[Radical surgery in the complex treatment of hepatoblastoma]. / Chirurgická radikalita v komplexní lécbe hepatoblastomu.

BACKGROUND: Evaluation of treatment results in children with hepatoblastoma--a retrospective study of clinical and pathological data of surgically treated children. METHODS AND RESULTS: At the clinic of authors, 28 children were operated for hepatoblastoma during 1991 to 2002. Average age of patients was 2.2 years. When tumors were classified according to PRETEX system, 3 children were in the stadium I, 12 children in stadium II, 12 children in stadium III, and one child in the stadium IV. At the diagnosis, lung metastases were found in 3 children. Till 1996 the treatment was initiated by a primary operation, since 1996 by a preoperative chemotherapy. Chemotherapy was administered according to the SIOP protocols. Primary operation was done in 13 children, 15 children were operated after the pre-operation chemotherapy. Liver resection included right-sided lobectomy in 7 cases, extended right-sided lobectomy in 4 cases, left-sided lobectomy in 8 cases, right-sided trisegmentectomy in 5 cases, left-sided trisegmentectomy in 3 cases. In our studied group the three years of event free survival was achieved in 75%, overall survival in 86%. Four children died, two of them because of the progression of the disease, two for the complication during the therapy. CONCLUSIONS: Combination of the radical surgical resection with preoperative and postoperative chemotherapy enables successful treatment of the childhood hepatoblastoma.

[Use of computer tomography for localization of a biopsy needle for tissue collection from the orbit]. / Vyuzití CT pro lokalizování bioptické jehly pri odberu tkáne z ocnice.

In four patients with orbital expansion the authors used computed tomography (CT) to locate the bioptic needle inserted into the orbit. In all these instances it proved possible to collect a sufficient amount of pathological tissue to assess the diagnosis. Serious side-effects were observed only in one case, where the patient developed after insertion of the needle transient bradycardia. CT was also used to locate the needle for exhausting of blood from a lymphangioma of the orbit.

[Mediastinal teratomas in children]. / Mediastinální teratomy u detí.

The objective of the work is a clinical and histopathological retrospective study of rare mediastinal teratomas in children. The authors present their experience with the treatment of eight children within the range from neonates to the age of 15 years with a histologically confirmed diagnosis of mediastinal teratoma. In two neonates the first symptom was postnatal dypnoea, teratomas in older children were diagnosed along with symptoms of acute respiratory infection. Complete resection of the tumor was performed in all patients, in neonates emergency operations were involved. Four tumors were histologically classified as mature teratomas, three as differentiated teratomas with an immature component and one as a malignant teratoma. The malignant teratoma contained mature tissues as well as tissues of embryonic carcinoma and a yolk sac tumor. Seven children with a mature or differentiated teratoma are without signs of disease 5-15 years after surgery. During the follow-up of the 15-year-old boy with the malignant teratoma the elevated alpha-fetoprotein serum level was evidence of a relapse of the disease after termination of comprehensive treatment. From the results ensues that radical extirpation is sufficient treatment for mature and differentiated teratomas. Malignant teratomas are indicated for primary resection and postoperative chemotherapy. Long-term follow up of alpha-fetoprotein and human beta-choriogonadotropin levels, clinical controls and controls by imaging methods are indicated in children with an immature or malignant component of the tumor.

[High-dose chemotherapy in the treatment of children with malignant lymphoma]. / Vysokodávkovaná chemoterapie v lécbe detí s maligním lymfomem.

BACKGROUND: Children with primary refractory or recurrent malignant lymphoma have usually poor prognosis. Less than 10% of those, who were treated with conventional-dose regimens had survived for 2 years. In an attempt to improve the outcome for these patients, we explored the role of consolidation high-dose chemotherapy with autografting. METHODS AND RESULTS: Forty-five patients with poor-prognosis lymphoma, of whom 27 were males, underwent megatherapy between January 1992 and December 1999. High-dose chemotherapy was indicated in patients with poor initial response to first-line chemotherapy (14 cases) or in the relapse (31 cases). The group consisted of 27 patients with Hodgkin's disease and 18 patients with non-Hodgkin's lymphoma. The median age was 14.7 years. The conditioning for Hodgkin's disease patients contained cyclophosphamide, etoposide and busulfan or carmustine. Patients with non-Hodgkin's lymphomas received cyclophosphamide, etoposide and busulfan or total body irradiation. Bone marrow was used as the source of haemopoietic stem cells in ten patients, peripheral blood in twenty-eight, and both sources were used in seven patients. After the median follow-up of 47 months, the final survival was 61%. Eleven patients died of the disease progression, four of the infectious complications, one at a car accident. Median time to relapse after the transplantation was 7.5 months. CONCLUSIONS: Further improvement of these results will require earlier transplantation, improved preparative regiments or early posttransplant immunotherapy.

Papillary cystic and solid tumor of the pancreas--surgical therapy with the use of CUSA, and a review of the pediatric literature.

Papillary cystic and solid tumor of the pancreas (PCSTP), so-called Frantz tumor, is a very rare tumor in children. Only 62 cases, 57 girls and 5 boys, have been reported in children since 1959. The tumor presents usually as a slowly growing abdominal mass with or without abdominal pain. Surgical resection of the tumor is an adequate mode of treatment, and the prognosis is excellent. The authors present 4 girls and 1 boy with PCSTP and demonstrate that the Cavitron Ultrasonic Surgical Aspirator (CUSA, Valleylab) is successfully used in surgical therapy.

[Rupture of the pancreas in children--present trends in diagnosis and therapy]. / Ruptura pankreatu v detském veku--soucasné trendy v diagnostice a terapii.

Evaluation of pancreatic injuries is presented in the professional literature in retrospective studies and views on treatment vary. The authors evaluated also in a retrospective study a group of eight child patients with a serious injury of the pancreas. The patients were 3-13 years old and were hospitalized at the Clinic of Paediatric Surgery at the Faculty Hospital Motol in 1985-1999. The group is formed by patients with pancreatic injuries (according to the Japanese classification type II and III). The authors evaluate not only diagnostic but also therapeutic procedures. In the diagnosis they evaluate the ultrasonographic examination (USG) as the examination of first choice which however need not record the extent and severity of the pancreatic injury. Computing tomography (CT) and magnetic resonance (MRI) can make the original suspicion of injury more accurate, ERCP is an examination which reveals in particular type III injuries--i.e. those with affection of the pancreatic duct. Injured children where a serious pancreatic lesion was not diagnosed primarily, had to be subjected to repeated surgical revisions. All children in the presented group recovered, none of the patients died.

[Surgical treatment of pectus excavatum and pectus carinatum]. / Chirurgické lécení pectus excavatum a pectus carinatum.

The authors present their experience with surgery of deformities of the chest wall in childhood. In the course of 1986 to 1992 they operated 93 children with the diagnosis of pectus excavatum. This number comprised 66 boys and 27 girls. The mean age of the operated children was 10.5 years. During the same period 17 patients (12 boys and 5 girls) were operated on account of pectus carinatum. The mean age of the latter group of patients was 13.5 years. 23% of the patients with a pectus excavatum had before operation mild scoliosis, two patients suffered from severe scoliosis. 76 patients after operation of pectus excavatum were followed up for more than one year after operation. In 44 patients the result was excellent (58%), in 31 patients the result was good. One patient developed a relapse. Fifteen patients after operation for pectus carinatum were followed up for more than one year. In 12 patients (80%) the result of the operation is excellent, in three patients (20%) the result is good. From the group as a whole seven patients had dehiscence of the wound after surgery, four patients developed a seroma in the surgical wound, three patients developed pneumothorax after operation and in one instance an abscess developed in the surgical wound. In the course of 1987 the authors changed the surgical technique in pectus excavatum from Jensen's to Holcomb jun. operation.

[Cystic lymphangioma of the retroperitoneum]. / Cystický lymfangiom retroperitonea.

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.