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Retroperitoneal sarcomas are rare malignant neoplasms that are often diagnosed late because of their typical nonspecific symptomatology. Radical surgical resection, if possible given the local anatomical conditions, is the most important treatment modality. However, even if the surgery is feasible and successful, the prognosis of retroperitoneal sarcoma is not good. The most frequent type of recurrence is local recurrence, and radical surgical resection remains the most important treatment modality even in such cases.Our study presents a case report of a patient with a retroperitoneal leiomyosarcoma that was radically (R0) removed. Two years later a local recurrence developed, which was eventually diagnosed by MRI. Again, radical (R0) surgical resection was approached (because of tumor invasion into inferior caval vein, the vein had to be partially resected as well and substituted with a vascular prosthesis).The report describes a relatively typical case of retroperitoneal sarcoma, while pointing out the importance of timely diagnosis (which is definitely not easy to achieve) and especially of radical surgical treatment.Key words: retroperitoneal sarcoma recurrence surgery.

Castleman disease is rare diagnosis. It was first mentioned in literature in 1954 by doctor Castleman and Lown. It is rare sickness which primary affects lymfatic nodes. There are four histo-morfologic subtypes and two clinic manifestations (uni and mulicentric). Comon clinical picture is simple lymfadenopathy of neck, medistinal a retroperitonal lymfatic nodes. Signs of this disease are heterogenic and depend on histological subtype. Also prognosis depends on type of illness. It should take place as benign diagnosis (unicentric form) but also can be potencionally malignant form which shlould exacerbate to malignant lymfoproliferation (multicentric form). Authors present the case of 29 years old men with clinical manifestation (jundice, intermitent bowel obstruction) of lagre retroperitoneal tumor in subhepatal localization. The treatment was radical surgical extirpation, surgery was without complications. Definitive histological diagnosis was confirmed as hyaline-vascular type of Castleman disease. Because the patient´s clinical form was multicentric, he is now undergoing adjuvant oncological treatment.Castleman disease reproperitoneal tumor.

The authors present the case of a 57-year-old woman with a very rare extragenital malignant retroperitoneal Müllerian carcinosarcoma invading the inferior vena cava. Tumor resection with partial resection of the vena cava wall and resection of metastases in the pelvic area is described. The authors further discuss diagnostic options of metastases of this tumour and the recommended adjuvant chemotherapy.extragenital Müllerian carcinosarcoma malignant mixed Müllerian tumour - diagnosis therapy.

A case report of a female patient with pathological liquid collection in the retroperitoneum with chronic and recurrent pain in the epigastric region and dyspepsia. After a safe laparoscopic approach and removal of a cystic tumour, the finding was histologically described as lymphangioma.

Various types of neurogenous tumours occur in the retroperitoneum. Hormonally inactive tumours are often asymptomatic and their diagnosis is mostly accidental. Authors are presenting their recent experience with three cases. They discuss diagnostic options and the therapy of retroperitoneal schwannoma, ganglioneuroma and paraganglioma.

Lymphangioleiomyomatosis (LAM) is a rare progressive disease affecting women of childbearing age. The disease is characterised by an abnormal proliferation of immature smooth muscle cells predominantly in the lung. It gradually leads to respiratory failure, and it frequently result in death. Extrapulmonary LAM typically presents with abdominal mass, abdominal pain and chylous ascites. In the case reports we describe two cases of premenopausal females with extrapulmonary LAM. In both cases they occur in pelvic location in the obturator fossa and around the external iliac artery. After surgical procedures patients were primary treated with progesterone. Sirolimus was second-line drugs.

Liposarcomas of the retroperitoneal area (LSRP) are rare mesenchymal tumour, which are literary ranked among the retroperitoneal soft-tissue sarcomas, they can reach enormous size, while patients complains aren't distinctive. The most common symptoms are abdominal discomfort and distention of abdominal volume with a palpable resistention. The only successful treatment of this tumor is surgical exstirpation. CASE REPORT 1: 77-year-old patient was investigated because of gradually accruing size of the abdomen. There was a lipoid tumour in the peritoneal cavity shown by computed tomography, suspected coming out of the retroperitoneal area. During the operation we found out giant well-differentiated liposarcoma which grew from the retroperitoneal area. Postoperative period was complicated by bleeding and consequently by MOF. 14th postoperative day patient died. Dissection finding was another location of liposarcomas in the retroperitoneal area. CASE REPORT 2: 66-year-old patient was investigated because of increasing girth. Abdominal ultrasonography and CT displayed adipoid formation which filled up most of the peritoneal cavity with compression of surrounding organs. The tumour was completely resected during operation. The patient has been followed in dispensatory for two years without any signs of recurrence of the illness.Liposarcomas are the most frequent histological type among primary retroperitoneal sarcomas. Metastatic potential of LSRP is low but these tumors often recur locally. In diagnostics and in follow-up are essential CT and MRI. Successful therapy is radical resection. It could be difficult to distinguish giant well-differentiated liposarcomas from normal retroperitoneal fat. Incomplete resection is a palliation operation without chance for long survival.

Primary retroperitoneal tumors are extremely rare tumors of this location. The authors present a case review of a 53-year-old patient operated for unexplained retroperitoneal neoplasm. No complications were recorded during the postoperative course and histology demonstrated extraadrenal paraganglioma structures, ranked among the above neoplasms. Preoperative hypertension subsided postoperatively, following the active neuroendocrinne tumor removal. The aim of this work is to present clinical and histopathological characteristics of this rare retroperitoenal neoplasm.

Localization of hemangioma in retroperineum causes late diagnosis of the disease. The tumour is usually detected only when the clinical symptoms caused by the pressure of the surrounding tissues occur. This article presents the case of retroperitoneal hemangioma preoperatively diagnosed as suspect feochromocytoma.

The authors present surgical therapy of liposarcoma in retroperitoneum. These mesenchymal tumors occur rarely in the population. The strategy for therapy requires determination of the relations to adjacent organs on the basis of precise preoperation examination of the patient. Surgery is considered as the basic therapy, provided radical extirpation of the tumor is possible. Our case report describes a huge liposarcoma in retroperitoneum with histology evolving from myxoid to low differentiated liposarcoma of retroperitoneum. The repeated relapse of the tumor was, as fart as possible, treated by extirpation of the tumor.

Two statistically irrelevant case histories of abdominal dissemination of malignant neuroblastoma with the retroperitoneum confirm the preference of an active surgical approach even in onstensible hopeless malignant disseminations of unknown origin. Via the achievement of extensive cytoreduction, the terrain become ready for subsequent complex therapy, with the possibility of Sugarbaker's operation (peritonectomia) with hypertermic intraperitoneal intraoperative chemotherapy.

The case-history presents a case of a rare retroperitoneal tumour--primary retroperitoneal classical seminoma. This type of tumour accounts for half the extragonadal germinal tumours. From the clinical aspect this group is important as in addition to surgery it can be successfully treated by chemotherapy and radiation. The clinical findings in the authors' patient led to the diagnosis of a retroperitoneal tumour and after repeated examinations the authors concluded on the presence of some form of malignant mesenchymal tumour. A more aggressive surgical procedure implies radical removal of the tumour, and treatment was, based on the final histological diagnosis, supplemented by chemotherapy. Despite the favourable diagnosis of seminomas, we have to foresee in case of a primary tumour in the retroperitoneum a poorer prognosis (sometimes by as much as 50%) and despite all conclusions attention should be devoted to a possible late development a testicular tumour.

The authors submit their experience with the surgical treatment of retroperitoneal neuroblastoma, which is one of the most frequent solid tumours of child age. They describe the principles of the surgical technique with emphasis on radical removal of the tumour even in the area of large blood vessels. They emphasize the advantage of using an ultrasonic surgical aspirator (CUSA, Valleylab Co., Pfizer) which makes it possible to remove residues of the tumour without damaging the large blood vessels. During the period between 1987-1992 they treated at the Motol Faculty Hospital 69 children with retroperitoneal neuroblastoma. Of these 60 were operated. In the first stage of the disease 9 patients were operated, all survive. In the second stage 4 patients were operated, 75% survive, in the third stage 24 patients 50% survive, in the fourth stage 20 of 29 patients were operated and 11 (38%) survive. In stage IV--S three patients were operated, one survives. The total survival of patients during the investigation period is 52%.

The author presents sonomorphological characteristics of retroperitoneal neuroblastoma, nephroblastoma, non-Hodgkin lymphoma and M. Hodgkin abdominis, hepatoblastoma and hepatocellular carcinoma at the time when the diagnosis was established. The objective of the investigation was to increase the sensitivity and specificity of ultrasonic diagnosis of the mentioned malignant tumours of the abdomen and retroperitoneum during the first sonographic examination.

The authors present the case-history of a patient with a cystic retroperitoneal lymphangioma. Its diagnosis was established only by histological examination of the resected tissue. The authors draw attention to the symptomatology, diagnosis, therapy and discuss contemporary views regarding the cause of this disease.

A busky tumour of yellow colour with pus-filled central cavities was removed from the left retroperitoneum of a 54 year old man. Histology revealed it to be a rare variant of fibrous histiocytoma characterized by an extremely large quantity of mature neutrophil granulocytes in all the tumour tissue messively phagocyted by tumour histiocytes. There was no relapse or dissemination when postmortem was performed 5 months after the operation.

In a group of 212 patients with germinal tumours of the testicle treated at the urological clinic in Hradec Králové in 1975-1987 there were 7 with an extragonadal tumour in the retroperitoneum. In all the dominating symptom was a palpable resistance in the epigastrium, backache and secondary signs of malignant disease. In the first four a radical extirpation of the tumour was performed, in one with replacement of the aorta always with left nephrectomy. After introduction of cisplatinum into combined chemotherapy this became the initial treatment. Of the seven patients two died from generalization of the basic disease, the third one from exsanguination through an erosion of an aortal graft.