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J Stefanowicz

Publications and source records attributed to J Stefanowicz.

5 recordsLinked to original sources

Heterogeneity of extraparenchymal primitive neuroectodermal tumors within the craniospinal axis.

Four cases of primitive neuroectodermal tumors (PNETs) with unusual localization (three intraspinal extramedullary and one pontocerebellar) are reviewed. Histologically, they were small round blue cell tumors with diverse patterns. Immunohistochemically, all tumors were positive for at least two neuronal markers, two cases were Mic-2 positive and one showed glial differentiation. The paraffin-embedded tumor specimens were examined by interphase FISH using dual-color probes specific for EWS, HER-2 and BCR loci. Molecular cytogenetic study revealed the presence of EWS rearrangement in two cases and the presence of i(17q) in one tumor. Three tumors exhibited 22 disomy and one was 22 polyploid. Extraparenchymal PNETs within craniospinal axis are heterogeneous from the clinical, histological, immunohistochemical and molecular point of view. These PNETs can be of a central or peripheral type. Multidisciplinary approach is of a basic importance in differential diagnosis of such cases.

Adolescent↗

Peripheral primitive neuroectodermal tumor within the spinal epidural space.

A case of an epidural spinal peripheral primitive neuroectodermal tumor (pPNET) in a 13-year-old girl is presented. The tumor was disseminated at the moment of diagnosis, thus only diagnostic oligobiopsy of the epidural mass was performed. Histologically the tumor was composed of small round blue cells. The neoplastic cells expressed MIC2 and features of neural differentiation on immunohistochemical staining (neuron-specific enolase, synaptophysin and NCAM positivity). Fluorescent in situ hybridization (FISH) analysis was performed for the final diagnosis and the translocation t(11;22)(q24;q12) was detected. The present case emphasizes the usefulness of FISH in differential diagnosis of tumors, especially when only routinely fixed material is available.

12E7 Antigen↗

[Clinical course and risk of fungal infections development in children with treated with chemo- and radiotherapy for solid tumors].

The authors estimated the incidence of mycotic infections among children patients with histological diagnosis of solid tumors during the period of neutropenia. Superficial mycoses including mucoses were observed in 35.3% of analysed patients, while a disseminated mycotic infections of a severe clinical course were seen in 2.9% neutropenic patients. Prophylactic administration of Diflucan (1-2 mg/kg/24h)(fluconazole) in neutropenic patients proved efficient in great majority of children, preventing the occurrence of severe mycotic complications.

Antifungal Agents↗

[Enormous tumour in the right atrium of a Wilms' tumour patient].

Formation of neoplastic thrombus in vena cava inferior (VCI) is well known in Wilms' tumour. We demonstrate a case of large neoplastic mass in the right atrium as prolongation from vena cava inferior in a 2 years and 2 months old girl with Wilms' tumour. The long preoperative chemotherapy (7 months) reduced the tumour and the thrombus. The surgical removal of the tumour and the thrombus was followed by further chemotherapy. During postoperative chemotherapy the control USG examinations showed the presence of a stable mass in a caval wall (a neoplastic mass or a scar?). The extension of the thrombus into the right atrium remains a therapeutic problem which has not been yet resolved.

Chemotherapy, Adjuvant↗

[Application of Ethyol during chemotherapy in patients with impaired kidney function. Description of three cases].

Nephrotoxicity is observed in 30% of patient's treated with chemotherapy schedules based on cisplatin analogues. Ethyol (amiphostin) was used as a protectant of kidney in 3 paediatric patients with neoplastic disease. Two patients suffered from Wilms tumour. One of them, a 5 month old boy presented with agenesis of the right kidney and stage II tumour in the left kidney. Bilateral Wilms tumour was recognised in a 6 year old girl. In both cases glomerular filtration was diminished. The third patient was a girl aged 5 who had isolated relapse of neuroblastoma in the central nervous system. Primary treatment caused nephrotoxic complications such as Fanconi syndrome and diminished glomerular filtration (66 ml/min). In these 3 cases Ethyol was used every lime before analogues of cisplatin were given. Tolerance and the effect of this protectant were good.

Amifostine↗