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To assess the influence of active surgical approach on the long-term postoperative outcomes in patients with retroperitoneal liposarcoma.A retrospective study included 190 patients with retroperitoneal liposarcoma. The effect of malignancy grade, adjuvant chemotherapy, number of separate tumor nodes in primary neoplasm and the first relapse, as well as the number of previous total resections on survival rate was analyzed.Overall and relapse-free survival is significantly worse in patients with high-grade retroperitoneal liposarcoma (G2-3) compared to low-grade (G1) tumor (The only potentially radical treatment for patients with retroperitoneal liposarcoma is surgery. We reported the advantages of active surgical approach for improvement of long-term outcomes in patients with retroperitoneal liposarcoma.Оценить влияние активной хирургической тактики на отдаленные результаты хирургического лечения больных забрюшинными липосаркомами (ЗЛС).В ретроспективное исследование включены 190 пациентов с ЗЛС. Изучено влияние на выживаемость степени злокачественности ЗЛС, адъювантной химиотерапии, количества отдельно расположенных опухолевых узлов при первичной опухоли и первом рецидиве, количества перенесенных радикальных операций.Общая (ОВ) и безрецидивная (БВ) выживаемость статистически значимо хуже при забрюшинной неорганной липосаркоме high grade (G2-3) по сравнению с low grade (G1) (Единственным потенциально радикальным методом лечения больных с ЗЛС является хирургический. В работе показана необходимость активной хирургической тактики для улучшения отдаленных результатов лечения больных с ЗЛС.

To evaluate feasibility and advantages of laparoscopic access in children with retroperitoneal neurogenic tumors.A comparative analysis of postoperative results was performed in 2 groups of patients with retroperitoneal neurogenic tumors without IDRF risk factors. Different surgical approaches were applied. The main group (laparoscopic access) consisted of 18 patients. The control group included 22 patients after laparotomy. We compared the following variables: surgery time, blood loss, postoperative need for analgesics, intraoperative and postoperative complications, timing of postoperative enteral feeding, postoperative hospital-stay and regional recurrence rate.Duration of laparoscopic and open surgery was similar (80.5 vs. 86.5 min,Laparoscopic resection of neurogenic retroperitoneal tumors in children is feasible if great vessels are not involved in neoplastic process. Open surgery is preferable in patients with invasion of great vessels.Обосновать целесообразность и преимущества лапароскопического доступа у детей с нейрогенными опухолями забрюшинного пространства.Проведен сравнительный анализ результатов лечения двух групп пациентов с нейрогенными опухолями забрюшинного пространства без факторов риска IDRF, оперированных из разных хирургических доступов. Основную группу составили 18 детей, оперированных с использованием лапароскопического доступа. В группу сравнения вошли 22 пациента, которым оперативное вмешательство выполнили открытым лапаротомным доступом. Группы исследования сравнивали по следующим параметрам: длительность операции, объем интраоперационной кровопотери, потребность в наркотических анальгетиках в послеоперационном периоде, частота развития интраоперационных и послеоперационных осложнений, срок возобновления энтерального питания, продолжительность пребывания пациента в стационаре после операции. Проведен анализ локальных событий.При сравнении длительности лапароскопических и открытых операций статистически значимого различия между группами не отмечено (80,5 мин против 86,5 мин,Применение лапароскопического доступа для удаления нейрогенных опухолей забрюшиннного пространства у детей целесообразно при отсутствии достоверных признаков инвазии опухоли в магистральные сосуды. При наличии признаков прорастания опухоли в магистральные сосуды целесообразно выбирать традиционный вариант оперативного вмешательства.

To determine the optimal surgical approach in patients with abdominal and retroperitoneal manifestations of Recklinghausen's disease.There were 4 patients (3 women and 1 man) with neurofibromatosis type I who were treated at Vishnevsky National Medical Research Center of Surgery.There were 3 robot-assisted procedures: excision of retroperitoneal tumors (plexiform neurofibroma and schwannoma) in 2 cases and right adrenal pheochromocytoma in 1 patient. We also included 1 clinical case of conventional surgery for neurofibromatosis type I followed by multiple gastrointestinal stromal duodenal and intestinal tumors. In one case, postoperative period was complicated by fluid accumulation in the bed of previously removed tumor that required US-assisted drainage. Postoperative period was uneventful in other cases.Robot-assisted surgery is safe and effective in patients with Recklinghausen's disease followed by single abdominal and retroperitoneal tumors. It is more expedient to choose conventional technique for multiple tumors located in different parts of retroperitoneal space or abdominal cavity.Нейрофиброматоз I типа (болезнь Реклингхаузена) - наследственное (аутосомно-доминантное заболевание), вызванное мутацией гена NF1. Опухоли брюшной полости и забрюшинного пространства при болезни Реклингхаузена встречаются редко.Выбор оптимального метода хирургического лечения у пациентов с проявлениями болезни Реклингхаузена в брюшной полости и забрюшинном пространстве.В НМИЦ хирургии им. А.В. Вишневского хирургическое лечение выполнено 4 пациентам (3 женщины и 1 мужчина) с нейрофиброматозом I типа.Выполнены 3 робот-ассистированные операции по удалению 2 неорганных забрюшинных опухолей (плексиформная нейрофиброма и шваннома) и 1 феохромоцитомы правого надпочечника. В исследование также включено одно клиническое наблюдение традиционного хирургического вмешательства по поводу множественных гастроинтестинальных стромальных опухолей двенадцатиперстной и тонкой кишки на фоне нейрофиброматоза I типа. В одном случае послеоперационный период осложнился формированием жидкостного скопления в ложе удаленной опухоли, что потребовало проведения дренирования под УЗ-контролем. В остальных случаях послеоперационный период протекал без осложнений.Проведение робот-ассистированных операций в лечении проявлений нейрофиброматоза I типа в забрюшинном пространстве и брюшной полости безопасно и эффективно у пациентов с болезнью Реклингхаузена при наличии единичных образований. При множественных опухолях, расположенных в разных отделах забрюшинного пространства или брюшной полости, целесообразно выбирать традиционный вариант оперативного вмешательства.

Retroperitoneal tumors, in general, are rare tumors which histopathological characteristics and biological behavior can be considered as benign or malignant. They originate from various tissue elements located in the retroperitoneal space. They often cover Anatomical structures of varying importance - mainly retroperitoneal vessels and organs, which determines the type of surgery - radical or palliative. They are composed of adipose tissue, vascular elements, smooth and striated muscle, neural elements germline structures. There are three main types of retroperitoneal tumors: mesodermal, neurogenic and teratosarcomas. As mentioned above each can be either benign and malignant. In most cases there are no specific symptoms except general weakness, heaviness in the abdomen, loss of appetite, progressive weight loss, headache and fever. The pain is most often located in the lumbosacral area. The tumor can also cause disturbances in urination. In patients with advanced stage can be observed significantly symmetrical or asymmetrical tumor growth in the abdominal area. The most commonly used techniques for the diagnosis of the disease are X-ray, CT-scan and MRI.Retrospective study involving 112 cases operated in the clinic for 14-years period (2000-2014) - 101 patients, 11 of them were operated on twice in a different time interval on the occasion of relapsed malignant process.Out of all operated cases, 42% are males (47 cases) and 58% - women (65 cases). In most cases (72% - 81 cases) tumors are malignant. All patients were treated surgically. There were followed up a total of 89 patients over a period of 3 months to 8 years (96 months) as of the studied malignant cases 3 year survival was observed in 21 patients, and one patient has survived 96 months after total extirpation of fibrosarcoma. Early postoperative mortality (till 20th postoperative day) was registered in 3 patients - 2.67% of all operated cases.About 80% of primary retroperitoneal extra-organ tumors are generally malignant. People of all ages are affected equally and no statistically significant difference in the number of men and women is detected. There are many theories about the emergence of retroperitoneal tumors, but currently none of them has not been fully proven. Treatment depends on the type of the lesion. Surgery is the main approach and it should be applied to both benign and malignant lesions. In the case of malignancy other methods than surgical are chemo- and radiotherapy but they are less effective.

Robot-assisted operations for non-organ retroperitoneal tumors were performed in 20 patients. The biggest tumor's diameter was 160 mm, the smallest--32 mm, mean value was 79.1±37.8 mm. According to morphological investigation data lymphangioma was diagnosed in 4 (20%) patients, lipoma--in 3 (15%) cases, peritoneal cyst--in 2 (10%) patients. Solitary lymphatic node, teratoma, leiomyosarcoma, accessory stomach, bronchogenic cyst, adenogenous cancer, ureterocele, megaureter, schwannoma, leiomyoma, malignant paraganglioma were observed in 1 (5%) case respectively.Surgery duration was 138±55.9 min. Blood loss was 69±112.7 ml (range 0-500 ml). Intraoperative complications (bleeding) were diagnosed in 3 (15%) patients. There were no conversions. Robot-assisted variant has been demonstrated in case of non-organ retroperitoneal tumors with diameter not more than 100-120 mm.Цель - представить технику робот-ассистированных операций и результаты хирургического лечения больных с неорганными забрюшинными опухолями. Материал и методы. Робот-ассистированные операции по поводу неорганных забрюшинных опухолей (НЗО) выполнены 20 больным. Наибольший диаметр опухоли был 160 мм, наименьший - 32 мм, среднее значение составило 79,1±37,8 мм. На основании данных морфологического исследования лимфангиома была диагностирована у 4 (20%) больных, липома - у 3 (15%), киста брюшины - у 2 (10%), по 1 (5%) больному было с солитарным лимфатическим узлом, тератомой, лейомиосаркомой, энтерогенной кистой, бронхогенной кистой, аденогенным раком, уретероцеле, мегауретером, шванномой, лейомиомой, злокачественной параганглиомой. Результаты. Продолжительность операции составила 138±55,9 мин. Объем кровопотери 69±112,7 мл (от 0 до 500 мл). Интраоперационные осложнения (кровотечения) зафиксированы у 3 (15%) пациентов. Конверсий доступа не было. Робот-ассистированный вариант операции показан при НЗО размером не более 100-120 мм.

Retrospective analysis of 228 patients with retroperitoneal liposarcoma, operated on in the Russian Oncology Center during 1971-2010, has been done. Of 223 performed operations, 124 were combined, i.e., required multi-organ resection. The kidney was the most frequently removed organ both in cases of primary and recurrent tumors. 10-year survival rate was 26% after combined operations and 35% after surgery, they did not need the multiorgan resection.

Retroperitoneal lesions/sarcomas are rare. Diagnosis is difficult and needs special methods. Diagnosis and Treatment is an interdisciplinary process. Molecular Diagnostics will become more and more necessary in the future.

Data of 5 patients, who required resection and prosthetics of abdominal aorta during radical excision of malignant retroperitoneal lesions, were analyzed. Therefore, 2 patients demonstrated retroperitoneal lymph node metastases of nonseminoma hermonogenous tumour, 1 patient had colorectal liver and lymph node metastases, another had ovarian tumor, and the rest--leiomyosarcoma of the aorta. Tubular Gore-tex aortic prostheses were used. Radical tumor excision provided absence of local recurrence in all patients. Only one patient showed further progression of the tumor as liver metastases. Thus, such an aggressive surgical approach could be appropriate in carefully selected group of patients.

Teratomas are germ cell tumors of a nonseminomatous type. Their incidence is characterized with a bimodal curve, but basically they are a pathology of the small age. Primary localization in the retroperitoneal space is seen in over 40% of cases with teratoma. Preoperative differentiation from other bulky pathologic processes in this region presents a challenge to the surgeon. An interesting case of a primary retroperitoneal teratoma in a 23 year old male is presented and a review of the literature is done.

Malignant neurogenic tumors /MNT/ are the third by frequency malignancies in children with mainly localization in the retroperitoneal space and posterior mediastinum.of this study is to compare the clinical presentation and the treatment results by young children with retroperitoneal and mediastinal MNT.For a period of 12 years (1993 -2004) 52 children aged fron 1 month to 3 years were admitted at the Department of Pediatric surgery-University Hospital "N.I.Pirogov", Sofia. The findings of different diagnostic methods are reviewed. Age and sex of the children, the frequency of location, maturity and stage of the tumor, type of operations and survival rate are compared.The MNT in young children encounter more common in the retroperitoneal space and are diagnosed in advanced clinical stage due to late clinical presentation. Most of them have an unfavorable histological variant and affect predominantly the adrenal glands. The mediastinal MNT are mainly manifested in the first age of life with good prognosis. The total survival up to 2004 year is 59,6 %.

Retroperitoneal tumors engaging large vessels require vascular reconstruction when surgically removed. We present a case of retroperitoneal rhabdomiosarcoma in a child, which required inferior vena cava (IVC) grafting, creating a new bifurcation of IVC, and an A-V fistula between greater saphenous vein and femoral artery as well as grafting of right common iliac artery.

Primary retroperitoneal extra-organ tumors (PRET) are a relatively rare group of diverse neoplasms. They are classified in three main groups according to their histogenesis: PRET from mesodermal origin, from ectodermal origin and from embryonic remnants. Most of PRET are malignant, a the most frequent malignant PRET are soft tissue sarcomas. Because of their specific localization PRETs grow silently for a relatively long period and are often considerably large at the time of diagnosis. These tumors usually have no specific symptoms. Computerized tomography and magnetic resonance imaging are the most useful methods in the diagnosis of PRET. Surgery is the only effective treatment of PRET with exclusion of some histologic types as lymphoma and malignant germ cell tumors. One of the most specific characters of PRET is their great tendency to recur locally, which is explained not only by their biological characteristics but the difficulties of accomplishing a radical resection. For the malignant PRET, especially the retroperitoneal soft tissue sarcomas, no adjuvant therapy has proved to be beneficial on survival.

Ectopic splenic tissue can be found as congenital or acquired condition. It is occasionally symptomatic and very rarely can simulate a neoplastic disease, especially when it locates in the retroperitoneal space. A case of ectopic splenic tissue simulating a retroperitoneal tumor is reported. A 32-years-old female was admitted in the clinic for a tumor in the left retroperitoneal space, discovered by ultrasound and computed tomography. 9 years before, an operation for a pheochromocytoma with similar localization was performed as the tumor was removed together with the spleen. At this admission, the tumor was considered a local recurrence of the pheochromocytoma and was resected. The result of the histopathological examination showed a splenic tissue.

In a patient, a woman of 62 years, one surgical team has performed consecutively over 3 h 45 min 10 operations for different diseases: extirpation of the uterus with appendages, removal of retroperitoneal fibrosarcoma, appendectomy, 30 cm-resection of the small intestine, polypectomy from the rectum, creation of sygmorectoanastomosis, plastic reconstruction of postoperative ventral hernia with duplication, sectoral resection of mammary gland, removal of papilloma and lipoma of abdominal wall. The outcome is favorable.

The clinical relevance of laparoscopy (LS) and laparoscopic echography (LSEG) in diagnosing and staging gastrointestinal carcinomas is assayed in the light of diagnostic methods in current use. The study covers 257 patients undergoing LS, and 23-LSEG. For the purpose a rigid 7.5 MHz echolaparoscopic probe is used. In 16 per cent of the cases LS fails to resolve the basic diagnostic problems faced, whereas in 35 per cent it alters completely the preliminary clinical diagnosis. In over 90 per cent of cases LSEG supplements or modifies the laparoscopic diagnosis. Emphasis is laid on the role played by LSEG in defining the loco-regional stage of gastric and colorectal carcinomas, as well as on the contribution of the method to establish pancreatic carcinomas not lending themselves to resection. What is more, LSEG demonstrates inoperable cases by detecting small, unsuspected metastases, not prominating above the hepatic surface.

Observations on one of the rare complications in children presenting malignant neoplasms of the abdominal cavity and retroperitoneal space are described. Over a 7-year period, operative treatment is undertaken in 42 children aged 3 to 15 years. In eleven of them (26.42 per cent) it is a matter of mechanical ileus. The type of bowel obstruction in the series of children under study is a follows: obturation-in three and adhesion-in five cases. The obturation involves the large intestine, and is due to pressure of a neoplastic process in advanced stage of development on the colon. Ileus due to adhesions occurs after operative removal of the neoplastic formation. The essential differences in type of intestinal obstruction in children with malignant neoformation in the abdominal region from the one in adult patients justify the report on the observations.

Observations of successfully operated huge retroperitoneal tumors, one of them weighing 14.700 kg, are reported. The advantages for precise diagnosis of modern visualizing techniques--echography and computer tomography--and the inability to establish always an exact cytologic diagnosis by puncture aspiration biopsy are demonstrated. Total extirpation of the tumor with subsequent combined chemo-, radio- and immunotherapy is advised.

Sixteen years experience (1974-1989) is recorded with the treatment of 18 patients with retroperitoneal sarcoma. The tumor was palpable in all. Four syndromes could be differentiated in these patients: gastrointestinal, renal, vascular and neurologic. All modern methods of diagnosis were applied: ultrasonography in 12 patients, computer tomography--13, angiography--3, X-ray examination of the gastrointestinal tract--7, gamma-renography--5, urography--2. Radical operative intervention is considered treatment of choice, followed by radio- or hemotherapy. Radical extirpation of the tumor was performed in 10 patients (55.5 per cent). Extended operative interventions with resection of adjacent organs were performed in 4 patients (22.2 per cent). After histologic verification 10 patients (55.5 per cent) were subjected to chemotherapy and 7 to radiotherapy. Postoperative case fatality rate was 17 per cent (3 patients died). Four patients 22.2 per cent) had relapse of the tumor between the first and the third year.

Experience with 62 combined operations on patients with nonorganic new growths of the true pelvis is discussed. They accounted for 33.3% of 227 radical interventions undertaken for this disease. The ages of the patients ranged from 4 months to 64 years. Ten patients were admitted after exploratory laparotomies. Benign new growths were diagnosed in 14 and malignant new growths in 48 patients. Different variants of operative approaches were used. Most frequently the tumor was resected together with bones, iliac vessels, genitalia, and urinary bladder. Various complications were noted in 39 patients who were operated on. Hemorrhage was the most frequent and menacing complication--in 26 patients. Two patients died. Recurrences were found in 8 of a group of 60 patients discharge from the clinic. After combined operations for malignant nonorganic tumors of the true pelvis 3-year survival was 36.0%, 5-year survival was 29.4%.

The results of observations over 116 patients with nonorganic retroperitoneal tumors of the pelvis in the diagnosis of which computerized and ultrasonic tomography were used among other methods showed these methods to expand the possibilities of early diagnosis significantly. Analysis of 83 operations conducted on patients with nonorganic tumors of the pelvis revealed the main reasons why a radical operative intervention failed to be performed. Hemorrhage and pyo-inflammatory processes in the pelvic fat were the most common and serious complications in the postoperative period.

The possibilities of various methods of examination in 22 patients with nonorganic retroperitoneal tumors were evaluated on the basis of complex diagnosis. The importance of ultrasonic examination for determining the tactics of the management of patients is shown. The method improves the results of examination of patients with nonorganic retroperitoneal tumors and allows the volume of radio-endoscopic studies to be reduced.

The authors had 170 patients with retroperitoneal nonorganic tumors under observation. Operations were conducted on 143 patients. Patients with malignant tumors received intensive radiotherapy in a dose of 5Gr every other day to a total dose of 20-30 Gr. A course of telegammatherapy was applied in 17 patients in the preoperative period; in 22 patients the seat of the removed tumor was treated by telegammatherapy after radical operation. In 25 patients telegammatherapy was conducted as an independent method of treatment in inoperable cases and after exploratory laparotomy. Operative treatment of patients with benign tumors produced the best late-term results. Among patients treated for malignant new growths the late-term results were best when the seat of the removed tumor was exposed to intensive radiotherapy.