[Undergraduate education in pathology in medical schools].
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Biomedical subjects
Publications and source records attributed to A Böör.
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AIM: To report a series of six cases of thyroid haemangiosarcoma (HAS) from a non-Alpine region. METHODS AND RESULTS: The patients were four females and two males, aged 54-81 years (average 68 years). The tumours presented as large haemorrhagic masses (diameter 40-70 mm, average 56 mm) with extensive necrosis. Histologically, they were composed of polymorphous epithelioid cells with vesicular nuclei and abundant eosinophilic cytoplasm with occasional intracytoplasmic lumina. Mitotic activity was high. Tumor cells expressed vimentin (6/6), CD31 (6/6), FVIII (5/6), CD34 (2/6), and cytokeratins (5/6). One tumour (1/6) over-expressed p53 protein in more than 20% of cells. Ultrastructurally, Weibel-Palade bodies were present (4/6). Clinical follow-up of four patients (range 3-24 months, median 9 months) showed that two of them have died of the disease 0.5 and 3 months after diagnosis, one died of unrelated causes (with 24 months' uneventful follow-up) and one is alive 21 months after operation with no evidence of disease. CONCLUSIONS: Although thyroid HAS is usually regarded as an extremely aggressive neoplasm with a dismal prognosis similar to anaplastic carcinoma, one of our cases suggests that HAS can behave in a less aggressive way. The morphological, immunohistochemical and ultrastructural findings support the hypothesis that thyroid HAS is a distinct entity, unrelated to other thyroid malignancies.
In certain primary and metastatic malignant melanomas diagnostic problems may arise due to their cytologic features and/or absence of synthesis of melanin. As the "classic" combination of S-100 protein and HMB-45 may occasionally fail to stain cells of malignant melanoma, we have tested a series of commercially accessible antibodies which were so far not compared by other authors in the three most frequent subtypes of this tumor. In surgical specimens from 104 cutaneous malignant melanomas (40 nodular melanomas, 46 superficially spreading malignant melanomas and 18 lentigo maligna melanomas) the staining intensity and the proportion of neoplastic cells stained with antibodies to S-100 protein, HMB-45, NKI/C3, NKI/beteb, MART 1 (Melan A), KBA 62 and Mitf was semiquantitatively analysed. The use of this group of antibodies against melanoma-associated antigens revealed it to be a favourable supplement for the bioptical or cytological diagnosis of malignant melanoma in case the traditional/conventional combination of S-100 protein and HMB-45 antibody fails. According to the authors' experience the antibody against KBA 62 has shown to be the most effective antibody followed by the antibodies against MART-1 (Melan A) and NKI/C3.
Fifty lung cancer samples (41 non-small cell lung cancer-NSCLC and 9 small cell lung cancer-SCLC) were immunohistochemically analyzed for lung resistance-related protein (LRP) and multidrug resistance-associated protein 1 (MRP1) expressions which were then correlated with histopathological subtype of the tumor. To detect these proteins, monoclonal antibodies LRP-56 and MRPm6 were used. NSCLC samples were divided into two groups, adenocarcinomas (17 samples) and squamous cell carcinomas (24 samples). Four categories of LRP and MRP1 quantity were distinguished: +++ = high level--90--100% of positive cells, ++ = lower level--10--90% of positive cells, + = low level--up to 10% of positive cells, - = negative cells--0% of positive cells. Within the NSCLC group the most samples (36/41) had the similar level of LRP and MRP1. Significantly higher expression of both proteins was observed in the adenocarcinomas in comparison with squamous cell carcinomas. The lowest positive staining for LRP and MRP1 proteins has been found in SCLC. It is suggested that our finding can confirm the overall empirical clinical knowledge about much higher chemosensitivity of untreated SCLC comparing to NSCLC.
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Mediastinal cysts, described also as homoplastic dysembryomas, account for 20% of mediastinal lesions. There are bronchogenic, oesophageal, gastrogenic and enterogenic, pericardial, non-specific cysts and cystic lymphangiomas. The authors present 6 patients with mediastinal cysts from a total number of 96 patients with mediastinal tumours subjected to surgery during a 14-year period (from Jan. 1 1987 to Dec. 31 2001). The group comprised 5 adults and one child. In four patients the authors selected thoracotomy as the route of access to the mediastinum, in one instance total sternotomy and once upper partial sternotomy. The cysts were removed as a whole. Histological examination revealed in four patients the diagnosis of a bronchogenic cyst, once a connective tissue cyst with respiratory epitheliumm and once a cyst lined with squamous epithelium. The postoperative course was in all patients free from complications. In the conclusion the authors emphasize the importance of complete removal of mediastinal cysts as relapses occur if part of the secretory eoithelium is not removed.
Based on a review of archival autopsy protocols an analysis of the frequency of pulmonary thrombembolism in consecutive autopsy material is presented. The study interval for the analysis was oriented to the period between the years 1949-2000. Our analysis has shown a gradual progression of incidence of pulmonary thrombembolism, as demonstrated in relative figures. The applied preventive measures against the development of thrombosis during hospitalization have not substantially contributed to decreasing its frequency, what is not in accord with some recent observations from other countries.
The authors report a rare clinical case of coincidence appendicitis and Fallopian tube torsion. A 14-years-old girl is presented with acute pelvic pain, dysuria and diarrhoea. Acute appendicitis and right side Fallopian tube torsion were detected by laparotomy. Symptoms, differential diagnoses, etiology and diagnostic procedures are discussed.
The paper presents a retrospective analysis of 20 patients with acute ethylene glycol intoxication who were treated from 1972 to 2001 in the Dialysis Centre of the IVth Medical Clinic and from 1997 at the L. Pasteur Nephrological Clinic of the Faculty Hospital and Safarík Medical Faculty in Kosice. The ethylene glycol intoxication was manifested by neurological symptoms, extreme metabolic acidosis, acute toxic hepatitis and acute renal failure. Laboratory examination revealed oxaluria in 17 patients and leukocytosis in all patients. Percutaneous renal biopsy was made for differential diagnostic or forensic reasons in the convalescent stage of acute renal failure in 6 patients. In the treatment of ethylene glycol intoxication the authors used ethyl alcohol as an antidote: in the first four patients administered by the i.v. route, in the remaining patients in dialyzation solution. Extreme metabolic acidosis improved in 15 patients after bicarbonate haemodialysis. Concomitant application of haemoperfusion over active charcoal during the first haemodialysis in four patients led to a potentiated effect on the uraemic syndrome. Conservative and extracorporeal elimination treatment of ethylene glycol intoxication succeeded in 16 patients (80%). According to the authors' experience early and fractionated application of bicarbonate haemodialysis with 100 mg% concentration of ethylalcohol in the dialysis solution is the method of choice in the treatment of ethylene glycol intoxication.
A fatal infantile storage disorder with hepatosplenomegaly and severe neurological disease is described. Sphingolipids, including monohexosylceramides (mainly glucosylceramide), dihexosylceramides (mainly lactosylceramide), globotriaosyl ceramide, sulphatides, ceramides and globotetraosyl ceramide, were stored in the tissues. In general, cholesterol and sphingomyelin levels were unaltered. The storage process was generalized and affected a number of cell types, with histiocytes, which infiltrated a number of visceral organs and the brain, especially involved. The ultrastructure of the storage lysosomes was membranous with oligolamellar, mainly vesicular, profiles. Infrequently, there were Gaucher-like lysosomes in histiocytes. The neuropathology was severe and featured neuronal storage and loss with a massive depopulation of cortical neurons and pronounced fibrillary astrocytosis. There was a paucity of myelin and stainable axons in the white matter with signs of active demyelination. Immunohistochemical investigations indicated that saposins A, B, C and D were all deficient. The patient was homozygous for a 1 bp deletion (c.803delG) within the SAP-B domain of the prosaposin gene which leads to a frameshift and premature stop codon. In the heterozygous parents, mutant cDNA was detected by amplification refractory mutation analysis in the nuclear, but not the cytoplasmic, fraction of fibroblast RNA, indicating that the mutant mRNA was rapidly degraded. The storage process in the proband resembled that of a published case from an unrelated family. Saposins were also deficient in this case, leading to its reclassification as prosaposin deficiency, and her mother was found to be a carrier for the same c.803delG mutation. Both of the investigated families came from the same district of eastern Slovakia.
A large trichinellosis outbreak in the Slovak Republic caused by the species Trichinella britovi and affecting 336 people also affected a pregnant woman. The mother was infected in the 10th week of pregnancy and was treated with mebendazole. On her own request abortion was performed in the 22nd week of pregnancy. Medium IgM and high IgG anti-Trichinella antibody titres were found. The placenta, body cavities liquid, tissues and organs of the foetus contained 0.02-30 larvae per gram of tissue, measuring 0.68 +/- 0.05-1.17 +/- 0.07 mm, with blurred inner structure. Immunocytochemical examination identified Trichinella larvae that infected the foetus in the early stage of development.
A 20-year-old woman presented with nasal obstruction and slight epistaxis. The obstructing lesion was excised and microscopy showed a neoplasm composed of comparatively uniform undifferentiated cells forming solid nests. The cytoplasm of the cells was clear but poorly demarcated, partly vacuolated and contained much glycogen. Although widespread in the nasal mucosa, the cells did not penetrate into the underlying bone. The cells expressed the MIC2 gene (using the CD99 marker). Electron microscopy showed simple cells with a small number of mitochondria, many glycogen particles; there were no neurosecretory granules present. Early surgical treatment followed by chemo- and radiotherapy have greatly improved the prognosis of EWS: extraskeletal Ewing's sarcoma (EWS/PNET).
The patient was a 54-year-old woman who had been suffering from chronic tubulo-interstitial nephritis for about seven years, requiring haemodialysis. More recently, she developed a polypoid mass in the left nasal cavity causing discomfort on breathing and slight epistaxis. The tumour was of gritty consistency and measured 28 x 8 x 5 mm. Microscopy showed a lobulated almost cystic structure composed of granulation tissue with comparatively few plasma cells and many multinucleated giant cells lining the spaces filled with crystalline deposits of calcium oxalate.
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Tumours situated in the posterior mediastinum and spreading to the spinal canal via the intervertebral opening are described as "dumbbell" tumours. The authors submit the case-history of a 44-year-old patient admitted to the Second Surgical Clinic Pasteur Faculty Hospital, Safarík University Kosice after repeated laminectomy and extirpation of the intraspinally spreading part of a mediastinal tumour. The mediastinal part of the tumour was removed surgically, the histological result was described as a melanotic schwannoma. The postoperative course was without complications, the patient was discharged home in a good condition on the 8th day after the operation. In the conclusion the authors emphasize the necessity to remove the tumour in toto. A one-stage operation in collaboration with a neurosurgeon seems appropriate.
Actinomycosis is subacute or chronic disease manifested by a defined granulomatous inflammation with the development of infiltrates, abscesses and fistulae. A 35-year-old female patient was admitted and operated at the Second Surgical Clinic because of symptoms of diffuse peritonitis. Laparotomy revealed a duplicit tumour of the small intestine, an abscess of the abdominal wall in the left mesogastrium and pyoovarium bilaterale. 70 cm of the small intestine were resected, incision of the abscess and bilateral adnexotomy were performed. Histological examination revealed a suppurative, partly fibroproductive inflammation with an actinomycotic etiopathology. After antibiotic treatment the patient was discharged home, the gynaecologist removed an intrauterine device. Three months after the first operation the patient in a serious septic condition was readmitted to the clinic with signs of diffuse peritonitis. A double perforation of the small intestine was found and an end-to-end anastomosis was made after resection of the small intestine. The postoperative course was complicated by respiratory failure and failure of the circulation associated with septic shock and subsequent death. In the conclusion the authors emphasize the problem of preoperative diagnosis of the abdominal form of actinomycosis, its possible development in relation to intrauterine contraceptive devices and its clinical manifestation as acute abdomen.
The authors present an account on patients with a teratoma of the mediastinum who were operated at the Second Surgical Clinic, L. Pasteur Faculty Hospital in Kosice. In the course of 10 years (Jan. 1, 1990-Dec. 31 1999) 73 patients with tumours of the mediastinum were operated. In four the diagnosis of teratoma of the mediastinum was confirmed by histological examination (5.47%): three adult patients and one child. In two patients the tumour of the mediastinum was diagnosed accidentally during X-ray examination of the chest. In one female patient surgical revision was indicated on account of a relapse of the process. In the conclusion the authors emphasize that teratomas of the mediastinum are frequently asymptomatic, and in case the process is in the anterior or upper mediastinum, teratomas must be taken into account and removed as a whole during surgical intervention.
An unusual location of a benign glomus tumour, outside of the constantly located regions, e.g. in the subungual location or deeply sited in extremities, was diagnosed in a 56-year-old white female in her posterior upper mediastinum. The single similar case report was published before the era of electron microscopy and immunohistochemistry and single cases of atypical and malignant forms in this unusual location were published only recently. The tumour measuring 5 x 4 x 2 centimeters has caused cough and was associated with occasional righ-sided chest pain. Its rich vascular supply has caused intensive intraoperative bleeding. The postoperative course was uneventful and the patient is free of neoplastic disease or symptoms six years after surgery. Numerous mast cells present within the tumour's interstices must be considered in relation to the possible pathogenesis of the up to now unexplained pain in glomus tumours.