Background. Interest in combined treatment modalities for signet ring cell carcinoma and other subtypes of poorly cohesive gastric carcinoma has increased in recent years. Objective. To study and compare the effectiveness of two approaches to the treatment of poorly cohesive carcinoma: perioperative chemotherapy (CT) and surgery at the initial stage. Materials and methods. A total of 112 patients with poorly cohesive gastric carcinoma were included. Of these, 40 patients treated between 2018 and 2023 received perioperative chemotherapy with FLOT or mFOLFIRINOX. The historical control group comprised 72 patients treated between 2015 and 2018 who underwent initial surgery, followed by adjuvant XELOX chemotherapy in cases of pT ≥ 2 and pN+. In both groups, the diagnosis of poorly cohesive gastric carcinoma was confirmed by histological slide review of surgical specimens. Results. The five-year overall survival (OS) rate was 56.8% in the perioperative chemotherapy group and 50.8% in the surgical treatment group. Although survival rates favored perioperative treatment, the difference was not statistically significant (Log-Rank test; p = 0.073). The median OS in the surgery group was 67 months, while it was not reached in the perioperative chemotherapy group. Patients in the surgical treatment group presented with more advanced tumor stages, which may have contributed to the observed OS differences. Stage-specific analysis revealed no significant differences in OS between groups at any stage. Univariate Cox regression analysis indicated a trend toward treatment affecting OS (p = 0.081), but multivariate regression identified TNM stage as the only significant predictor of OS. Perioperative chemotherapy was not an independent prognostic factor. No differences in three-year OS were observed among patients with different tumor grades, regardless of perioperative chemotherapy regimen. In the FLOT and FOLFIRINOX groups, the three-year OS rates were 77.7% and 80.5%, respectively (Log-Rank test; p = 0.44). The median OS in the FOLFIRINOX group was 49.7 months, while it was not reached in the FLOT group. Conclusion. The lack of significant differences in survival prevents a definitive conclusion regarding the superiority of perioperative chemotherapy compared to initial surgery in patients with poorly cohesive gastric carcinoma. Further investigation in randomized controlled trials is warranted.

There is no clear consensus regarding the extent of surgical procedure for distal diffuse gastric cancer (GC). Most surgeons prefer the total gastrectomy (TG) to achieve greater radicality and reduce the likelihood of possible local recurrence of the disease. Perhaps, the distal gastrectomy is not inferior in terms of survival rates in distal diffuse gastric cancer.

В данном разделе указаны критерии оценки клинической значимости применения дорогостоящей противоопухолевой лекарственной терапии в соответствии со шкалой, разработанной экспертной группой (см. стр. 7). В тексте они обозначены, как магнитуда клинической значимости (МКЗ).

357 Background: Signet ring cell (SRC) gastric cancer (GC) has its unique clinicopathological features with poor tissue differentiation, high invasiveness, diffuse growth pattern and poor prognosis. Survival rates and clinicopathological characteristics of patients with SRC GC regardless of the stage of the disease has not been well studied. Methods: The retrospective analysis was undertaken of 993 patients with diagnosed gastric cancer. These patients received surgical resection in period from January 2013 to December 2018 in N.N. Blokhin National Medical Research Center of Oncology. We compared clinical and pathological features as well as prognostic factors between these groups. Results: For early SRC carcinoma, the median survival rates weren’t reached; 3- and 5-year survival rates were 100% and 89.0 %; for non-SRC carcinoma 91.4 and 85.3 % (HR=0.73; 95 % CI 0.22–2.42, р = 0.6) respectively. For locally advanced/metastatic SRC carcinoma, the median survival rates, 3- and 5-year survival rates were 38 months, 53.0 and 38.4 %; for non-SRC carcinoma 51.1 months, 59.2 and 48.0 % (HR=1.2; 95 % CI 0.91–1.54, р = 0.2). At univariate analysis SRC morphology was found as independent risk factor for overall survival (HR=1.19; CI 95% 0.94 – 1.50; p<0.0001). Multivariate analysis did not reveal SRC morphology to be significant and independent risk factor for overall survival (HR=1.00; CI 95% 0.72 – 1.38; p=0.98). Conclusions: Overall survival rates in patients with early (mucosal and submucosal tumor regardless of lymph nodes status) SRC gastric cancer did not show any significant differences in comparison with other histological types of GC. Thus, the prognosis of early SRC GC is equivalent or better than that of other histological subtypes of GC. Overall survival rates of locally advanced/metastatic SRC gastric cancer are worse than non-SRC cancer, but the differences are not statistically significant.

В данном разделе указаны критерии оценки клинической значимости применения дорогостоящей противоопухолевой лекарственной терапии в соответствии со шкалой, разработанной экспертной группой (см. стр. 7). В тексте они обозначены, как магнитуда клинической значимости (МКЗ).

Thyroid diseases in childhood occupy the second place after obesity in the structure of the general pathology of the endocrine system, eating disorders and metabolic disorders in children in the Russian Federation. Thyroid cancer in children makes up from 1.5 to 3 % of all malignant tumors, and from 8 to 22 % of malignant solid tumors of the head and neck, and the younger the child’s age, the more aggressive the disease proceeds.Familial forms of thyroid diseases may be associated with geographical features (living in iodine-deficient regions), but may also be part of hereditary syndromes, such as: multiple endocrine neoplasia syndromes (Sipple syndrome, Gorner syndrome, familial medullary thyroid cancer), DICER1 syndrome, Gardner syndrome, Cowden syndrome, McCune–Albright–Braitsev syndrome et al.This article describes several cases of thyroid pathology associated with DICER1 syndrome.

This article presents changes to clinical guidelines for the treatment of metastatic colon cancer in 2024. The new provisions in the clinical guidelines are complemented by a brief overview of the research results that underlie them. The changes considered concern not only systemic antitumor treatment, but also surgery and molecular genetic diagnostics. The differences between the recommendations of RUSSCO and the Ministry of Health of Russia are given. The introduction of information to determine the clinical benefit of expensive therapeutic options in relation to the use of the ESMO-MCBS and RUSSCO-MCBS scales is discussed.Aim. Bringing information to a wide range of readers on planned changes in clinical guidelines.

Введение: Лечение больных псевдомиксомой брюшины — одна из сложных и нерешенных проблем в онкологии. В отечественной медицинской литературе тема псевдомиксомы брюшины представлена единичными разрозненными публикациями. В основных зарубежных национальных клинических руководствах данная тема также не освещена. Основными документами, определяющими подходы к лечению псевдомиксомы брюшины, являются национальные консенсусы. Настоящий Консенсус является первой попыткой предложить стандартизованные подходы к проблеме псевдомиксомы брюшины в России.Методы: Настоящий Консенсус принят на основании анализа литературных данных, анализа известных зарубежных консенсусов, а также в результате серии обсуждений с участием специалистов, имеющих значительный личный опыт в диагностике и лечении псевдомиксомы брюшины.Результаты: Итоговый документ Консенсуса представлен в формате расширенных клинических рекомендаций, освещающих все аспекты данной патологии — эпидемиологию, патогенез, классификацию, клиническую картину, диагностику, и возможные варианты лечения, наблюдение после лечения и организационные вопросы.Заключение: Настоящий Консенсус создан с целью предложить практикующим врачам оптимальную стратегию лечения псевдомиксомы брюшины, а также информировать врачей (как онкологов, так и общего профиля) об особенностях данной редкой патологии.

Background. Signet ring cell gastric carcinoma of the stomach traditionally characterized by its poor prognosis. Survival of patients with signet ring cell gastric carcinoma regardless of the stage of the disease has not been well studied.Aim. To compare clinicopathological characteristics and survival of the patients with signet ring cell gastric carcinoma of the stomach and non-signet ring cell carcinoma depending on the stage of the disease.Materials and methods. We performed retrospective review was undertaken of patients with gastric cancer who received surgical treatment in period from 2013–2018.Results. At early signet ring cell cancer, the 3and 5-year survival rates was 100,0 and 89.0 %; for non-signet ring cell carcinoma 91.4 and 85.3 % (р = 0.6; Hazard Ratio (HR) 0.73; 95 % Confidence Interval (CI) 0.22–2.42) respectively. For advanced signet ring cell carcinoma, the median survival rates, 3and 5-year survival rates was 38 months, 53.0 and 38.4 %; for non-signet ring cell carcinoma 51.1 months, 59.2 and 48.0 % (р = 0.2; HR 1.2; 95 % CI 0.91–1.54).Conclusion. Long-term results in patients with early (T1) signet ring cell cancer of the stomach did not significantly differ in comparison with other histological types of gastric cancer. Long-term results of advanced signet ring cell cancer are worse than non-signet ring cell cancer, but the differences are not statistically significant.

Introduction: The main current approach to the treatment of patients with resectable cancer of the stomach and gastroesophageal junction (GEJ) is perioperative FLOT chemotherapy. The mFOLFIRINOX regimen has been shown to be effective and safe in disseminated adenocarcinoma of the stomach and GEJ. This article presents preliminary results of the efficacy and safety assessment of perioperative FOLFIRINOX chemotherapy in patients with resectable cancer of the stomach and gastroesophageal junction.Materials and Methods: The FOLFIRINOX / FLOT study is a phase 2 / 3 open-label, randomized trial. Study enrollment was started in January 2019 and is currently ongoing. The inclusion criteria are: histologically confirmed resectable adenocarcinoma of the stomach or gastroesophageal junction, Siewert types II–III, clinical stage cT4aN0M0, cT1–4N1–3M0 or cT2–4N0–3M0, with total or subtotal involvement of the stomach. The following regimens were used for perioperative chemotherapy: FLOT — docetaxel 50 mg / m2 on day 1, oxaliplatin 85 mg / m2 on day 1, leucovorin 200 mg / 2 on day 1, 5FU 2600 mg / m2 × 24 hours starting on day 1, or mFOLFIRINOX — irinotecan 180 mg / m2 on day 1, oxaliplatin 85 mg / 2 on day 1, leucovorin 200 mg / m2 on day 1, 5FU 250 mg / m2 bolus on day 1 and then 2200 mg / m2 × 48 hours on day 1. The primary endpoint was 5‑year overall survival.Results: All planned preoperative courses of chemotherapy had been administered to 25 (86 %) patients in the FLOT group (n = 29) and 22 (92 %) patients in the FOLFIRINOX group (n = 24). Four (12 %) and 2 (8 %) patients in the FLOT and FOLFIRINOX groups, respectively, discontinued the treatment. The surgical staging was used in 48 patients (91 %) (25 [86 %] in the FLOT group and 23 [96 %] in the FOLFIRINOX group). Complete tumor regression (Mandard grade 1) had been achieved in 4 patients (2 [7 %] in the FLOT group and 2 [8 %] in the FOLFIRINOX group). Postoperative complications were detected in 2 patients (8 %) in the FLOT group and 4 (17 %) in the FOLFRIRNOX group. Thirty-three patients (62 %) received all scheduled postoperative treatment courses (n = 19, 66 % for FLOT and n = 14, 58 % for FOLFIRINOX).Conclusions: The preliminary results of the FOLFIRINOX / FLOT study showed comparable tolerability of the regimens and comparable complete pathological response rates. However, there was a higher incidence of postoperative complications detected among patients who received the FOLFIRINOX regimen compared to the FLOT group.

Introduction. Hereditary disorders in the DNA repair system can lead to the development of malignant neoplasms in childhood. DNA constitutional mismatch repair deficiency syndrome (CMMRD) is a very rare genetic autosomal recessive disorder caused by homozygous mutations in one of the four mismatch repair genes (MLH1, MSH2, MSH6, and PMS2). The frequency of occurrence is 0.0000001 of the adult and child population. For now about 150 observations have been published in the world literature. The prognosis for CMMRD syndrome is extremely unfavorable. The spectrum of tumors that make up the CMMRD syndrome is very wide, and includes mainly malignant brain tumors, tumors of the digestive tract, hematological malignancies, embryonic tumors, all of which develop in childhood.The purpose of the study is to report a case of CMMRD-associated embryonic rhabdomyosarcoma in a 3-year-old child.Conclusions. A review of the literature and the clinical case we have described show that rhabdomyosarcoma belongs to the tumor spectrum of the CMMRD syndrome. An immunohistochemical study revealed an isolated loss of PMS2 gene expression. Taking into account the clinical course of the CMMRD syndrome, a thorough study of the family history in patients with rhabdomyosarcoma is recommended, as well as a molecular genetic study, including the search for germinal mutations in genes in the DNA repair system and the assessment of microsatellite instability in the material of the tumor tissue. The clinical symptoms of CMMRD syndrome are nonspecific and depend on the morphological variant of the primary tumor. Distinctive molecular genetic features of this syndrome are: homozygous mutations with loss of function of the germline genes of the MMR system (mismatch repair) (MLH1, MSH2, MSH6 or PMS2).

Primary-multiple malignant neoplasms develop in about 9 % of cancer patients in the Russian Federation, while synchronous tumors are detected in a quarter of them, which dictates the need for a mandatory multidisciplinary approach to the choice of treatment. Localization of synchronous primary-multiple tumors in one anatomical zone requires a qualitative interpretation of instrumental diagnostic methods and morphological analysis after biopsy of each tumor. Along with this, there is a possibility of an erroneous opinion about the presence of different histological structures of the detected tumors and the data of visual assessment methods are interpreted in favor of a single disease. This paper presents a clinical observation of a rare combination of gastric adenocarcinoma and peritoneal mesothelioma. The case was discussed at a multidisciplinary consultation with the participation of a surgeon, a chemotherapist and a radiologist. Taking into account the clinical stage of gastric body cancer c T3N1M0, III stage, it was decided to conduct preoperative polychemotherapy at the first stage. Repeated diagnostic laparoscopy revealed no negative dynamics, multiple small dropouts in the peritoneum persisted. Subsequently, the operation was performed in the volume of gastrectomy, total parietal peritectomy and appendectomy without macroscopic signs of a residual tumor. It was decided to refrain from intra-abdominal chemoperfusion with hyperthermia due to the large volume of surgery and the previous 8 courses of polychemotherapy. Microscopic examination of the altered part of the stomach wall revealed residual adenocarcinoma (type according to Lauren – intestinal) with signs of therapeutic pathomorphosis G3 (according to Mandard), which had grown into the subserous layer and the fiber of the small omentum, with signs of perineural invasion, in the absence of vascular invasion; resection edges – R0. On the surface of the removed fragments of the peritoneum, various foci of malignant epithelioid mesothelioma were found. At the control examination a year later, according to computed tomography and esophagogastroduodenoscopy, there were no signs of progression, the patient’s condition was satisfactory.

This study was aimed at determining the indications for combined and organ-preserving operations. The study included 190 patients with retroperitoneal liposarcoma (RLPS). The influence of the following factors on the overall survival (OS) and recurrence-free survival (RFS) were studied: involvement of adjacent organs in the tumor, volume of surgical intervention. OS and RFS were worse in pathologically confirmed visceral invasion in the both RLPS low grade and high grade ( p = 0.000). In RLPS low grade, there was no significant difference in OS and RFS between the group of patients who underwent combined surgery without confirmed visceral invasion and the group of patients who underwent organ–preserving surgery ( p > 0.080). In RLPS high grade, OS and RFS were higher in the group of patients who underwent combined surgery without confirmed visceral invasion than in the group of patients who underwent organ–preserving surgery ( p < 0.050). In RLPS low grade, it is advisable to perform organ-preserving operations, including nephrosaving operations. In RLPS high grade, the organ-preserving operations worsen long-term results and prognosis. Combined operations including nephrectomy are justified in RLPS high grade.

Background. Uterine leiomyosarcomas are highly aggressive tumors with a poor prognosis. The main prognostic factors are the stage of the disease, the size of the primary tumor, and the grade of malignancy. It is well known that the clinical course and prognosis of uterine leiomyosarcoma do not correlate with the FNCLCC histological grade, based on assessment of the mitotic index and the presence and amount of necrosis in sarcomas. There is published data on a more unfavorable course of non-spindle cell uterine leiomyosarcomas. Aim. To evaluate the influence of pathological factors (histological subtype, mitotic index, necrosis and its type) on survival rates in uterine leiomyosarcoma. Materials and methods. The study included 58 patients who underwent radical surgery for uterine leiomyosarcoma. The following morphological parameters were evaluated on histological slides: histological subtype, mitotic index, the presence of necrosis in the tumor and its type. Further, the analysis of the influence of these morphological characteristics on the indicators of progression-free survival and overall survival was carried out. Statistical analysis was carried out using the IBM SPSS Statistics Professional 20.0 statistical software package. Long-term results are presented in the form of actuarial survival calculated by the Kaplan–Meier method. Results. Most cases of uterine leiomyosarcoma were of the spindle-cell variant (82.8 %), had a mitotic index >20 per 10 high power field (93.1 %) and large foci of necrosis (70.7 %), coagulation necrosis was more often detected (43.1 %). There was a significant difference in the median overall survival of patients with spindle-cell variant of uterine leiomyosarcoma and non-spindle-cell variants (epithelioid or myxoid): 77 months vs. 49 months (p = 0.05). There was no significant relationship between the mitotic index of the primary tumor, the size of necrosis foci in the tumor and survival rates. The presence of coagulative necrosis in the tumor was significantly associated with a deterioration in progression-free survival (9 months vs. 14 months, p = 0.05) and overall survival (51 months vs. 104 months, p = 0.05). Conclusion. The spindle-cell variant of leiomyosarcoma is associated with higher overall survival rates compared to other histological subtypes. The presence of coagulative necrosis in the tumor in comparison with other types of necrosis is an unfavorable prognostic factor associated with a decrease in both progression-free survival and overall survival.

Salivary gland carcinomas is a rare family of neoplasms with different histological characteristics and biological behavior. Treatment regimens have remained virtually unchanged for decades, leaving the leading role for surgical treatment and radiation therapy. However, a special place in the salivary gland carcinomas group is occupied by adenoid cystic carcinoma, which is quite often characterized by a recurrent and / or metastatic course with the use of chemotherapy as the main option in treatment. It is worth noting the fact that the results of standard chemotherapy regimens show unsatisfactory results, respectively, with recurrent or metastatic adenoid cystic carcinoma, treatment options are minimal. Accordingly, there is a need to study new therapeutic methods for the treatment of this disease. In this situation, high hopes were placed on targeted therapy, in particular, the family of epidermal growth factor receptors, c-Kit are the most frequently studied molecular targets. Due to the rare occurrence of adenoid cystic carcinoma, the number of cases of using targeted therapy for analysis is relatively small, so each clinical observation is of particular value. The following is a clinical case of treatment of recurrent adenoid cystic carcinoma using a personalized treatment approach.Aim. To describe the clinical observation of the use of targeted drugs in recurrent or metastatic adenocystic cancer of the salivary glands.

Ewing’s sarcoma (ES) of the kidney is an extremely rare malignant tumor characterized by an aggressive course, and therefore the disease has an unfavorable prognosis. Due to the rare occurrence, standards for the treatment of ES of the kidney have not been developed; an integrated approach is often used in therapy. In this article, we present a description of two clinical cases of kidney damage with ES in patients aged 10 and 16 who were treated at the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia. In the first case, there was a metastatic lesion of the kidney with the localization of the primary focus in the pelvic bones, in the second, primary ES of the kidney was diagnosed. Both patients had stage IVb at the time of diagnosis (TNM classification American Joint Commission on Cancer). In both patients, after induction chemotherapy, a complete therapeutic response (complete therapeutic pathomorphosis) was obtained. In an integrated approach, in addition to standard chemotherapy, surgery and radiation therapy, in both cases, high-dose chemotherapy with autologous peripheral stem cell transplantation was used, which made it possible to create favorable conditions for long-term remission in one of the patients, even despite the initial prevalence of the process.

Introduction. Primary spinal osteosarcoma and rare metastatic heart lesion are considered significant factors for poor prognosis. The available work indicates that combined treatment, including the radical surgery of heart solitary metastatic lesion, probably provides the best outcome.Case report. Man, 27-year-old, with Th8 spinal osteosarcoma and solitary left atrial metastasis. From October 2019 to February 2020 5 cycles of chemotherapy (high doses of platinum and doxorubicin) were carried out with positive effect. Next steps were surgery: March 2020 – spondylectomy of the Th8 vertebra with combined stabilization; September 2020 – subtotal resection with plasty of the right atrium, tricuspid valve and prosthesis of the right coronary artery. Between the first and second surgery, 4 more same chemotherapy cycles were carried out. Currently, 24 months after completion of treatment, there are no signs of the disease.Conclusion. The article presents a unique case of treatment of primary osteosarcoma and metastatic lesion of rare location. Multidisciplinary approach and combination treatment in specialized centers increase the probability of favorable outcome in such cases.

Intriduction. Hibernomas account for less than 2 % of all benign lipomatous tumors and 1 % of all lipomatous tumors. They are usually found in 20–40-year old adults (the average age of patients is 38 years). The clinical manifestations are painless soft tissue mass that are mostly slow growing; rapid growth is only found in a small number of patients. We present a case of successful surgical treatment of a pathologically confirmed rare lipomatous tumor of large size, which was located in the soft tissues of the thigh.Clinical case. A 46-year-old woman was admitted to the hospital due to a tumor 20 × 10 × 10 cm in size, that had developed 10 years ago in the anteromedial aspect of the proximal thigh. Furthermore, she had a history of hypoesthesia one month prior to her admission. During surgery, the femoral nerve was found to be compressed by the large tumor. The resultant symptoms probably caused the patient to seek medical care. Resection was performed by careful dissection, femoral nerve and vessels were spared. Histopathological examination and cytogenetics showed findings suggestive of a hibernoma. At the 6-month follow-up local tumor recurrence or metastasis was not found.Conclusions. Asymptomatic small-sized hibernoma does not require surgical resection or other treatment. When the tumor is too large and/or has the potential to compress the surrounding tissues, timely radical surgical intervention is reuired.

Transmission of a malignancy from a donor’s organ to the recipient of the graft is a rare event, though it is a severe complication that can result in a poor outcome. Usually, immunosuppressive therapy is discontinued and the allograft is removed. However, treatment of patients with the disseminated cancers implies that after the graft removal and cessation of the immunosuppression, radiotherapy, chemotherapy, or immunotherapy with alpha-interferon (INF-α) or interleukin-2 (IL-2) are required. The case report presents a clinical case of a transmitted kidney graft with multiple metastases (MTS) in a 31-year-old woman with the spontaneous regression of the metastatic cancer after transplantectomy and cancellation of the immunosuppressive therapy. Obviously, the determining factor is the recognition of the tumor by the effectors of the antitumor immunity due to the human leukocyte antigen (HLA) mismatch between the donor and the recipient. Therefore, cancellation of the immunosuppressive therapy in cases of transferal of a malignancy with a transplanted organ allows the effectors of the immune system to distinguish the tumor as a foreign tissue and effectively eliminate this neoplasm.

This study was aimed at creating an effective model for predicting the course of the disease in retroperitoneal well-differentiated (WDLPS) and dedifferentiated (DDLPS) liposarcomas after surgery. The study included 111 patients with WDLPS and 74 patients with DDLPS. We developed a methodology for stratification of patients into prognostic groups. Overall survival (OS) and recurrence-free survival (RFS) were analyzed in accordance with it. The highest OS was achieved in the group "favorable prognosis," while the shortest OS was in the group "extremely poor prognosis" (p < 0.001). The median OS in the "favorable prognosis" group was 225 (95% CI, 174, 276) months; "intermediate prognosis" - 130 (95% CI, 115, 145) months; "poor prognosis" - 90 (95% CI, 79, 101) months; and "extremely poor prognosis" - 22 (95% CI, 15, 29) months. The highest RFS was achieved in the group "favorable prognosis," while the shortest RFS was achieved in the group "extremely poor prognosis" (p < 0.001). The median RFS in the "favorable prognosis" group was 80 (95% CI, 65, 95) months; "intermediate prognosis" - 47 (95% CI, 33, 61) months; "poor prognosis" - 26 (95% CI, 24, 28) months; "extremely poor prognosis" - 10 (95% CI, 6, 14) months. The method of predicting recurrence-free and overall survival demonstrates an adequate distribution of patients and the reliability of intergroup differences in the survival rate.