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Biomedical subjects

W Biernat

Publications and source records attributed to W Biernat.

At least 19 recordsLinked to original sources

Successful treatment of leukaemia cutis with cladribine in a patient with B-cell chronic lymphocytic leukaemia.

Cutaneous presentation of B-cell chronic lymphocytic leukaemia (B-CLL) is uncommon, and the influence of skin changes on B-CLL prognosis is unclear. We report a patient with B-CLL Rai II, with multiple nodular skin infiltrations on the trunk, upper arms and thighs as well as constitutional symptoms, who was successfully treated with cladribine. The peripheral blood (PB) lymphocytes were CD19, CD20, CD23 and CD5 positive, which confirmed the diagnosis of B-CLL. Skin biopsy of one of the lesions showed an intense infiltrate composed of small lymphocytes with no epidermotropism. These cells also showed the expression of CD19, CD20, CD23 and CD5 antigens similar to those presented on PB lymphocytes. Polymerase chain reaction performed on bone marrow lymphocytes and a lesional skin biopsy using consensus primers for immunoglobulin heavy-chain genes also showed the same monoclonal population of B lymphocytes both in the bone marrow and in the skin. The patient received four courses of cladribine 0.12 mg kg-1 daily as a 2-h infusion for five consecutive days. The courses were repeated at monthly intervals. The lymphocytosis gradually decreased and the PB count normalized after three courses. At the same time, a significant decrease in the cutaneous symptoms was observed. The patient became free of skin tumours after the fourth course of cladribine; only slight discoloration at the previous sites of cutaneous infiltration remained. There was no relapse of leukaemia cutis during a further 7 months of observation.

Aged↗

January 2000: 12 year old boy with recent onset seizures.

A 12-year old boy presented with new onset of seizures and a CT scan showed a left frontal lobe tumor which was removed completely. Neuropathological examination showed a pleomorphic ganglion cell tumor with necrosi, and endothelial proliferation. The diagnosis was extraventricular atypical neurocytic neoplasm ("cystic ganglioneurocytoma").

Brain Neoplasms↗

Ductal sebaceoma (sebomatricoma).

We present a case of an unusual tumour arising in the forehead of a 52-year-old female. The tumour, diagnosed as sebaceoma (or sebomatricoma), showed predominantly differentiation towards ductal protion of the sebaceous gland. It was marked in tissue sections as areas of "poroid" cells and the tumour required differentiation from poroma. Focal areas of necrosis en masse in the present tumour contributed further difficulties in to the differential diagnosis. As single multivacuolar sebocytes were found and no cuticular cells could be identified, the tumour was diagnosed as ductal sebaceoma (sebomatricoma). The aspects of morphological distinction between sebomatricomas and poromas are presented with its clinical implications.

Diagnosis, Differential↗

Syringomatous adenoma of the nipple.

Syringomatous adenoma of the nipple (SAN) is an extremely rare neoplasm of the breast. We present a case of 41-year-old female with a retroareolar mass in her left breast. The patient has been operated on twice and remains free of disease for 20 months after the second operation. The nosological status of SAN and its relationship to the microcystic adnexal carcinoma of the skin is discussed.

Adenoma↗

2000 World Health Organization classification of tumors of the nervous system.

This is a review of the 2000 World Health Organization (WHO) classification of tumors of the nervous system. It contains an overview of the most important changes and short descriptions of the new entities or variants of already existing entities included in the current classification. These are: chordoid glioma of the third ventricle, cerebellar liponeurocytoma, large cell medulloblastoma, medulloblastoma with extensive nodularity and advanced neuronal differentiation, atypical teratoid/rhabdoid tumor, perineuroma, and rhabdoid meningioma. In contrast to the former WHO tumor classification series, the present one is based on the complex criteria, which include not only the clinical course and histologic appearance of the neoplasm but its immunophenotypic features and molecular/cytogenetic profile as well. Thus, it is strongly disease-oriented and uses extensively the recent advances in the basic sciences.

Humans↗

A case history of glioma progression.

Low-grade diffuse astrocytomas have an intrinsic tendency for malignant progression but the factors determining the kinetics of this process are still poorly understood. We report here the case of a male patient who developed a fibrillary astrocytoma at the age of 33 years and who underwent six surgical interventions over a period of 17 years without radiotherapy or chemotherapy. The first three biopsies spanned a period of 11 years and led to the diagnosis of low-grade, diffuse astrocytoma (WHO grade II), with a growth fraction (MIB-1 labeling index) of 2.3-3.7%. The fourth to sixth biopsies showed histological features of anaplastic astrocytoma (WHO grade III), with growth fractions between 5.0 and 10.5%. The fraction of gemistocytic neoplastic astrocytes also increased, from 0.3% in the first biopsy to 17.5% in the last biopsy and preceded the increase in proliferative activity and transition to anaplastic astrocytoma. The fraction of tumor cells immunoreactive to BCL-2 increased from 0.3% to 8.2%. A p53 mutation in codon 273 (CGT-->TGT, Arg-->Cys) was identified in the first biopsy and persisted throughout the course of the disease. However, the fraction of cells with p53 protein accumulation increased significantly during progression, from 3.2% in the first biopsy to 13.7% in the last. The absence of additional genetic alterations (PTEN mutations, loss of chromosome 10 and 19q) may be responsible for the slow progression and lack of glioblastoma features even after a 17-year disease duration.

Adult↗

Retiform/racemiform neoplasm with features of clear cell hidradenoma.

We report a case of a 78-year-old woman with a tumor of the left cheek. The tumor was a well-circumscribed cystic/solid nodule with a racemiform and reticulated pattern of growth of its epithelial cells, and mucinous and fibrocytic stroma. The epithelial cords and strands were continuous with the apocrine lining of large cystic structures. The main bulk of the epithelial component was formed by the proliferation of clear cells. This tumor is an example of an unusual benign neoplasm with racemiform and retiform patterns having a histogenetical link with the folliculo-sebaceous-apocrine unit.

Adenoma, Sweat Gland↗