L-asparaginase is one of the most effective drugs in pediatric and adult acute lymphoblastic leukemia treatment. But drug side effects are an important problem. pancreatitis and pancreatic necrosis are not common (2–18 %) complication, but high chance of severe disease with fatal outcome make to bring in careful attention of pediatric oncologists-hematologists, surgeons, intensivists, radiologists. Recognizing multidisciplinary importance of this problem, at June 21st, 2023 at Morozov Children’s Clinical hospital a Round table on pancreatitis/pancreatic necrosis after L-asparaginase use was organized. This article presents expert recommendations from federal and regional clinics in diagnosis and treatment of such severe complication.

The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer with a very poor prognosis. Surgery remains the best treatment modality for adrenocortical carcinoma in the early stages. The appropriate treatment for metastatic ACC is not well established, and the effectiveness of chemotherapy and radiotherapy, is not proven. Five-year survival for patients with stage IV tumors ranged from 0 to 17 %. The data regarding the effectiveness of surgery in the management of metastatic tumor remain scarce.Aim of the study – to present our results of surgical treatment for metastatic ACC in children and to determine the risk factors of local relapse.Materials and methods. The results of treatment of 10 patients with a median age of 5.8 (1.5–15.1) years with stage IV ACC were retrospectively analyzed. The tumors were hormone-producing in 6 of 10 cases. Four (40 %) patients experienced a Cushing syndrome. Primary tumor resection R1 and R0 was performed in 8 (80 %) and 5 (50 %) patients respectively. The median volume of resected tumor was 183 (3.6–1608) cm3. All patients received mitotane combined in 80 % with systemic chemotherapy consisted of etoposide, doxorubicin, cisplatin (EDP-M).Results. Two (20 %) of 10 patients are alive with tumor in 14.1 and 28.8 months respectively. The median follow-up in the hole group was 21.8 (10.4–33.9) months and the median period to disease progression was 11.3 (6.4–17.9) months. The risk of local relapse in the first 8 months after surgery was 33.3 % vs. 100 % in R0 and R1/R2 groups respectively. The period from diagnosis to death from tumor progression was significantly longer in 7 patients with 2 and more EDP-M courses compared to 3 patients who received one EDP-M or no further treatment (24.9 vs. 14.4 months, p = 0.02). Two and five-year overall survive were 57.1 % and 0 % respectively.Conclusion. The radical surgical resection (R0) significantly reduced the risk and incidence of local relapse in children with stage IV ACC. Adjuvant EDP-M therapy did not affect the unfavorable prognosis of the disease, but positively influenced life expectancy of patients with disseminated ACC.

Objective: to compare short-term and long-term postoperative complications between patients who have undergone central pancreatectomy (CP) and distal pancreatectomy (DP).Materials and methods. This retrospective study included patients who had CP for benign pancreatic tumors and tumors of low malignant potential (cases) and patients who had DP for similar reasons (controls). The controls were randomly selected and matched cases for tumor size, presence of diabetes mellitus (Dm), and ASA physical status. we evaluated the incidence of grade ≥III complications (Clavien–Dindo classification), clinically significant pancreatic fistulas, Dm, and impaired exocrine pancreatic function in the late postoperative period.Results. There were 25 cases and 25 controls. Both groups were matched for the main clinical characteristics. Surgeries were significantly longer in the CP groups compared to the DP group (230 min vs 180 min, р < 0.0001). There was no difference in the overall incidence of postoperative complications (9 (36 %) vs 14 (56 %), р = 0.26); there was a trend towards a higher incidence of postoperative complications in the CP group. Two patients after CP (8 %) required repeated surgeries. none of the study participants died. Clinically significant (B and C) pancreatic fistulas were registered in 8 (32 %) and 11 (44 %) patients, respectively (p = 0.56). Two patients in the DP group (8 %) developed impairments of exocrine pancreatic function that required pharmacotherapy. none of the patients developed Dm postoperatively.Conclusion. Despite the fact that CP and DP outcomes were comparable in terms of the main parameters evaluated, severe post-CP complications indicate that there is a need for careful selection of patients for such interventions and further accumulation of experience. Our findings can be used in the subsequent analysis of the experience of different clinics.

Introduction. Enhanced recovery after surgery and the early initiation of chemotherapy is a significant advantage of laparoscopic surgeries for malignant tumors of abdominal cavity in children. Despite the extensive experience in using caudal block in pediatric patients, it has not yet been determined whether the use of ultrasound navigation provides any clinical advantage in post-operative recovery after laparoscopic surgeries in pediatric oncology. Materials and methods. The study included 40 patients of Pediatric Oncology and Hematology, Federal Blokhin National Medical Research Center, ASA II–III, who underwent laparoscopic surgeries for malignant tumors of the abdominal cavity during 2017–2019. Patients were divided randomly into 2 groups. The CB (caudal blockade) group included 23 children. In CB group caudal epidural block as a regional component of combined anesthesia was used (CB group n = 23). GA (general anesthesia) group included 17 children who underwent general anesthesia (GA group, n = 17). Results. The median of the total dose of fentanyl in the CB group was 7.29 (6; 9.25) μg/kg, in the GA group — 10.7 (7.6; 12.5) μg/kg (р ≤ 0.012). In the postoperative period, patients in CB group didn’t require additional analgesia for 24 hours after caudal-epidural administration of morphine. After caudal-epidural administration of trimeperidin, 12 hours later. Enteral nutrition in children in the CB group was started in 4.7 ± 0.5 hours. In the GA group, enteral nutrition in 10 (59 %) patients was started in 20 hours after the end of the surgery. 5 children developed Conclusions. This study demonstrates that caudal epidural block for laparoscopic surgeries performed under ultrasound navigation provides effective analgesia in both perioperative and postoperative periods, promotes early start of enteral nutrition, minimizing the frequency of postoperative nausea and vomiting. Ultrasound navigation increases the likelihood of success and safety of the caudal epidural block.

Т. С. Мирзаев, Д. В. Подлужный, Р. Е. Израилов, А. Г. Котельников, Е. В. Глухов, И. М. Файнштейн, П. А. Керимов, Н. Е. Кудашкин, А. Н. Поляков, Б. И. Сакибов ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н. Н. Блохина» Минздрава России; Россия, 115478 Москва, Каширское шоссе, 23; ГБУЗ «Московский клинический научный центр им. А. С. Логинова Департамента здравоохранения г. Москвы»; Россия, 111123 Москва, шоссе Энтузиастов, 86; Клинический госпиталь Лапино «Мать и дитя», Онкоцентр «Лапино»; Россия, 143081 Лапино, 1-е Успенское шоссе, 111 / 1

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland. Despite the low incidence of ACC the mortality rate ranges from 0.04 to 0.2 %, in the overall structure of cancer mortality. Treatment of ACC is mainly surgical and radical surgical excision is the treatment of choice for local disease stages.Aim of the study — to present our results of surgical treatment of localized and locally advanced ACC in children and to determine the risk factors of relapse.Materials and methods. Twenty-eight patients (median age of 47.8 (06—216) mo.) with localized and locally advanced ACC underwent a retrospectively analysis. Stage I, II, and III revealed in 12 (45 %), 7 (25 %), and 9 (30 %), respectively. In 19 (68 %) cases the secretion of one or more hormone observed. Macroscopically and microscopically complete resection were performed in 26 (93 %) and 23 (82 %) patients, respectively. The median tumor volume was 183 (3.6—1608) cm3 and the median tumor weight was 207.9 (48—710) g.Results. Five-year overall (OS) and relapse-free (RFS) survival were 71 % and 69 %, respectively. OS and RFS according to stage I, II, and III were 100 % vs. 71 % vs. 17 % and 100 % vs. 71 % vs. 14 % respectively. The radical surgical resection and the level of Ki-67 expression influenced significantly the rates of OS and RFS (p < 0.001).Conclusion. The main factor affecting the survival rate of ACC in children with stages I—III is the radical surgical resection. It should be taken into account when planning postoperative therapy. Some of biological characteristics of the tumor could also significantly affect the results of treatment.

Relevance. The success of treatment of children with hepatoblastoma (HB) is associated primarily with the optimization of diagnostic and therapeutic strategies. The introduction of a system of therapeutic definition of a risk group developed by the International Society for the Study of Liver Tumors in Children’s Hepatic tumors International Collaboration (CHIC) in patients with HB allows for effective risk stratification. Patients of the very low-risk group have a favorable prognosis and, unlike patients of other groups, they can perform surgery at the 1st stage, and children from the very low-risk group with a “pure” fetal version of the morphological structure of the tumor can be cured without chemotherapy – only by surgical method. This article presents an analysis of a series of observations of children with HB very low-risk.Methods and patients. A retrospective analysis of the data of 138 patients who received treatment at the Research Institute of Pediatric Oncology and Hematology of N.N. Blokhin National Medical Research Centre of Oncology of the Ministry of Health of Russia from January 2006 to February 2018, all patients underwent examination in the following volume: computed tomography (CT) of the chest, ultrasound of the abdominal cavity and retroperitoneal space, CT and/or magnetic resonance imaging of the abdominal cavity with contrast enhancement. Serum levels of human chorionic gonadotropin and alpha-fetoprotein (AFP) were measured. The prevalence of tumor lesions was retrospectively evaluated using the PRETEXT system (Pre-Treatment Extent of Disease, assessment of the prevalence of the tumor process before treatment, 2017 version). Based on the data obtained, 18 (13 %) patients with hypertension were identified in the very low-risk group using the CHIC stratification system. Of 18 (100 %) patients of the very low-risk group, 8 (44.4 %) received therapy in the interval from January 2006 to October 2010 according to the treatment protocols of the SIOPEL group. The treatment included a combination of neoadjuvant chemotherapy with delayed surgery and adjuvant chemotherapy. Since November 2010, the Research Institute of Pediatric Oncology and Hematology adopted a protocol for the treatment of children with hypertension, suggesting the possibility of performing surgical treatment at the 1 st stage in patients with a localized stage of the disease with the spread of PRETEXT I/II. From December 2010 to November 2018, 10 (55.6 %) patients from the very low-risk group were operated on at the 1 st stage. A tumor biopsy was not performed before the intervention. After a histological examination, 4 (22.2 %) patients had an epithelial variant of the structure of HB that did not meet the criteria of a “pure” fetal variant. He was given adjuvant chemotherapy according to the SIOPEL protocol. And in 6 (33.3 %) patients, according to the results of a morphological study, the HB structure variant was interpreted as a “pure” fetal variant. Further therapy in this group of patients was not performed. The analysis of the results of treatment of these 6 patients without the use of chemotherapy is presented in our article.Results. The average age of patients is 3.7 (1–6) years, the median is 3.7 ± 2.0. Boys/girls – 4:2. In all cases, there was an asymptomatic course of the disease, a neoplasm was detected by chance during an ultrasound scan. All children had an elevated AFP level in the range of 95.2–695 (286.0 ± 116.8) IU/ml. Distribution according to PRETEXT I/II – 2 (33.3 %) and 4 (66.7 %) patients, respectively. Patients did not have additional PRETEXT criteria and metastases. Surgical treatment was performed by the laparoscopic method in 2 of 6 patients. Scope of operation: right-sided hemihepatectomy – 3, left-sided – 1, bisegmentectomy – 1, atypical resection – 1. Lymphatic dissection in the area of the hepatoduodenal ligament was not performed. There were no complications of treatment and repeated surgical interventions. The radicalness of the intervention in the volume of R0 was confirmed by histological examination in all patients. Morphological examination in all cases revealed a “pure” fetal version of the structure of the tumor. All patients are alive without signs of relapse at follow-up periods of 14.7 to 59.2 (32.0 ± 18.7) months.Conclusion. Using the system of therapeutic stratification into risk groups CHIC allows you to effectively distinguish patients with very low-risk groups, and for some children with a “pure” fetal version of the histological structure of the tumor, only the surgical approach can be used for treatment. At the same time, the short observation period for the patients and their small number do not allow us to draw final conclusions and dictate the need for a multicenter study. It should be noted that this cohort of patients requires careful observance of the post-therapeutic observation algorithm.

Birkitt lymphoma (BL) is one of the most studied and curable type of lymphoma in children. But inspite of lymphomagenesis deep understanding on immunologic, molecular and genetic levels, diagnosis of some rare clinical presentations of disease become challenging task for well-qualified clinicians. In the current issue we present rare case of skin involvement in pediatric 4 year old patient with disseminated BL. It is presented clinical and morphologic characteristic of specific skin presentation. Used multiagent chemotherapy with rituximab deliver the result of complete response. Skin site regression was found on 2d day of treatment. Parents give their agreement to use personal data, including fotos, in research and publications.

Relevance. The success of treatment of children with hepatoblastoma (HB) is associated primarily with the optimization of diagnostic and therapeutic strategies. The introduction of a system of therapeutic definition of a risk group developed by the International Society for the Study of Liver Tumors in Children’s Hepatic tumors International Collaboration (CHIC) in patients with HB allows for effective risk stratification. Patients of the very low-risk group have a favorable prognosis and, unlike patients of other groups, they can perform surgery at the 1st stage, and children from the very low-risk group with a “pure” fetal version of the morphological structure of the tumor can be cured without chemotherapy – only by surgical method. This article presents an analysis of a series of observations of children with HB very low-risk. Methods and patients. A retrospective analysis of the data of 138 patients who received treatment at the Research Institute of Pediatric Oncology and Hematology of N.N. Blokhin National Medical Research Centre of Oncology of the Ministry of Health of Russia from January 2006 to February 2018, all patients underwent examination in the following volume: computed tomography (CT) of the chest, ultrasound of the abdominal cavity and retroperitoneal space, CT and/or magnetic resonance imaging of the abdominal cavity with contrast enhancement. Serum levels of human chorionic gonadotropin and alpha-fetoprotein (AFP) were measured. The prevalence of tumor lesions was retrospectively evaluated using the PRETEXT system (Pre-Treatment Extent of Disease, assessment of the prevalence of the tumor process before treatment, 2017 version). Based on the data obtained, 18 (13 %) patients with hypertension were identified in the very low-risk group using the CHIC stratification system. Of 18 (100 %) patients of the very low-risk group, 8 (44.4 %) received therapy in the interval from January 2006 to October 2010 according to the treatment protocols of the SIOPEL group. The treatment included a combination of neoadjuvant chemotherapy with delayed surgery and adjuvant chemotherapy. Since November 2010, the Research Institute of Pediatric Oncology and Hematology adopted a protocol for the treatment of children with hypertension, suggesting the possibility of performing surgical treatment at the 1 st stage in patients with a localized stage of the disease with the spread of PRETEXT I/II. From December 2010 to November 2018, 10 (55.6 %) patients from the very low-risk group were operated on at the 1 st stage. A tumor biopsy was not performed before the intervention. After a histological examination, 4 (22.2 %) patients had an epithelial variant of the structure of HB that did not meet the criteria of a “pure” fetal variant. He was given adjuvant chemotherapy according to the SIOPEL protocol. And in 6 (33.3 %) patients, according to the results of a morphological study, the HB structure variant was interpreted as a “pure” fetal variant. Further therapy in this group of patients was not performed. The analysis of the results of treatment of these 6 patients without the use of chemotherapy is presented in our article. Results. The average age of patients is 3.7 (1–6) years, the median is 3.7 ± 2.0. Boys/girls – 4:2. In all cases, there was an asymptomatic course of the disease, a neoplasm was detected by chance during an ultrasound scan. All children had an elevated AFP level in the range of 95.2–695 (286.0 ± 116.8) IU/ml. Distribution according to PRETEXT I/II – 2 (33.3 %) and 4 (66.7 %) patients, respectively. Patients did not have additional PRETEXT criteria and metastases. Surgical treatment was performed by the laparoscopic method in 2 of 6 patients. Scope of operation: right-sided hemihepatectomy – 3, left-sided – 1, bisegmentectomy – 1, atypical resection – 1. Lymphatic dissection in the area of the hepatoduodenal ligament was not performed. There were no complications of treatment and repeated surgical interventions. The radicalness of the intervention in the volume of R0 was confirmed by histological examination in all patients. Morphological examination in all cases revealed a “pure” fetal version of the structure of the tumor. All patients are alive without signs of relapse at follow-up periods of 14.7 to 59.2 (32.0 ± 18.7) months. Conclusion. Using the system of therapeutic stratification into risk groups CHIC allows you to effectively distinguish patients with very low-risk groups, and for some children with a “pure” fetal version of the histological structure of the tumor, only the surgical approach can be used for treatment. At the same time, the short observation period for the patients and their small number do not allow us to draw final conclusions and dictate the need for a multicenter study. It should be noted that this cohort of patients requires careful observance of the post-therapeutic observation algorithm.

Introduction. Solid-pseudopapillary tumors (SPT) of the pancreas are rare, not exceeding 1-2% of all exocrine pancreatic tumors. Treatment of patients with this pathology consists of the radical removal of the tumor which has to a favorable prognosis of the disease. If the tumor is localized only in the pancreas tail or in the body with invasion into the tail, distal subtotal splenopreserving or distal splenopreserving pancreatic resections are performed. Objective. To demonstrate potentials of laparoscopic access in the surgical treatment of children with SPT. Material and methods. The article discusses 9 patients with diagnosed SPT who had the abovementioned surgical interventions via laparoscopic access . Discussion. The researchers demonstrated that pancreatic resections can be performed laparoscopically. Conclusions. The laparoscopic access is a priority for distal, distal subtotal pancreatic resections. It reduces surgical time, minimizes surgical trauma, accelerates patients’ rehabilitation , improves cosmetic effects as well as makes hospital stay shorter. In addition, it does not worsen immediate and long-term outcomes of the treatment.

Congenital mesoblastic nephroma (CMN) is a rare primary kidney tumor in young children, characterized by intermediate biological behavior. The main treatment method is the radical removal of the tumor. The purpose of this study is to analyze our own experience in the treatment of patients with a diagnosis of CMN. Parents of patients agreed to the use of information about them, including photographs, in scientific research and publications.

Infantile fibrosarcoma (IF) is a rare soft tissue sarcoma that occurs with a frequency of 1% of all cases of malignant neoplasms in children in the first year of life. IF most often affects the soft tissues of the upper and lower extremities. Rare localizations include the trunk, the head and neck, and the gastrointestinal tract. The article describes a rare clinical case of congenital IF of soft tissues of the chest wall in a newborn, as well as a literature review.

Birkitt lymphoma (BL) is one of the most studied and curable type of lymphoma in children. But inspite of lymphomagenesis deep understanding on immunologic, molecular and genetic levels, diagnosis of some rare clinical presentations of disease become challenging task for well-qualified clinicians. In the current issue we present rare case of skin involvement in pediatric 4 year old patient with disseminated BL. It is presented clinical and morphologic characteristic of specific skin presentation. Used multiagent chemotherapy with rituximab deliver the result of complete response. Skin site regression was found on 2d day of treatment. Parents give their agreement to use personal data, including fotos, in research and publications.

Congenital mesoblastic nephroma (CMN) is a rare primary kidney tumor in young children, characterized by intermediate biological behavior. The main treatment method is the radical removal of the tumor. The purpose of this study is to analyze our own experience in the treatment of patients with a diagnosis of CMN. Parents of patients agreed to the use of information about them, including photographs, in scientific research and publications.

About 20—25 % of all rhabdomyosarcomas in children are located in the urogenital zone. Due to introduction of new drug treatment protocols and the use of radiation therapy, most patients are cured by conservative methods. Only about 20 % of patients undergo surgical treatment, significant number of which are underwent organ-preserving operations. In those cases when the tumor has resistance to conservative treatment, when there is a residual tumor or relapse of the disease develops, radical organ-resecting operations are considered. Among the methods of urine derivation after cystectomy, preference is given to options that allow patients to carry out urine excretion voluntary: the formation of heterotopic reservoirs connected to the urethra or opening on the skin surfaces, which are constructed from different parts of the intestine. A clinical example of late postoperative complication is presented — a calculi formation within the pouch 7 years after radical cystprostatectomy with continent urinary diversion in a patient operated for urogenital rhabdomyosarcoma.

Актуальность. Преимуществом лапароскопических вмешательств при злокачественных новообразованиях брюшной полости у детей является уменьшение сроков послеоперационной реабилитации и раннее начало химиотерапии. Несмотря на большой опыт использования каудальной блокады в педиатрии, до сих пор не установлено, дает ли использование ультразвуковой навигации какое-либо клиническое преимущество в выполнении каудальных блокад при лапароскопических операциях в детской онкохирургии. Цель исследования. Оценить влияние каудальной эпидуральной блокады, выполненной под ультразвуковым контролем, на течение периоперационного периода и частоту послеоперационной тошноты и рвоты при лапароскопических вмешательствах. Материалы и методы. Обследовано 40 пациентов НИИ Детской онкологии и гематологии ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России, ASA II–III, оперированных в 2017–2019 гг. по поводу опухолей брюшной полости лапароскопическим методом. Все дети были разделены на 2 группы. В первую группу вошли 23 ребенка, которым на фоне сочетанной анестезии применялась каудальная эпидуральная блокада, выполняемая под контролем УЗИ (группа КА, n = 23). В группу контроля вошли 17 детей, которым проводилась общая анестезия (ОА). Проведен анализ интраоперационного и послеоперационного периодов. Результаты. Суммарная доза фентанила в группе КА составила 7,29 (6–9,25) мкг/кг, а в группе ОА — 10,7 (7,6–12,5) мкг/кг (р = 0,012). В послеоперационном периоде дополнительное обезболивание в группе КА после каудально-эпидурального введения раствора морфина не требовалось в течение 24 ч, а после каудально-эпидурального введения тримеперидина — в течение 12 ч. Энтеральное питание у детей в группе КА подключали с 4,7 ± 0,5 ч. В группе ОА энтеральное питание у 10 (59 %) пациентов подключили через 20 ч после окончания оперативного вмешательства. Заключение. Каудальная блокада, выполненная под ультразвуковым контролем до начала оперативного вмешательства, обеспечивает эффективную анальгезию в 100 % случаев, как в интра-, так и в послеоперационном периоде длительностью более 12 ч, способствует раннему началу энтерального питания, снижает частоту послеоперационной тошноты и рвоты и не сопровождается развитием осложнений.

Introduction: a solid pseudopapillary tumor (SPPT) of the pancreas is an extremely rare disease occurring in 0.13-2.7% of all pancreatic tumors. This tumor is characterized by a fairly favorable clinical course, with low metastasis potential. The clinical picture is highly non-specific, it is more common in girls of puberty and young women under 30 years of age. The main treatment method is surgical. The purpose of the study is to study the clinical and pathological characteristics of this disease and the possibilities of surgical treatment of patients with this pathology. Material and methods. The analysis of clinical data of patients diagnosed with SPPT in our Institute over the past 10 years (2007-2017). And also therу was reviewed the morphological data of SPPT, types of surgical treatment, results, and complications. Results. For 10 years, SPPT was detected in 24 patients. All patients were girls of puberty from 9 to 17 years. In most cases, the disease was asymptomatic and was diagnosed as an "accidental find" during a medical examination. Two patients had liver metastases. All patients underwent surgical treatment in a different volume: 8 patients - laparoscopic distal pancreatic resection, 3 - gastropancreatoduodenal resections, 3 - pancreatoduodenal resection, 6 - central pancreatic resections, 3 - distal subtotal pancreas resection and 1 - resection of uncinate process of the pancreas with resection of the duodenal wall. Complications occurred in 6 patients: in 4 - postoperative pancreatitis with the formation of pancreatic fistula and in one case - bleeding from the pancreatic branch of the splenic artery and bleeding from the wound surface. The follow-up period lasted from 3 months to 11 years. All patients are alive. Conclusion. SPPT is a rare tumor with a benign course potential. Clinical signs and symptoms are relatively nonspecific. Surgical treatment is the main method of treatment, although it is characterized by a high risk of postoperative complications. In general, the prognosis of this disease is favorable, even in the presence of distant metastases.