors.

[Gastrointestinal stromal tumours: clinico-morphological features, pathogenesis and modern treatment strategies].

Gastrointestinal stromal tumours (GIST) are the commonest mesenchymal tumours of gastrointestinal organs accounting for 1-3% of all gastrointestinal neoplasms and almost 5% of all soft tissue sarcomas. Most GIST (95%) express transmembrane receptor KIT or CD117, CD34 (70%), vimentin (80%), specific smooth muscle and neurogenic markers (with different frequency). Up to 85% of the stromal tumours have mutations in the KIT gene (exones 9, 11, 13, 17) and 3-18% in the PDGFRA (platelet-derived growth factor receptor-alpha) gene (exones 12, 14, 18). Mutations are absent in 5% of KITand PDGFRA genes. Mutational status, mitotic index, size and location of the tumour are the most informative criteria for the choice of treatment strategy and prognosis of the disease.